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erythropoietin ( epo ) is a 34 kda glycoprotein hormone that controls the proliferation , differentiation , and survival of erythroid progenitor cells through an antiapoptotic mechanism . it has become apparent that epo protects many organs , including brain , heart , kidney and liver , against the injury caused by ischaemia / reperfusion , hemorrhagic shock and systemic inflammation . in this issue of critical care , kao and colleagues they found that recombinant human epo ( 400 iu / kg ) exerts significant beneficial effects when it is given as late as 18 hours after caecal ligation and puncture ( clp ) . although epo had no effect on the ( small ) decline in blood pressure , the decline in platelet and white blood cell counts , or the rise in lactate , it significantly increased tissue perfusion and reduced tissue hypoxia . within 18 hours after clp there was a decline in the number of perfused capillary beds ( reduced oxygen delivery ) ; this , in turn , resulted in an impairment in mitochondrial electron transport and hence respiration ( measured as increase in mitochondrial nadh fluorescence ) in skeletal musle ( extensor digitorum longus ) . most notably , epo rapidly ( within 10 min ) reversed both of these effects of clp , and hence it increased the number of patent capillaries and increased mitochondrial function . unfortunately , the authors did not measure any parameters of organ injury and dysfunction , and the model of clp used did not ( within 24 hours ) result in any deaths . thus , it remains to be seen whether the improvement in oxygen delivery or mitochondrial function afforded by epo also results in a significant improvement in outcome . the study by kao and coworkers is of particular importance because epo protects the brain , heart , kidney and liver against the tissue injury and dysfunction caused by ischaemia / reperfusion ( for review ) . the pathophysiology of the shock associated with trauma / haemorrhage also comprises elements of ischaemia / reperfusion injury ( because of hypovolaemia and resuscitation ) as well as excessive inflammation . in 2004 , we reported that administration of epo ( 300 iu / kg intravenously ) upon resuscitation reduced the renal dysfunction and liver injury caused by severe haemorrhage and resuscitation in rat . these beneficial effects of epo were associated with a reduction in tissue ( renal ) apoptosis secondary to prevention of activation of caspase-3 , -8 and -9 . interestingly , epo also prevents motor neurone apoptosis and associated neurological disability in an experimental model of spinal cord injury . low doses of epo ( 300 iu / kg intravenously ) did not affect the organ injury / dysfunction caused by high doses ( 6 mg / kg ) of the toll - like receptor-4 agonist lipopolysaccharide ( lps ) within 6 hours in the rat . however , higher doses of epo ( 4,000 iu / kg given 30 min before lps ) attenuated the renal dysfunction ( decline in glomerular filtration rate ) , which occurred at 16 hours after injection of low - dose lps ( 2.5 mg / kg intraperitoneally ) in mouse . this beneficial effect of epo in murine endotoxaemia was not due to effects of epo on either renal blood flow or apoptosis , but it was associated with prevention by epo of a fall in renal tissue superoxide dismutase activity associated with endotoxaemia . thus , mitra and coworkers concluded that the observed beneficial effects of epo in murine endotoxaemia are secondary to both antioxidant and anti - inflammatory effects of epo , which have been reported in other models of disease . thus , the beneficial effects of epo in rodent models of endotoxaemia may vary with doses of epo and lps , as well as species ( rat or mouse ) used . interestingly , higher doses of epo reduce both the systemic inflammation and the organ injury caused by the toll - like receptor-2 agonist zymosan in mouse . specifically , treatment of mice with epo ( 1,000 iu / kg subcutaneously , 1 and 6 hours after zymosan ) attenuated the signs of local ( peritoneal exudation ) and systemic ( lung inflammation ) , as well as organ ( lung , liver and pancreas ) injury and dysfunction ( kidney ) caused by zymosan . most notably , epo reduced the high mortality ( 70% ) caused by zymosan over the observation period of 7 days . the reported beneficial effects of epo in preclinical models of shock , trauma and haemorrhage are exciting , but further studies are warranted to determine the effects of epo on outcome ( organ injury / dysfunction and survival ) in models of clp . interestingly , in 86 patients admitted to a long - term acute care facility , administration of weekly recombinant human epo ( n = 42 ) resulted in a significant reduction in exposure to allogeneic red blood cell transfusion and higher haemoglobin levels than placebo ( n = 44 ) during the initial 42 days of epo therapy . although not significant , the mortality rates in patients treated with epo ( 12% ) were lower than in the patients treated with placebo ( 23% ) . the author is funded by the william harvey research foundation ( unrestricted research grant ) to study the tissue - protective effects of epo and has presented a number of invited lectures in this area of research .

erythropoietin protects many organs against the tissue injury and dysfunction caused by ischaemia / reperfusion and excessive inflammation . this editorial comment discusses the effects of erythropoietin in preclinical models of septic shock , endotoxemia , hemorrhagic shock , spinal cord trauma and zymosan - induced multiple organ failure .

electrocardiogram ( ecg ) is an important diagnostic tool for the prompt recognition of myocardial infarction ( mi ) . identification of st elevation mi by ecg in the critically ill patient population is limited by the lack of specificity due to a variety of non - ischemic processes . this is a case report of a 77-year - old male patient who presented with generalized weakness , confusion , somnolence , recurrent falls and tea - colored urine for 3 - 4 days . his past medical history was significant for hypertension , chronic obstructive pulmonary disease and coronary artery disease with a remote history of mi . his vitals showed blood pressure 97/42 , heart rate of 51 , respiratory rate of 12 and oxygen saturation of 93% on room air . there were extensive ecchymoses in different stages of healing on all 4 extremities and on the torso . baseline electrocardiogram during admission to the hospital the patient was rehydrated with intravenous normal saline and nephrotoxic medications were withheld . his renal function improved and on the 2 day , his chest x - ray revealed patchy bilateral infiltrates . on the 3 day , he developed respiratory distress with an increase in oxygen requirements . the chest computed tomography revealed bilateral ground glass opacities with consolidation consistent with acute lung injury / acute respiratory distress syndrome . after failing a trial of non - invasive positive pressure ventilation , he was intubated and mechanically ventilated for hypoxic respiratory failure . he developed intermittent supraventricular tachycardia , atrial flutter and atrial fibrillation were treated with intravenous esmolol and diltiazem after which he converted to sinus rhythm . on the 4 hospital day , he developed increasing oxygen requirements and asynchronous volume control ventilation ; his ventilator settings were switched to pressure control ventilation . an echocardiogram revealed left ventricular ejection fraction of 45 - 50% with grade ii diastolic dysfunction and no wall motion abnormalities . he later became progressively hypoxic and tachypneic on the ventilator during the next couple of days . his clinical status worsened and his ventilator settings were changed ; the positive end - expiratory pressure ( peep ) was increased to 13 mm hg . ecg at the time showed st segment and t wave changes concerning for st elevation mi . careful review of the ecg revealed bizarre , broad - based apparent st - segment elevation in the precordial leads where the upward shift of the baseline started slightly before the onset of the qrs complexes . a more obvious dome and spike pattern was seen in the inferior leads [ figure 2 ] . his central venous pressure decreased from 12 to 7 mm hg and tidal volumes were increased from 550 to 750 cc during this period . an urgent bedside echocardiogram showed preserved left ventricular ejection fraction with no wall motion abnormalities . the patient passed away on the 11 day of hospitalization from acute respiratory distress syndrome . st - t segment changes of precordial leads after increase in positive end - expiratory pressure to 13 mm hg . german military helmet with dome and spike ( inset ) reproduced with permission from mayo clinic proceedings resolution of st - t segment changes in precordial leads after the discontinuation of positive end - expiratory pressure our case represents an uncommon finding of dome and spike st - t wave changes mimicking st elevation mi in the setting of a critical non - cardiac illness . these findings were similar to those reported by littmann and monroe and tomcsnyi et al . the st - t wave changes in these cases were likened to a spiked german military helmet [ inset figure 2 ] the pickelhaube , introduced in 1842 by friedrich wilhelm iv , the king of prussia . the mechanism of this phenomenon is unknown and several postulated mechanisms for this finding include ( 1 ) artifact ( 2 ) a sudden increase in the intra - abdominal or intra - thoracic pressure ( 3 ) direct stimulation of inferior wall of left ventricle by the diaphragm , ( 4 ) stimulation of the diaphragm by the left phrenic nerve , ( 5 ) synchronized contraction of diaphragm along with the cardiac cycle in the setting of alkalosis with latent tetany , ( 6 ) acute stretch of skin overlying the chest wall with each cardiac cycle and ( 7 ) diaphragmatic breathing that alters ecg patterns . the acute rise in intrathoracic pressure with an increase in peep most likely contributed to the dome and spike pattern in our patient . this abnormal ecg finding with a dome and spike helmet sign pattern in a critically ill patient is a marker for very high mortality . it is an under - recognized phenomenon and further research is needed as early recognition can possibly assist clinical management .

a 77-year - old male patient presented with rhabdomyolysis . he developed progressive respiratory failure and acute respiratory distress syndrome during his hospital stay requiring mechanical ventilation . an electrocardiogram during mechanical ventilation showed findings suggestive of st elevation myocardial infarction . closer review showed dome and spike findings that have been likened to a spiked helmet . this finding has been associated with significant mortality . we discuss this under - recognized finding and the potential contributing mechanisms .

in the previous issue of critical care , van herpe and colleagues describe a new method of blood glucose reporting for hospitalized patients , the glycemic penalty index ( gpi ) . a stepped scale weights the clinical significance of differences of blood glucose ( bg ) from target . the gpi scale is appropriately accordioned on the hypoglycemic range and expanded on the hyperglycemic range . the authors recommend that use of the index should be combined with counting of episodes of severe hypoglycemia . the index , capturing both overall hyperglycemia and hypoglycemia , could permit analysis of the ability of an algorithm to control between - patient glycemic variability . in the evaluation of glycemic control , the measures that are simplest to ascertain are the average and standard deviation , using the bg as the unit of observation . in the leuven , belgium surgical intensive care unit , the standard deviations in intensively and conventionally treated groups was 19 and 33 mg / dl , respectively . it could have been asked whether the greater bg variability in the conventionally treated group reflected the contribution of a hyperglycemic subgroup . until a second report was issued referring to control at the patient level , within the conventionally treated group even the relationship between hyperglycemia and mortality rate was unclear . in order to evaluate patient outcomes , there is no substitute for reporting on the patient as the unit of observation . a key question is whether patient outcomes relate to overall hyperglycemia during a critical timeframe or to specific episodes of severe hyperglycemia . the answer , predicated upon the mechanism of harm , could depend upon what outcome is being studied . single episodes of severe hyperglycemia , such as the in - hospital development of diabetic ketoacidosis or hyperglycemia - associated dehydration , may result in specific consequences , such as dialysis fistula thrombosis , readmission to a critical care unit , or treatment - related pulmonary edema . the association of single episodes with outcomes was recognized by stagnaro - green and colleagues . furnary and colleagues , capitalizing upon a critical window of time in the postoperative interval following heart surgery , have reported on outcomes in relation to the " 3-day bg , " each value representing a patient 's 3-day average of postoperative bg measurements . recent literature supports the importance of overall prevention of hyperglycemia , at least during critical windows of time , with respect to survival and morbidities such as sepsis , renal failure , duration of ventilator dependency , or transfusion requirement . unfortunately , we have no simple measure comparable to the a1c by which the short - term inpatient overall glycemic control of an individual may be described . although hypoglycemia might simply reflect severity of comorbidities , the correlation between hypoglycemia and mortality of hospitalized patients is well known . evidence from a mixed intensive care unit suggests that hypoglycemia is an independent predictor of mortality . among groups of patients having myocardial infarction or congestive heart failure , observational patient - level data suggest that when mortality is considered as a function of overall bg concentration , there may be a j - shaped curve , such that patients having the lowest and the highest averages experience outcomes worse than those having intermediate range bg control . is there a plausible mechanism by which modest overall reduction of average bg might cause harm , and if so , what specific harm is caused ? alternatively , is harm a consequence of isolated episodes of severe hypoglycemia , to which the population having lower average bg is more vulnerable ? in the literature concerning strict glycemic control , serious or fatal consequences of hypoglycemia occasionally are reported . however , even when severe hypoglycemia is reported , sublethal permanent neurological injury seldom is described . if a patient - level metric relied only upon bg averaging methods , isolated episodes of severe hypoglycemia could be overlooked that had resulted in altered function with respect to the activities of daily living or reduction of intellectual capacity . application of the gpi index is cumbersome , such that other centers may have difficulty in adopting the method ; therefore , the measure may remain unfamiliar . perhaps overall hyperglycemia and discrete episodes of hypoglycemia ought not to be captured by the same metric . an extra mental step is needed to quantify the contribution of hypoglycemia and hyperglycemia as a fraction of the total value of the index . multiple small episodes of hypoglycemia , none having clinical impact , might be weighted equally to one severe life - changing episode . analysis of episodes of severe hypoglycemia should complement the use of the gpi , as the authors acknowledge . with that caveat , can a single measure sum up patient risk for a variety of outcomes that might be related to glycemic control ? we await evidence that the gpi will improve upon our ability to define glycemic targets and predict clinical outcomes .

the article by van herpe and colleagues in the previous issue of critical care describes the glycemic penalty index ( gpi ) , which weights both hyperglycemic and hypoglycemic blood glucose measurements commensurate to their clinically significant difference from target . although certain adverse consequences result from isolated severe hyperglycemic episodes , several specific outcomes depend upon overall hyperglycemia . in contrast , although mortality has been related epidemiologically to overall low blood glucose , specific negative outcomes may depend upon isolated episodes . capturing both hypoglycemia and hyperglycemia in a single index will be shown to be useful if the gpi enables us to better define insulin strategies , outcomes , and targets .

urethral diverticular adenocarcinoma is a rare cancer found in female patients , presenting only 5% of female urothelial cancers . we report a case of a urethral diverticular adenocarcinoma after the fenestration of the anterior vaginal wall for pelvic floor abscess . a 61-year - old woman presented to the gynecology department with complaints of atypical genital bleeding . magnetic resonance imaging ( mri ) revealed a localized urethral tumor extended to vagina ( fig . 1 ) . computed tomography did not reveal any evidence of metastasis . under the diagnosis of urethral tumor , the patient was referred to our department . because the fenestration of the anterior vaginal wall was performed under the diagnosis of the abscess in the pelvic floor for the patient 15 years ago and the histological test demonstrates the adenocarcinoma , we suspected a urethral diverticular adenocarcinoma . figure 1:sagittal t2-weighted mri of the pelvis revealed a localized urethral tumor extended to vagina . sagittal t2-weighted mri of the pelvis revealed a localized urethral tumor extended to vagina . on admission , we performed standard open total cystectomy with lymph node excision and ileal conduit . the resected specimen showed the mass on the anterior vaginal wall ( fig . 2 ) . histopathological examination of the specimen showed adenocarcinoma tumor that continues to anterior vaginal wall , but urethral diverticular wall could not be identified ( fig . we could not establish a diagnosis of urethral diverticulum ; however , we clinically diagnosed as urethral diverticular adenocarcinoma . the proximal margin was negative , and all lymph nodes were negative . because the post - surgical course was uneventful , the patient was discharged from our hospital on day 27 post - surgery . figure 3:histopathological examination of the specimen showed adenocarcinoma tumor which continues to anterior vaginal wall , but urethral diverticular wall could not be identified . histopathological examination of the specimen showed adenocarcinoma tumor which continues to anterior vaginal wall , but urethral diverticular wall could not be identified . , there are less than 60 reported cases of urethral diverticula carcinoma , including adenocarcinoma and squamous cell carcinoma . urethral bleeding is the most common presenting symptom , occurring in 51% patients . a painless growth , found initially as either an anterior vaginal mass or a urothelial obstruction , was present in 24% patients therapy for urethral diverticular carcinoma consists of local excision such as diverticulectomy or more aggressive therapy such as definitive radiation therapy or radical cystourectomy with pelvic lymph node dissection . in our case , it is possible that drainage treatment was performed for urothelial diverticulum 15 years ago . patients with urethral diverticula often present with a variety of nonspecific lower urinary symptoms such as frequency , urgency , localized pain or dysuria . approximately 4% cases of female urethral diverticulum cause urinary retention . an asymptomatic patient with urethral diverticulum can be managed by observation . on the other hand , the first and most common choice of treatment for symptomatic urethral diverticulum is complete vaginal excision . however , because of several risks and the high recurrence rate of complete vaginal excision , we have advocated that the management by transvaginal drainage could be the appropriate treatment for urethral diverticulum on the past published case report . this procedure is effective and conservative with few complications , but we should not forget very rare cases of carcinoma arising in urethral diverticula have been reported . in this case , if the urethral diverticulum had removed completely 15 years ago , the generation of malignant tumors might have been avoidable . anyway , for the patient with urethral diverticula , close long - term follow - up for the recurrence or generation of malignant tumors by genitourinary examinations or images is necessary .

a 61-year - old woman presented to the gynecology department with complaints of atypical genital bleeding . magnetic resonance imaging revealed a localized urethral tumor extended to vagina . histological test of the biopsy tissue of the mass suggested the adenocarcinoma . the patient was performed the fenestration of the anterior vaginal wall 15 years ago . under the diagnosis of urethral diverticular adenocarcinoma , we performed standard open total cystectomy with lymph node excision and ileal conduit . we could not establish a diagnosis of urethral diverticulum from the histological test ; however , we clinically diagnosed as urethral diverticular adenocarcinoma . because carcinoma arising from urethral diverticula is reported , a close long - term follow - up for the recurrence or generation of malignant tumors by genitourinary examinations or images is necessary , for the patient with urethral diverticula .

eccrine poroma is a benign neoplasm of the sweat glands originating from the intraepidermal eccrine duct.1 ) this unusual tumor is known to arise in bare skin areas , such as the sole of the foot . recently it was revealed that it could occur in more various skin region.2 ) but only few cases have been reported in the head and neck area . a 54-year - old female presented with a gradually increasing mass on the auricle for one year . on physical examination , there was a soft , protruding , and purple - colored , solitary mass , 1.01.0 cm in size on the retroauricular surface of the left auricle ( fig . the patient reported serous discharge but there was no pain nor tenderness . with an impression of an atypical nevus or a melanoma histopathological findings revealed that broad anasto - mosing bands extended from the epidermis to dermis with multifocal fibrovascular cores and irregular duct - like structures ( fig . there were also tightly packed small uniform cells with central round to oval nuclei , prominent nucleoli , and occasional clear cytoplasm ( fig . we did not find any necrosis , nuclear atypia , or mitosis in the specimen . after complete excision , the patient has been followed up without any recurrence for more than 6 months . eccrine poroma was first described by goldman , et al.2 ) in 1956 as a neoplasm composed of cells that are analogous to cells in the walls of eccrine sweat pores . it is composed of poroid cells and cuticular cells that have much eosinophilic cytoplasm , similar to eccrine ductal cuticles.1 ) vacuolizations in the intracytoplasmic and intercellular areas are characteristic histological features , resembling the formation of eccrine ducts.1 ) this tumor is occurred in bare areas of skin such as the sole of the foot . goldman , et al.2 ) reported that the most common sites were soles of feet ( 65% ) followed by the hands ( 10% ) . only one case of the ear ( external auditory canal ) has been previously reported , and only three cases reported in the postauricular scalp skin , not on the auricle worldwide . among seven cases reported in the korean literature , the scalp was the most common site.3,4,5,6 ) moore , et al.7 ) compared patients with poroma in the head and neck area with lesions occurred in extremities . lesions of the head and neck were mostly asymptomatic mass , but some patients with poroma in the extremities complained symptoms such as pain or discharge . and more pigmented lesions were noted in the head and neck , compared with mass of the extremities . in our case , histopathological diagnosis is crucial because eccrine poroma should be differentiated from basal cell carcinoma and seborrheic keratosis.3 ) poroma seems to have more organoid appearance than basal cell carcinoma . also , poroma has fewer epithelial cells compared to the seborrheic keratosis of the sweat duct . it has been found that about 18% of poromas transform to procarcinoma.8 ) in a case of reported malignancy , it causes multiple cutaneous metastases leading to death.4 ) porocarcinoma shows more exphytic and ulcerative appearance than eccrine poroma . when the eccrine poroma develop into malignancy , it shows spontaneous bleeding , ulceration , sudden itching sense or pain , and rapid growth in a short period.8 ) so early detection , complete excision and close follow up are important in the management of the tumor .

eccrine poroma is described as a benign neoplasm originating from the intraepidermal eccrine duct of sweat glands . this tumor is known to arise in bare skin areas , but more rarely appeared in head and neck region . a 54-year - old female presented with a mass on the retroauricular aspect of the left auricle . there was a soft , protruding , and purple - colored , solitary mass of about 1.01.0 cm in size . after authors performed an excisional biopsy , eccrine poroma was confirmed histopathologically . thus , we report a rare case of eccrine poroma of the ear with the review of literature .

infantile tremor syndrome ( its ) is characterized by anemia , skin pigmentation , tremors , physical , and mental regression without a defined etiopathogenesis and low incidence . we have studied 9 patients over 1 year for the changing clinical and laboratory variables of patients with its . neuroregression and anemia were presented in all followed by tremors in 5 and hypotonia in 2 . infantile tremor syndrome ( its ) is a syndrome characterized by anemia , skin pigmentation , tremors , physical , and mental regression . it has been reported in children between 5 months and 3 years of age with a male predominance . the purpose of this case series was to determine factors responsible for its continuing prevalence despite better knowledge and weaning practices . we have studied 9 patients presenting to us with typical features of its and pre - its in a tertiary care hospital of hilly terrain [ table 1 ] . a detailed history and examination investigations included complete hemogram , peripheral smear to type the anemia and malaria parasite , serum electrolytes , renal and liver function tests along with sepsis screen , and urine routine examination . blood and urine cultures , serum b12 levels , chest x - ray , and tuberculosis screen were done when indicated . written informed consent was taken from all . total 9 patients , 3 males and 6 females presented over the period of 1 year . the mean age of presentation was 13.5 months , ranging from 7 months to 18 months . all ( 100% ) patients admitted with the complaint of lethargy and weakness with a mean duration of 21 days . neuroregression was present in all ( 100% ) with a mean delay of 7 months ranging from 4 to 11 months in all spheres of development . tremors were present in 5 ( 55% ) and hypotonia in 2 ( 22% ) reflecting neurological changes . seven ( 78% ) were exclusively breastfed and 2 ( 22% ) improperly weaned with diluted milk . protein energy malnutrition was present in 5 ( 55% ) out of which 2 ( 40% ) had grade i and 3 had grade ii ( 60% ) protein energy malnutrition as per iap classification . ofc records of all patients were within normal range for their age [ table 2 ] . all had severe pallor ( 100% ) with knuckle hyperpigmentation and sparse hair in most . hepatomegaly was present in 4 ( 44% ) , splenomegaly in 2 ( 22% ) , and pneumonia in 1 ( 11% ) . peripheral blood film examination revealed the macrocytic picture in 4 ( 44% ) , dimorphic in 4 ( 44% ) , and microcytic in 1 ( 11% ) . chest x - rays had infiltrated in 3 ( 33% ) but workup for tubercular screening was negative . urine cultures were positive in 2 ( 22% ) with escherichia coli and pseudumonas in 1 each . serum b12 levels were low in 4 out of 5 patients but could not be performed in all due to financial constraints . the mean duration of hospital stay was 9 days ranging from 4 to 16 days . total 6 ( 66% ) with positive sepsis screen or cultures received antibiotics for the average duration of 9 days . improved after treatment with supplements such as vitamins a , d , k , b - complex , zinc , calcium , and magnesium along with dietary management . on monthly follow - up , those with pre - its had better catch up in growth and development in all spheres . infantile and pre - its is a well - known clinical entity reported primarily from the developing nations . despite its decreasing incidence , the finding of additional clinical features made us present this series . associated nutritional deficiencies such as protein , vitamin a , d , c , and b - complex may be present . our series also found that all children with this syndrome had anemia , lethargy , and neuroregression and presented throughout the year with no seasonal clustering as previously stated . furthermore , the type of anemia in this disease is not specific . on the other hand , it reflects the pattern of prevalence of the hematinic deficiencies in the region . however , unlike previous reports , there were more female patients , which may be due to bias in nutritional status . the study adds the need for rational antibiotic use in patients of its , unlike many previous reports where all patients have been administered antibiotics . the disease process may be superadded by urinary infections probably due to lower immunity state as a result of malnutrition , the leading cause of acquired immunodeficiency . it is also a reflection of changing epidemiology with no seasonal variation , showing the role of non - viral etiologies although previous studies show a seasonal pattern of disease . it highlights the need for better education for weaning practices and need for better community support .

background : infantile tremor syndrome ( its ) is characterized by anemia , skin pigmentation , tremors , physical , and mental regression without a defined etiopathogenesis and low incidence.materials and methods : we have studied 9 patients over 1 year for the changing clinical and laboratory variables of patients with its . neuroregression and anemia were presented in all followed by tremors in 5 and hypotonia in 2.result:sepsis screen was positive in 6 and urine cultures in 2 . antibiotics were required in 6 . its with changing parameters still significantly contributes to healthcare burden.conclusion:it is important to screen for urinary infection and septicemia to avoid antibiotic abuse .

coexistence of hyperthyroidism and primary immune thrombocytopenia ( itp ) is a rather rare condition . underlying untreated graves disease leads to worsening of the itp and rendering it refractory to standard therapy . a 71-year - old female patient presented with a complaint of excessive sweating for nearly a month to a hematology unit where she had been followed - up with after a diagnosis of itp . her laboratory tests revealed manifest hyperthyroidism in addition to thrombocytopenia , and hence she was referred to our endocrinology unit . on her physical examination , her pulse rate was 112 bpm , and her skin was warm and damp . increased levels of anti - thyroid peroxidase , anti - thyroglobulin , and thyroid - stimulating antibody and enhanced uptake of radioactive iodine were observed , and the diagnosis of graves disease was confirmed . methimazole therapy at daily doses of 30 mg was initiated . without any additional immunosuppressive treatment , at week 12 of methimazole therapy , thyroid stimulating hormone ( tsh ) levels returned to normal , and platelet counts rose to tolerable levels . when her hospital records were analyzed , they revealed that a year ago , when she had been diagnosed with itp , her tsh values had been suppressed . at that time she had been given intravenous immune globulin ( 0.5 g / kg / day for 4 days ) and oral steroid ( 1 mg / kg / day ) for the treatment of itp . the patient , whose platelet counts returned to being within normal limits , was followed - up with for 6 months . during this period , her platelet values were maintained at normal levels , and during her control visits , her tsh levels were measured twice and were within normal limits . the patient 's laboratory test results are shown in chronological order [ table 1 ] . afterwards , concomitancy of autoimmune thyroid diseases such as hashimoto thyroiditis and graves disease with itp has been reported many times . in graves disease , the lifespan of platelets is shortened significantly , and moderate degrees of thrombocytopenia are frequently seen . the potential mechanism involved in this process the association between itp and graves disease is explained by immune dysregulation , which is the main mechanism underlying both diseases and involves an increase in the levels of antiplatelet and anti - thyroid antibodies in these patients . because of the strong fundamental autoimmune mechanism underlying both diseases , it is known that the treatment of associated autoimmune thyroid disorder contributes to the remission of itp . it has been reported that the treatment of underlying graves disease in patients refractory to standard immunosuppressive therapy ensured remission of itp . besides , only anti - thyroid treatment without additional immunosuppressive treatment enabled remission of itp . in our case , although spontaneous remissions can be seen in graves disease , we think that immunosuppressive therapy given for the treatment of itp - induced a transient euthyroidism in graves disease . during the 6 months following itp therapy of our patient , whose tsh values remained at normal levels , graves disease and concomitant itp recurrences were observed , and only anti - thyroid therapy increased platelet counts to tolerable levels . it should be kept in mind that immunosuppressive therapy without considering thyroid function tests may result in a transient euthyroid state , which potentially masks graves disease accompanying immunosuppressive therapy and associated recurrent itp attacks .

a 71-year - old female patient followed primary immune thrombocytopenia ( itp ) was admitted to endocrinology unit with excessive sweating . we started methimazole for graves disease . without any additional immunosuppressive treatment , at week 12 of methimazole therapy , thyroid stimulating hormone ( tsh ) levels returned to normal , and platelet counts rose to tolerable levels . when her hospital records were analyzed , they revealed that a year ago , when she had been diagnosed with itp , her tsh values had been suppressed . after immunosuppressive therapy , her platelet values were maintained at normal levels , and during her control visits , her tsh levels were measured twice and were within normal limits . we think that immunosuppressive therapy for itp without considering thyroid function tests may result in a transient euthyroid state , which potentially masks graves disease accompanying immunosuppressive therapy and associated recurrent itp attacks .

a 57-year - old woman presented with a three - day history of photophobia and diplopia in the left eye . she was diagnosed with histologically confirmed nasal nktl of the right nasal cavity one month prior . the snellen visual acuity of the affected eye with correction was 1.0 / 0.63 , and the intraocular pressure in the right and left eyes was 17 and 18 mmhg , respectively . the patient had mild left eye conjunctival injection , ptosis , and swelling of the eyelid . opthalmoplegia , vitreous opacity , and an iris mass developed in the left eye ( fig . thirty - five percent of the cells obtained from the aspiration were morphologically atypical lymphocytes . skin biopsy confirmed neoplastic infiltration of small- to medium - sized pleomorphic lymphocytes with irregular nuclei , inconspicuous nucleoli , and scant cytoplasm ( fig . immunohistochemical staining was positive for cd56 ( fig . 2c ) and cd3 ( fig . the patient was diagnosed with nasal nktl with ocular involvement . after receiving radiotherapy at 900 cgy , her visual acuity improved and her anterior uveitis , vitreous opacity , and iris mass resolved ( fig . 3a and 3b ) . however , the patient 's condition continued to deteriorate despite radiotherapy and chemotherapy , and her ptosis and ophthalmoplegia gradually progressed . extranodal nktl , including nasal nktl , previously known as lethal midline granuloma , is a definitive diagnostic entity according to the world health organization lymphoma classification . however , histopathologically identical tumors may be identified at other extranodal sites , including the skin , muscle , gastrointestinal tract , liver , kidney , and retroperitoneal space . did report a case of histologically confirmed nasal nktl with intraocular involvement , however , in most cases intraocular involvement has not been confirmed histologically . the presence of intraocular involvement raises the possibility of leptomeningeal or central nervous system dissemination , and prompts consideration for external - beam radiotherapy to the eye and orbit in addition to systemic chemotherapy . nktl involving the ocular adnexa is generally a rapidly progressing disease , with a short survival from time of diagnosis , despite standard therapy . this was true in the current case as well ; the patient died three months after diagnosis . since nktl with ocular and orbital involvement is very rare and is characterized by rapid disease progression and a poor prognosis , nktl should be considered in the differential diagnosis of uveitis or orbital pseudotumor refractory to therapy .

herein , we report a case of nasal natural killer t - cell lymphoma ( nktl ) with intraocular involvement . a 57-year - old woman was referred due to a three - day history of photophobia and diplopia in the left eye . one - month previously , she was diagnosed with nasal nktl of the right nasal cavity . ophthalmic examination revealed conjunctival injection and ptosis . the left pupil was fully dilated and non - reactive to light . ocular motion was restricted on left - upper gaze . five days later , anterior uveitis developed and persisted despite topical steroid treatment . an orbital magnetic resonance imaging was without specific findings , however , ophthalmoplegia , vitreous opacity , and an iris mass were observed . a diagnostic anterior chamber aspiration was performed . aqueous humor aspiration revealed 35% morphologically atypical lymphocytes . after an intravitreal triamcinolone injection , radiotherapy and chemotherapy were administered ; this resolved the uveitis and iris mass . when refractory uveitis or orbital pseudotumor occurs in patients with nasal nktl , ocular and orbital involvement of the nktl should be considered .

many authors have discussed the importance of measuring cardiac output and then titrating therapy according to these measurements in patients in the operating theatre and intensive care environments . indeed , in some circumstances these measurements have led to changes in therapy that , in themselves , have been associated with improvements in outcomes . the ' art ' or ' science ' of measuring this variable is therefore rightly given significant airplay in the ongoing literature of our specialty . these include methodologies based on indicator dilution or thermodilution , doppler principles , the fick technique and also pulse pressure analysis . the pulse pressure analysis techniques have become increasingly popular due to the rising number of companies now marketing these devices . it is incumbent on us as practicing clinicians to understand the similarities and differences between these devices so that we can ensure that we use techniques that we can rely upon to be accurate and precise in the clinical environment and also then integrate with therapies that are beneficial to our patients . if we step back and look carefully at how these tools are used , then we would purport that there are two different scenarios that could be discussed . this needs an accurate and precise measurement in order to provide useful information [ 5 - 7 ] . the second scenario is where clinical interventions are titrated against changes in cardiac output - for instance , with a passive leg raise or volume challenge . in this scenario it is less relevant that we have an accurate and precise measurement , although it is more important that we can track the changes in the underlying signal reliably . on the whole , the pulse pressure analysis techniques for estimating cardiac output are better placed at helping us with this second scenario than the first . in order to have an accurate and precise measurement , the relationship between arterial pressure and central impedance needs to be clarified and this usually means having to make an independent measurement as impedance is notoriously difficult to measure . most companies therefore market these devices combined with another method of measuring cardiac output to calibrate the pulse pressure algorithm at baseline for this problem - commonly with either transpulmonary thermodilution or lithium ( indicator ) dilution techniques . on a beat to beat basis pulse pressure provides a very good surrogate of changes in stroke volume . as the time interval lengthens , however , this relationship becomes less robust as the vascular tone will change , thereby adversely influencing this signal . the same holds true for the measurement of changes in stroke volume and/or cardiac output from pulse pressure tracking techniques . over time many of the competing influences on the systemic vasculature will alter - level of preload , compliance , arterial resistance , and so on this makes the assumption that changes in the arterial pressure signal directly relate to changes in flow less robust . on a beat unfortunately , these tools are rarely used over a beat to beat basis and are more commonly used over a period of time that may be 30 minutes or perhaps over an hour . if we look at the variety of methodologies used for giving a fluid challenge we can see this all too vividly . after 60 minutes it is quite possible that the vascular tone has changed significantly , thereby raising the question as to whether the change in flow estimated from the pressure signal is real or artefactual . in order to understand this problem a number of authors have investigated these techniques under changing circulatory conditions . in an elegant study , marquez and colleagues demonstrated that the lidcoplus algorithm , when compared against aortic flow probes , was able to track changes in stroke volume in response to a venous occlusion , although there tended to be an underestimation at higher values . yamashita and colleagues assessed how the precision of the algorithms was maintained under therapeutic vasodilatation with prostaglandin e1 during cardiac surgery . they tested the lidco plus and the pulse contour method of the piccoplus versus the intermittent thermodilution of the pulmonary artery catheter . these studies suggested that after significant haemodynamic change ( vasodilatation ) , the algorithms may underestimate the cardiac output and therefore not give a reliable estimate in the change of the signal . more recently , monnet and colleagues assessed how the piccoplus and the vigileo ( v1.10 ) handle vasoconstriction induced by infusion of norepinephrine . they concluded that the vigileo algorithm was less able to track the changes in cardiac index during these situations . a further important consideration from all of these studies is that each algorithm , or algorithm update , will behave differently and will require independent validation . this can be seen in the meta - analysis published by mayer and colleagues looking at the new and older versions of the vigileo algorithms where dramatically differing levels of accuracy and precision were seen . it seems clear that if these devices are to be used to be able to track changes in cardiac output induced by changes in preload , then much care must be taken to ensure that in addition there are no major influences from altered vascular tone . the only way of ensuring this is to make the time interval between measurements short - perhaps minutes rather than hours . if we want to assess the circulation over longer time intervals , then a measurement independent of pulse pressure analysis needs to be included to compensate for these changes in vascular tone . when designing methodologies for assessing the response to a passive leg raise , an end expiratory occlusion , a valsava manoeuvre or a fluid challenge this message needs to be understood . perform the intervention quickly and the monitor should be able to track the change reliably and the correct interpretation should be made .

pulse pressure analysis algorithms are commonly used to measure cardiac output and to allow for the rational titration of therapy in critically ill patients . the ability of these algorithms to accurately track changes in stroke volume ( and cardiac output ) is thus very important . most of the currently available algorithms can provide robust data so long as there is no fundamental change in the vasomotor tone ( arterial compliance or impedance ) . if the tone changes significantly , for instance with vasodilatation or vasoconstriction , then the data become less robust . for this reason , unless there is a mechanism for compensating for changes in vasomotor tone , these algorithms are best used only over short time periods in order to get the most accurate and precise data on changes in cardiac output .

a 28-year - old male patient came to our hospital outpatient department with complaints of short hair ( 5 - 8 mm in length ) coming out from the left upper eyelid since childhood as reported by his parents . on close examination by the dermatologist and the physician , the ectopia cilia was located 7 mm superior - lateral to the left upper lid margin . the patient 's maternal grandfather ( since deceased ) had similar ectopia cilia on left upper eyelid as was reported by patient 's mother [ figure 2 ] . ectopia cilia with eleven hair lash bundles pedigree chart of ectopia cilia an out - patient surgical excision procedure under local anesthesia was done . an incision parallel to the lid margin was given and root of the tuft dissected . histological findings were suggestive of eccrine sweat glands with no features of apocrine glands and dermoid cyst seen as reported by the pathologist . after 3 month 's follow - up , no recurrence was detected . the presentations in published works falls in two distinct categories : cilia protruding from the anterior surface and cilia protruding from posterior surface of the tarsal plate . the anteriorly placed cilia are uniformly located on the lateral quarter of the upper eyelid and associated with the presence of apocrine sweat glands and are congenital in origin . dalgleish presented two cases - one of them had large apocrine sweat glands attached to the follicle . baghestani and banihashemi reported first case of ectopia cilia with pedigree analysis in a 14-year - old iranian boy with a positive history of the same anomaly in his paternal grandfather demonstrating evidence of an inherited genetic disorder . reported that only nine cases of ectopia cilia have been reported so far till 1991 . the differential diagnosis of ectopia cilia includes several eyelash abnormalities such as cilia incarnata , a condition in which an extra eyelash grows from a normal origin through the eyelid to the inner aspect of the tarsal conjunctiva or outward to the skin . wang et al . presented an unusual case of a dermoid cyst presenting with black hairs emerging from a sinus tract on the upper eyelid and mimicking the appearance of ectopic cilia . distichiasis and trichiasis are other conditions which also need to be differentiated from ectopia cilia . trichiasis is a condition in which cilia originates from a normal position on the eyelid turn inward due to entropion . the nearness of tuft to the tarsal plate , the texture and the direction of the cilia suggest that the origin of the ectopia cilia may probably be related to the eyelashes in our patient .

we present a case of ectopia cilia in a 28-year - old male patient . ectopia cilia was were seen in the outer third of left upper eyelid . the patient 's maternal grandfather also had ectopia cilia of the left upper eyelid as reported by the patient 's mother . ectopia cilia is a rare condition seen in humans . only 12 cases of ectopic cilia in humans have been reported so far in the world . the present case of ectopia cilia is the second case report in the world with pedigree analysis .

qt was received as gifted by torrent pharmaceuticals pvt . ltd . , polyox n 10 , polyox n 80 , polyox n 750 and polyox n 205 were gifted by dow chemicals pvt preliminary trials were carried out for selection of appropriate grades of polyox from different grades mentioned above . concentration of polymer and plasticizers were optimized by central composite design . in this design 2 factors the amount of polyox n750 ( x1 ) and peg 400 ( x2 ) were selected as independent variables . the tensile strength , folding endurance , % drug released at 10 min ( y10 ) and disintegration time were selected as dependent variables . preliminary trials were carried out for selection of appropriate grades of polyox from different grades mentioned above . concentration of polymer and plasticizers were optimized by central composite design . in this design 2 factors the amount of polyox n750 ( x1 ) and peg 400 ( x2 ) were selected as independent variables . the tensile strength , folding endurance , % drug released at 10 min ( y10 ) and disintegration time were selected as dependent variables . different grades of polyox were used for film formation like n10 , n80 , n750 and n205 . initially , preliminary trials were carried out to select ideal grade of polymer used for film formation and then used for optimization of other parameters of film . different mentioned grades were tried between 1 - 4% concentrations . from the result , it was observed that by increasing the concentration of polymer up to 2% , thickness and strength of film was improved . but by increasing concentration more than 2% , folding endurance of film was improved but increase in disintegration time more than limit . thus , 2% concentration of polymer was used for further optimization of plasticiser and sweeteners . plasticizer tried were glycerin , peg 400 , and propylene glycol in 15% concentration each . glycerin and propylene glycol showed more sticky film which was unable to detach from surface . polyox n750 was selected for further optimization of film property due to its excellent film forming nature with optimum viscosity for rapid dissolving film . comparison of % elongation of design batches comparison of film thickness of design batches optimization of concentration of polyox n750 and % of peg as plasticisers was optimized systematically using with central composite design ( ccd ) . it was observed that disintegration time of all the batches ranges between 6 - 30 sec , tensile strength between 0.106 0.760 kg / cm , folding endurance between 10 - 89 and drug release profile with in 10 min 80%-99% . all the batches showed more than 80% drug release profile in initial 10 min . from all evaluation parameter , r10 batch with 2% polyox 750 and 15% of peg 400 was considered as promising batch with all satisfactory parameters . for validation of optimum formulation , check point r14 was prepared and evaluated for all the response . observed value was found close to the predicted value , which indicated good correlation of results . optimized film was analyzed by dsc spectra and compared with dsc spectra of pure drug . spectra showed presence of drug peak at same energy of enthalpy as that of pure qt . formulation was stored at 65% relative humidity and 37c temperature in the humidity oven . after 3 months film some predictable changes were observed in film property like decrease in disintegration time and more softness of film due to higher amount of moisture . film texture was evaluated after 3 months of storage which indicates increase in stickiness of film at higher relative humidity . the results of central composite design revealed that the entire factor significantly influences the dependent variable . thus , it can be concluded that by adopting a systematic formulation approaches , an optimum point can be reached in the shortest time with minimum effort . thus , developed film can be useful for curing the emergency condition like schizophrenia which gives rapid relief within short time . stability study data shows need of proper storage condition for film to protect from effect of moisture .

quick dissolving film prepared by various grades of polyox like polyox n10 , n80 , n750 and n205 . polyox having excellent film forming capacity with rapid hydration power which leads to rapid disintegration of film upon contact with saliva . film is optimized for concentration of polymer and plasticizer using ccd design . the tensile strength , folding endurance , % drug released at 10 min ( y10 ) and disintegration time were selected as dependent variables . the data revealed that 2% of polyox n 750 and 15% of peg 400 showed excellent film forming property with rapid drug release profile .

duplication of the alimentary tract is an important surgical condition , which includes a wide variety of mass lesions . we encountered a patient who presented with perforation of the duplication , which was associated with malrotation with midgut volvulus . being an extremely uncommon surgical emergency , it is being presented with a brief review of the relevant literature . a 4-year - old male child was referred to us for complaint of pain in abdomen . however , there was occasional history of abdominal pain , which was relieved by medication . on examination , his abdomen was distended , but it was non - tender . a computerized tomography ( ct ) scan of abdomen was advised for confirmation of the diagnosis . on next day , just after ct abdomen , the patient suddenly developed severe abdominal pain . after initial stabilization , the patient was explored under general anesthesia . on exploration , we noticed malrotation of the gut , which was associated with two turns of midgut volvulus . ladd 's bands were also present . the peritoneal cavity was grossly contaminated with fecal fluid . the perforation was at the mesenteric side , and it was associated with a soft mesenteric mass communicating with the normal bowel . the perforation was just at the junction of the mesenteric mass and the normal bowel [ figure 1 ] . the other thumb forceps is in the perforated duplication after performing the ladd 's procedure , attention was placed towards the perforation . on attempting to resect the mesenteric mass , we noticed two lumens in the bowel distal to the perforation site and single lumen proximal to that [ figures 2 and 3 ] . when an attempt to ascertain the length of the duplication was made by inserting two ryle 's tube through the lumens , it was reaching up to ileocecal region . as the general condition of the patient was poor , complete resection of the bowel was not done , and a double barrel ( triple barrel , to be more precise ) was fashioned . ( a ) duplication cyst , ( b ) junction of duplication and normal bowel , and it was the site of perforation , ( c ) normal ileum , ( d ) duplicated ileum reaching up to cecum , and ( e ) cecum and probable site of duplication termination in the postoperative period , there was wound infection with superficial wound dehiscence . the patient was discharged after 3 weeks with proper follow - up advise . during the follow - up these abnormalities include developmental obstructive defects of the small intestine , anomalies of rotation and fixation , intestinal duplications , etc . duplications of the alimentary tract are rare congenital malformations and may occur anywhere in the intestinal tract . the initial suspicion , on the basis of usg , was of some cystic mass lesion compressing the upper small bowel . the ct plates , reviewed later on , also showed twisting of mesenteric vessels suggestive of malrotation . probably the perforation of the duplication was not spontaneous ; it may be because of the volvulus and resultant ischemia of the wall . as malrotation with midgut volvulus is always a surgical emergency , and perforated duplication is also a surgical emergency , a combination of the two entities is definitely a surgical emergency , which needs immediate intervention . the ideal treatment would have been a ladd 's procedure , along with resection of the complete duplication . this was , however , not possible due to the poor general condition of the patient and large length of the duplication , which may had led to extensive resection of the bowel . the association of duplication of small bowel and malrotation , though described in the literature , is a rare one . . to conclude , this being one of a very rare combination of two pediatric surgical emergencies ; needs proper surgical and postoperative management . the exact preoperative diagnosis may be difficult , but a high degree of suspicion is warranted .

duplication of the alimentary tract is an important surgical condition . it may occur anywhere in the gastrointestinal tract . an important complication of this entity is perforation of the normal or abnormal gut . malrotation with midgut volvulus can be a surgical emergency . we present a patient , who presented as malrotation with midgut volvulus associated with perforated ileal duplication . the patient was successfully managed .

a 49-year - old man with a history of sick sinus syndrome presented with quadriplegia and ophthalmoplegia . brain magnetic resonance imaging ( mri ) and an angiography examination , which were performed a day after quadriplegia , revealed acute brain infarction in the bilateral midbrain , right median thalamus , and superior cerebellum associated with the distal basilar artery occlusion . it is also unclear whether the mechanism of symptomatic bruxism is identical with that of idiopathic sleep bruxism . it has been shown that rhythmic jaw movement is generated by a neuronal activation in the brainstem of animals.7 rhythmic firing of interneurons in the pontine reticular formation ( prf ) and a connection between the oral motor nuclei and the prf have been observed.7 - 10 rodents move their jaws continuously and cows ruminate throughout the day . however , the activity of the generator is so weak that the release from high cortical inhibition during normal sleep may not be sufficient to cause bruxism . thus , the release phenomenon due to sleep and other factors such as dental problems , smoking , alcohol , and the coma state6 may cause bruxism . in our case , bilateral midbrain infarction may have caused the interruption of higher cortical inhibition and damaged the dentato - rubro - olivary pathway . although higher cortical inhibition is interrupted , the brainstem generator may not be sufficient to cause bruxism . additional damage to the dentato - rubro - olivary pathway may cause bilateral olivary hypertrophy , which can stimulate the prf . because no connection between the oral motor nuclei and the inferior olivary nuclei has been observed in animal study , we do not think that the hypertrophied olivary nucleus directly stimulates the motor nucleus in the brainstem.8 instead , hyperactive bilateral prfs may cause bruxism . pollack and cwik have reported bruxism after cerebellar hemorrhage,5 which may also be related to the damage to the dentato - rubro - olivary pathway . unlike palatal myoclonus , bruxism requires bilateral harmonic rhythmic contraction and relaxation of involved muscles . since the neurons in the prf project to both the trigeminal motor nuclei,8 the prf may induce the contraction of one side and relaxation of the opposite side . on the other hand , the normal side can influence both we suspect that this is a reason why bruxism is very rare in patients with brainstem infraction . in conclusion , furthermore , we suggest that the human bruxism generator may be located in the prf . large bilateral midbrain lesions that interrupt cortical inhibition seem to produce bilateral olivary hypertrophy , resulting in bruxism in the presence of the intact pontine generator .

delayed - onset continuous bruxism due to brain stem infarction has not yet been reported . a 49-year old man presented with quadriplegia and ophthalmoplegia . brain mri showed acute infarction in the bilateral midbrain , right thalamus and the superior cerebellum . one month later , the patient developed bruxism which persisted during sleep . a palatal myoclonus was not observed . follow up mri taken 4 months later showed bilateral olivary hypertrophy . we suggest that the patient 's bruxism may be related to the olivary hypertrophy . the bruxism generator may be located in the pontine - reticular - formation ( prf ) . bilateral large midbrain lesions interrupting the cortical inhibition may have produced bilateral olivary hypertrophy , which could stimulate the prf , producing continuous bruxism .

non - gestational choriocarcinomas are rarely seen outside the gonads , with the mediastinum being the main site for these non - gestational extragonadal choriocarcinomas . there is no consensus regarding the use of systemic chemotherapy [ 1 , 2 ] . studies undertaken on treatment for the metastatic disease indicate that chemotherapies based on cisplatin , methotrexate or 5-fu are not efficient [ 1 , 2 ] . there is no metastatic gastric choriocarcinoma case in the literature that showed a complete response to chemotherapy . herein , we present a human epidermal growth factor receptor-2 ( her2)-positive gastric adenocarcinoma case which has a choriocarcinomatous component and showed a complete response to trastuzumab chemotherapy . a 57-year - old female patient was admitted for weakness , lack of appetite , weight loss and abdominal pain . the patient did not have a disease history and had not been under any medical treatment . on physical examination the mucosa was pale and no abdominal mass was detected . laboratory analysis revealed hypochromic , microcytic anemia ( hb : 9 g / dl ) . upper endoscopic examination revealed a mass in the gastric antrum , which was reported to be adenocarcinoma by biopsy . postoperative pathological analysis revealed the presence of adenocarcinoma associated with choriocarcinoma ; histologically , intestinal type adenocarcinoma composed of columnar gland - forming cells interspersed with cytotrophoblast and syncytiotrophoblast cells staining positive for beta - human chorionic gonadotropin ( -hcg ) on immunohistochemistry ( fig . 1 , fig . 2 ) . the patient 's serum -hcg level , which was measured in light of these pathological findings , was 22.823 iu / ml . multiple liver metastases and a metastasis in the right iliac bone were detected on pet - ct imaging for tumor staging . the patient was given two cycles of dcf ( docetaxel , cisplatin , 5-fu ) as treatment . however , the patient did not tolerate the treatment due to the development of grade 3 diarrhea and mucositis . therefore , the dcf treatment was replaced with a combination of docetaxel , carboplatin and trastuzumab . -hcg level had decreased to 6 iu / ml , and pet - ct scans showed a complete response ( fig . the patient is currently on the 24th month of the treatment and the complete response is still ongoing . the most widely accepted theory holds that the trophoblastic cells in gastric choriocarcinoma develop from dedifferentiation of a poorly differentiated adenocarcinoma . in fact , cytogenetic studies show that gastric choriocarcinoma genetically possesses characteristics of both adenocarcinoma and choriocarcinoma . in our patient choriocarcinoma accompanied adenocarcinoma and therefore , pathological analysis of the biopsy sample by an experienced pathologist increases the chance of establishing a diagnosis . consecutive measurements of serum -hcg levels in the postoperative or post - chemotherapy periods are valuable for the assessment of treatment response or recurrence of the disease . the most common metastasis sites are lymph nodes ( 87% ) , followed by liver ( 45% ) , peritoneum ( 23% ) and lungs ( 8% ) . gastric choriocarcinomas are highly malignant and most patients die within a year of diagnosis . although more than 140 gastric choriocarcinoma cases have been reported in the literature until now , there is no consensus concerning the treatment strategy for the disease . in addition to surgery and radiotherapy , various cytotoxic drugs have been used for the treatment . however , contrary to gestational choriocarcinomas , all chemotherapy regimens were found to be ineffective in metastatic gastric choriocarcinoma . a choriocarcinoma case who received trastuzumab therapy was not found in the literature . in gastric cancers however , her2-positive gastric cancer patients benefit significantly from trastuzumab therapy . when compared with normal placenta or her2 expression furthermore , choriocarcinoma components showed significantly higher her2/neu expression compared to other histological subtypes in testicular germ cell tumors ( p = 0.0095 ) . these data suggest that cerbb2 oncoprotein may be important in the pathogenesis of choriocarcinoma , but there is no information concerning the use of trastuzumab for the treatment . overexpression was present in our case and complete response was obtained after adding trastuzumab to the treatment . in order to establish a preoperative diagnosis for gastric choriocarcinoma , it is important to bear this tumor in mind and adopt a skeptical approach . most patients have metastases at the time of establishing a diagnosis and the tumor has a significantly poor prognosis . determining her2 expression in every patient and using trastuzumab treatment in positive cases may lead to positive therapeutic outcomes , as in our case .

gastric choriocarcinoma is a rare neoplasm and usually accompanies gastric adenocarcinoma . the prognosis is poor due to the aggressive course of the disease . a 57-year - old female patient with weight loss and abdominal pain was examined . the patient was operated following the examination , and pathological analysis revealed the presence of a gastric adenocarcinoma associated with choriocarcinoma . immunohistochemical analysis showed a positive reaction with antibodies to beta - human chorionic gonadotropin and overexpression of the cerbb2 proto - oncogene . staging revealed multiple metastases in the liver . a complete response was obtained with a combination of trastuzumab and chemotherapy . the diagnosis of gastric choriocarcinomas without pathological examination is difficult due to their rare occurrence . a complete response can be obtained with trastuzumab in the treatment of cases with overexpression of the cerbb2 protein .

totally subcutaneous intravascular portals ( port - a - cath ) are frequently used to administer chemotherapeutic agents . we present a case of port - a - cath fracture with distal embolization causing non - sustained ventricular tachycardia and its percutaneous retrieval . a 68-year old male with diabetes , hypertension and dyslipidemia controlled by medication and metastatic colon cancer to the liver was admitted to hospital for a non - flushing port - a - cath which had been inserted six years earlier to receive chemotherapy . upon questioning , his physical examination revealed blood pressure 142/64 , pulse 64 , body temperature 37c , and an arterial oxygen saturation of 98% . this demonstrated a catheter fracture at the first rib with extravasation of contrast at the site of insertion with the distal catheter fragment traversing the right atrium and the right ventricle with the tip within the right ventricular apex ( fig . 1 ) . a linogram demonstrating catheter fracture at the medial border of the first rib with extravasation of contrast material ( single arrow ) . the proximal portion of the catheter is located in the right atrium crossing into the right ventricle ( double arrow ) . injection of contrast into the port - a - cath caused left shoulder pain . during hospitalization , cardiac telemetry demonstrated non - sustained runs of both supraventricular as well as ventricular tachycardia ( fig . the morphology changes as the catheter embolus moves through the right heart . given the ventricular arrhythmia , a decision was made to retrieve the catheter fragment . the patient was transferred to our catheterization laboratory where repeat fluoroscopy showed the distal catheter had embolized further into the left main pulmonary artery ( fig . subsequent cardiac catheterization demonstrating the catheter had migrated into the left pulmonary artery ( a ) . a gooseneck snare was placed over the distal end of the catheter embolus ( b ) and maneuvered through the right ventricle , inferior vena cava and femoral vein ( c ) where it was extracted . a right femoral vein approach was used and a 6 fr multipurpose catheter was placed into the pulmonary artery via a 7fr femoral introducer sheath . through this , a 0.014 - 180 cm grand slam coronary wire ( abbott vascular , abbot park , il , usa ) with a cinch was advanced and the multipurpose catheter removed . the port - a - cath embolus was snared with a 25 mm 6 fr . amplatz gooseneck snare kit ( covidien , plymoth , mn , usa ) and withdrawn out of the pulmonary artery , into the right ventricle , then the inferior vena cava , and removed through the femoral sheath ( fig . 3d ) . a follow - upechocardiogram demonstrated no evidence of pulmonary hemorrhage or cardiac trauma . port - a - caths are widely used in the field of oncology . catheter fracture and embolization a case series of 333 port - a - cath insertions showed that catheter fractures and distal embolization occurred in 5 cases ( 1.5% ) ( 1 ) . aitkins et al . described a possible mechanism for subclavian port - a - cath fracture where compression between the clavicle and the first rib causes catheter fracture ( 2 ) . they describe the pinch off sign where the caliber of the catheter narrows as it passes over the first rib . the most common presentation of catheter embolization is asymptomatic discovery on chest radiology , with other presentations including infra / supraclavicular swelling ( 3 ) . to our knowledge , there has been only one previous description of ventricular tachycardia caused by port - a - cath fracture and embolization ( 3 ) . in that case , there was an increase in ectopy when the patient changed position . in our case , the patient had an unusual presentation of palpitations and ventricular tachycardia , but given his malfunctioning port - a - cath , further imaging allowed the cause of the arrhythmia to be diagnosed . prompt removal of catheter fragments is preferred due to potential complications including pulmonary thromboembolism , cardiac perforation , cardiac arrest and endocarditis ( 4 ) . a previous case series of 20 patients with asymptomatic catheter embolization and per cutaneous retrieval demonstrated successful retrieval in all cases : 16 with a snare and 4 using a basket retrieval system ( 5 ) . interestingly , 2 cases had thrombus formation in the distal catheter similar to our case . in summary , port - a - cath fracture and distal embolization has been described in the literature . our case describes a rare yet potentially catastrophic complication of catheter embolization , i.e. symptomatic cardiac arrhythmia and in situ thrombosis with a catheter fragment in the pulmonary artery . given the subtle nature of the embolization and the potential complications , clinicians should be vigilant for catheter fracture and embolization . percutaneous removal has been shown to be a safe procedure , allowing for prompt removal of the embolized catheter fragments .

we describe the case of a patient with a previously placed port - a - cath who was admitted to hospital for new onset of non - flushing catheter and palpitations with ventricular tachycardia . a chest x - ray and a linogram showed a port - a - cath fracture and distal embolization into the right ventricle resulting in ventricular tachycardia . the catheter was removed percutaneously using a goose neck snare with no complications and resolution of the ventricular tachycardia . the removed segment demonstrated thrombus . prompt removal of the embolized catheter fragments should be undertaken given the subtle nature of the embolization and the potential complications .

pulmonary tumor thrombotic microangiopathy ( pttm ) also known as carcinomatosis endarteritis is a highly fatal respiratory complication seen in cases of malignancies especially adenocarcinomas of the stomach . there are only around 80 cases reported in literature so far including less than 10 cases where the primary malignancy has not been detected even after extensive search . we present an autopsy case of a 47-year - old male patient who developed acute breathlessness and died suddenly . at autopsy there was mild right ventricular dilatation and lungs showed few firm areas and prominent bronchovasculature . on microscopy , the lungs showed the presence of florid fibrocellular intimal proliferation of the arterioles and small arteries [ figure 1 ] . vessels showed fibrin thrombi and occasional vessel showed tumor emboli composed of neoplastic adenocarcinoma cells showing glandular and occasional signet ring morphology . there were similar tumor emboli in the myocardium , peripancreatic tissue , and perigastric lymph nodes [ figure 3 ] . despite extensive sectioning no primary tumor tumor cells were negative for ck 7 , ck10 , ttf 1 , and psa . the final cause of death was given as acute cor pulmonale following pulmonary tumor thrombotic microangiopathy in an unknown primary adenocarcinoma . lungs showing presence of florid fibrocellular intimal proliferation with fibrin thrombi of the arterioles and small arteries ( h and e , 100 ) lungs showing presence of lymphangiosis carcinomatosis ( h and e , 100 ) signet ring cells seen in the lymph nodes ( h and e , 400 ) pttm is characterized by intimal proliferation in pulmonary small arteries and arterioles with or without tumor emboli leading to vascular stenosis and pulmonary hypertension . there was florid fibrointimal proliferation , fibrin thrombi with some showing occasional tumor emboli and lymphangimatosis carcinomatosis . the tumor cells in this condition invade the pulmonary vascular system occluding the small arteries , arterioles and activate coagulation systems releasing inflammatory mediators , growth factors like serotonin , vascular endothelial growth factor and osteopontin . some authors have proposed screening with serum vegf and d - dimer testing for early detection of pttm . the primary sites of malignancy reported are gastrointestinal tract , ovary , breast , pancreas , liver , uterus , gall bladder , and prostate . however , rarely there are reported cases where primary has not been detected inspite of extensive search and immunohistochemical stains . in our case too despite an extensive search no primary mass was found . thus , our case belongs to the rare set of pttm with unknown primary of which only less than 10 cases have been reported . it should be distinguished from ordinary pulmonary thromboembolism and primary pulmonary hypertension and should be considered as a differential diagnosis in patients with acute respiratory symptoms especially in cases with an underlying cancer . there are only occasional case reports of antemortem diagnosis with patient recovering completely , and hence it is important to have a higher clinicopathological awareness of diagnosing this highly fatal condition . role of 2-(f-18)-fluoro-2-deoxy - d - glucose positron emission tomography ( fdg - pet ) has been described in literature and has been described to be helpful for the diagnosis of pttm .

pulmonary tumor thrombotic microangiopathy ( pttm ) is a highly fatal complication of cancer leading to acute cor pulmonale and pulmonary hypertension . we present a case of 47-year - old male patient who developed acute breathlessness and died suddenly . the pulmonary vessels at autopsy on histopathologic examination showed the presence of fibrocellular intimal proliferation , fibrin thrombi and few tumor emboli consisting of malignant adenocarcinoma cells . there was associated lymphangiosis carcinomatosis . no primary visceral tumor was found despite extensive search . the patient had died following acute cor pulmonale with sudden pulmonary hypertension due to pttm . this entity ( pttm ) must be kept as a differential diagnosis in patients presenting with acute breathlessness especially in cases of cancers .

teratoid wilms tumor , a rare variant of nephroblastoma , has a predominance of heterologous elements . to date less than 30 cases of teratoid wilms tumor have been reported in the literature.[13 ] there is only one case in the literature , which has the squamous epithelial component comprising 70% of the tumor . we describe a case in which the squamous epithelium with keratin cysts comprised majority ( about 75% ) of tumor mass . metastasis is seldom reported in teratoid wilms . a -2-year - old boy presented with palpable mass on the right side of abdomen for 2 months . physical examination revealed a firm mass that was palpable in the lumber region . contrast - enhanced computed tomography ( cect ) scan showed large hetrodense lesion in the right lumber region measuring 17 cm 8 cm 7 cm . the right lung showed nodular opacities in posterior and lateral basal segments of about 0.5 cm in diameter suggestive of metastasis . cect scan done after 6 weeks of chemotherapy showed little change in the size of tumor and the patient underwent nephrectomy . grossly , nephrectomy specimen measured 17 cm 8 cm 6 cm that was soft in consistency . the cut section was predominantly solid and pale grey with areas of cystic change , necrosis , and hemorrhage . a thin segment of normal kidney was seen compressed at periphery [ figure 1 ] . gross - predominantly cystic appearance of teratoid kidney microscopically , the tumor showed classical triphasic pattern with blastemal , epithelial , and mesenchymal components without anaplasia ; about 75% of the tumor revealed areas of squamous differentiation with keratin pearl formation but without adnexal structures [ figures 2 and 3 ] . there were also small foci of mature adipose tissue and glial tissue [ figure 4 ] . wilms tumor is an embryonic tumor typically composed of variable admixture of blastematous , epithelial and stromal components . wilms tumor can include heterologous elements in addition to these components and variend et al in 1984 , coined the term teratoid wilms tumor for them . . defined teratoid wilms as a tumor that contains hetrologous elements comprising more than 50% of the tumor mass . to date , less than 30 cases of teratoid wilms tumors have been reported.[13 ] the pathogenesis of this entity is still debated ; it is likely that it originates from totipotent primitive metanephric blastema . the variable presence of intracellular matrix proteins may influence the presence , extent , and diversity of hetrologous differentiation . the teratoid elements seen frequently are skeletal muscle , smooth muscle , adipose tissue , glial tissue , cartilage , bone , and are generally assumed to represent aberrant mesenchymal differentiation . there is only one case of teratoid wilms in the literature , reported by karaka i et al , which had squamous epithelial component comprising 70% of the tumour . we report a case that showed the familiar triphasic pattern of nephroblastoma along with the extensive squamous epithelial components constituting about 75% of the tumor . the differential diagnosis of teratoid wilms is intrarenal teratoma , metastatic germ cell tumor , and retroperitoneal infiltration of teratoma . in our case , classical triphasic pattern of wilms tumour was seen and there was no attempt to form the adnexal structures or organogenesis thus ruling out intrarenal teratoma . also , the presence of normal testis excluded the possibility of metastatic testicular germ cell tumor . renal capsule was also free of tumor so retroperitoneal infiltration of teratoma was also ruled out . although it is reported that teratoid wilms tumor is not usually aggressive or has metastatic potential . the case reported by karaka et al , which had extensive squamous areas , died of metastasis . whether the squamous differentiation is associated with higher stage at presentation remains to be seen . the treatment of teratoid wilms tumor has not yet been established because of its rarity and varying tumor components . both surgeons and pathologists should be aware that the treatment of this rare variant should be focused on total surgical removal of the tumor .

teratoid wilms tumor is an unusual histological variant of nephroblastoma with predominant heterologous component . frequently present components include adipose tissue , glial tissue , muscle , cartilage or bone . the presence of squamous epithelial component on the other hand is rarely reported . we describe a case of unilateral teratoid wilms tumor in a 2-year - old boy with lung metastasis . in this case , tumor showed the familiar triphasic histologic pattern of nephroblastoma along with extensive squamous epithelial component .

case one was 13 years old and had severe sudden onset low back pain with radiation to anterior aspect of right thigh during extension of leg . computerized tomography at l2-l3 level ( figure 1 ) showed a bony fragment similar to an osteophyte extending to canal . in lumbosacral mri we detected a l2-l3 disc bulging with impingement of l3 root and thecal sac compression ( figure 23 ) . the second case was 14 years old and had sudden onset low back pain with radiation to right lower extremity for one month . he had pain in territory of l5 root with intact muscle power and deep tendon reflexes . note the avulsed bony particle from apophyseal rim intervertebral disc herniation at level of l2-l3 in sagittal view mri ( axial view ) from l2-l3 inter vertebral disc lateral lumbosacral radiography showed a small bony extension into vertebral canal at l4-l5 intervertebral disc level ( figure 4 ) . he was disable and had no recovery after several medical and conservative management and physiotherapy . lateral lumbosacral x - ray .small bony particle adjust to posterior margin of l4 rim is seen in his lumbosacral mri , he had a herniated l4-l5 disc herniation with compression effect on the thecal sac and right l5 root ( figure 5 ) . mri(axial view ) from l4-l5 inter vertebral disc due to failed conservative management by other referral centers , we operated them in prone position . avulsed , hard and sharp particles of the posterior rim of vertebral body , including overlying cartilage of the annulus fibrosus similar to takata classification type - ii were seen . retropulsed bony fragment was removed after careful separation from peripheral tissue . then herniated intervertebral disc the calcification of the cartilaginous rim occurs around age 13 and fused with the vertebral body by the end of skeletal growth , i.e. by the age of 18 - 25 years.3791011 the development occurs at different rates in various spinal segments . intervertebral disc of immature spine is fastened to the ring apophysis by the outermost fibers of annulus fibrosis ( sharpey 's fibers).3 avulsion of lumber vertebral apophyseal rim is very rare and there are two possible mechanisms by which the fracture can occur . first , the force transmitted to sharpey 's fibers by annulus pulpous during herniation may cause disruption at the weak point of osteocartilagenous junction , thus resulting in an avulsion fracture . second , migration of the nucleus pulpous may occur through the weak point , similar to the mechanism which results in limbus vertebra.3710 apophyseal avulsion is a very rare lesion and accurate diagnosis is often delayed because of concerns for neoplasm , infection and spondylolisthesis.4111317 takata et al3 have classified these fractures in three types on basis of ct - scan studies : type i , a simple separation of the entire arcuate posterior margin of the vertebra , type ii , an avulsion fracture of the posterior rim of the vertebral body , including the overlying cartilage of the annulus fibrosus , resulting in a thicker and larger fragment and type iii , a more localized fracture involving a large amount of the vertebral body so that the resulting fragment is larger than the vertebral rim.317 according to takata classification , type i and type ii can be treated conservatively and type iii and iv require surgical intervention . epstein et al proposed an additional cate - gory : type iv , a fracture of both cephalad and caudal end plates , which spans the full length of the posterior margin of vertebral body.317 these fractures need to be accurately diagnosed compared to simple disc herniations , specially in children and adolescents.56711 these type of fractures require more suspension and knowledge for diagnosis and need more extensive exposure during laminectomy for better resection of pathology and decompression of nerve roots.313 so , unilateral laminatomy is not recommended in these patients because mobile and sharp retropulsed particle can displace cephalad or caudal and can damage neural structures . for early detection of epiphyseal fracture , computerized tomography is recommended to show bony component of herniated material.6912 computerized tomography scans can be a good diagnostic technique for early detection of epiphyseal slipping , so we recommend it . mrf : carried out the design , coordinated the study and participated in most of the experiments and prepared the manuscript .

avulsion or fracture of posterior ring apophysis of lumbar vertebra is an uncommon cause of radicular low back pain in pediatric age group , adolescents and athletes . this lesion is one of differential diagnosis of disc herniation . we reported two teenage boys with sever low back pain and sciatica during soccer play that ultimately treated with diagnosis of slipped vertebral apophysis .

capmul mcm was gifted by abitec corporation , usa . tween 20 and peg 400 were purchased from sulab chemicals , vadodara , india . all the other reagents were analytical grade . for the selection of components of microemulsion , solubility study was carried out in number of components according to procedure given by lianli et al . the oil , surfactant and cosurfactant were selected on the basis of solubility of drug in the solvent , hlb value as well as their gras status . to find out the microemulsion region , microemulsions having different composition [ table 1 ] were prepared by dissolving the required amount of drug ( 27 mg/ ml ) in a mixture of oil - smix at 405c and after cooling at 305c adding required volume of water . they were evaluated for globule size , pdi , zeta potential and % transmittance for optimization . for the selection of components of microemulsion , solubility study was carried out in number of components according to procedure given by lianli et al . the oil , surfactant and cosurfactant were selected on the basis of solubility of drug in the solvent , hlb value as well as their gras status . to find out the microemulsion region , microemulsions having different composition [ table 1 ] were prepared by dissolving the required amount of drug ( 27 mg/ ml ) in a mixture of oil - smix at 405c and after cooling at 305c adding required volume of water . they were evaluated for globule size , pdi , zeta potential and % transmittance for optimization . from the solubility data , capmul mcm , tween 20 and polyethylene glycol 400 were selected as oil , surfactant and cosurfactant component respectively as rosuvastatin is having higher solubility in above solvents . tween 20:peg 400 [ 3:1% w / w ] resulted in higher microemulsion region compared to [ 1:1% w / w ] and [ 2:1% w / w ] . this indicates that increasing the surfactant concentration , maximum amount of oil can be solubilized . pseudo - ternary phase diagram table 1 indicates the different batches of microemulsion and results of evaluation parameters . optimized batch was selected which is having lower globule size , zeta potential , pdi and higher % transmittance . the logic behind selecting these criteria for optimization is that lower globule size can result in enhanced permeation as well as provide larger surface area for drug release , pdi is the measure of uniformity of the formulation and less than 1 is desirable . more negative zeta potential is considered as more physical stability of the formulation and % transmittance was selected as higher as it shows the isotropic uniform system . from the results shown in table 1 , batch me-1 ( 5% capmul mcm , 50% smix ) was selected as optimized batch having globule size of 19.56 nm , -2.62 mv zeta potential , 0.334 pdi and 99.2% transmittance . from the solubility data , capmul mcm , tween 20 and polyethylene glycol 400 were selected as oil , surfactant and cosurfactant component respectively as rosuvastatin is having higher solubility in above solvents . tween 20:peg 400 [ 3:1% w / w ] resulted in higher microemulsion region compared to [ 1:1% w / w ] and [ 2:1% w / w ] . this indicates that increasing the surfactant concentration , maximum amount of oil can be solubilized . optimized batch was selected which is having lower globule size , zeta potential , pdi and higher % transmittance . the logic behind selecting these criteria for optimization is that lower globule size can result in enhanced permeation as well as provide larger surface area for drug release , pdi is the measure of uniformity of the formulation and less than 1 is desirable . more negative zeta potential is considered as more physical stability of the formulation and % transmittance was selected as higher as it shows the isotropic uniform system . from the results shown in table 1 , batch me-1 ( 5% capmul mcm , 50% smix ) was selected as optimized batch having globule size of 19.56 nm , -2.62 mv zeta potential , 0.334 pdi and 99.2% transmittance . present study demonstrates the potential use of microemulsion system for enhancement in solubility and hence bioavailability for the poorly water soluble drug .

due to very less bioavailability ( 20% ) of rosuvastatin calcium , an attempt was made to develop and optimize microemulsion formulation . capmul mcm , tween 20 and peg 400 were selected as oil , surfactant and cosurfactant respectively as the drug is having higher solubility in them . 3:1% w / w s : cos was selected as it gave higher microemulsion area . optimized batch ( me-1 ) was selected having 5% capmul mcm , 50% tween 20:peg 400 and 45% water based on evaluation parameters globule size , zeta potential , pdi , % transmittance .

a 35-year - old healthy lady presented with high grade fever , severe abdominal pain , nausea , vomiting and profuse watery diarrhea , sometimes green in color . there was no history of animal contact , recent travel or camping . on exam , the patient was hypotensive and was looking acutely ill . she also had hypokalemia of 3.3 mmol / l , acute kidney injury with elevated creatinine of 1.6 mg / dl from a baseline of 0.6 secondary to dehydration . murphy 's sign was positive which prompted us to do a right upper quadrant ultrasound which showed thickened gall bladder wall of upto 1 cm consistent with cholecystitis . patient started feeling better after the surgery and was discharged home . during the post - hospitalization follow - up after 2 weeks campylobacter is a small , slender , gram - negative curved rod , which is one of the most common causes of enteritis in humans . campylobacter fetus may have some attraction towards the gallbladder as in a survey , 20% of slaughtered 700 cattle and sheep harbored this bug in their gallbladder.1 campylobacter can cause cholecystitis without diarrhea unlike the case that we report here . the diagnosis of campylobacter cholecystitis is usually missed because culture of campylobacter is not routinely requested after cholecystectomy . however , even if the bile is cultured , campylobacter appears to be a less common cause of cholecystitis . darling et al cultured about 280 bile samples post cholecystectomy for campylobacter . but none of them grew campylobacter.2 hence routine ordering of bile culture under microaerophilic condition is not recommended unless the gram stain shows gram negative curved rods . 3 resistance of campylobacter fetus to cephalosporins and penicillins was reported as early as 1986.4 majority of the reported cases including our patient had good outcome with cholecystectomy and antibiotics especially erythromycin ( see table 1 ) . only one of the reported cases died , however she had advanced hepatocellular carcinoma.3 there is one case report of relapse of campylobacter bacteremia in a aids patient in about 8 months after the first episode of campylobacter cholecystitis.1 in conclusion , campylobacter cholecystitis is rare but should be kept in the back of the mind while treating a patient with campylobacter enteritis .

there are 13 cases of campylobacter cholecystitis reported so far in the medical literature . among them , only 4 patients had diarrhea . we report another case of acalculous cholecystitis in a setting of campylobacter enteritis . the case report is followed by a literature review regarding this rare condition .

the aim of this report is to describe a single case of vitiligo induced by carbamazepine . the case was a patient with bipolar i disorder whose medications were changed from valproate to carbamazepine and who developed vitiligo after a short while . when depigmentation occurred , we immediately discontinued carbamazepine after which the depigmented areas improved gradually . about three years later , he received carbamazepine again , but depigmentation did not recur . carbamazepine - induced vitiligo is not an absolute contraindication for the prescription of carbamazepine if other choices fail to respond or are not tolerated . the patient was a 34 -year -old male with a diagnosis of bipolar i disorder who was admitted to iran psychiatric hospital with a manic episode with psychotic features in september 2007 , and this was his first manic episode . he received sodium valproate as well as antipsychotic medications for the active phase treatment and was stabilized on 600 mg of valproate . in the post - discharge follow up , the tremor was mainly of a postural type and was so severe that made eating , drinking and shaving very difficult for the patient . propranolol was added and titrated up to 120 mg , but was not successful in controlling the tremor . therefore , valproate was tapered and replaced with carbamazepine to a total dose of 800 mg / d . nearly one month later , areas of depigmentation appeared on the patient s face ( figure 1 ) . although depigmentation decreased gradually in the next two months , it could still be observed in an attenuated form in a close inspection . then , lithium was tapered to discontinuation and a combination of lamotrigine ( up to 100 mg / d ) and olanzapine ( 10 mg / d ) was the next treatment choice . despite a mild tremor , the patient remained well for the following 18 months when he was admitted for one week in march 2011 for an episode of methamphetamine- induced psychotic disorder . after discharge , the patient was visited by a physician unaware of his history of vitiligo who decided to change the medications from lamotrigine and olanzapine to carbamazepine and aripiprazole . it seemed that the decision had been made to decrease the side effects of the drugs ( weakness , dryness of mouth , and tremor ) . the patient continued to improve and vitiligo did not recur ( or was not aggravated ) after re - challenging the patient with carbamazepine for the following 6 months . currently , the patient is receiving carbamazepine ( 800 mg / d ) and aripiprazole ( 20 mg / d ) and remains in remission and is trying to get a job ( figure 2 ) . few case reports of medication - induced vitiligo have reported the following mechanisms for this condition : activation of t cd8 + cells against melanocytes , apoptosis of melanocytes as a direct effect of the drugs , and damage to sympathetic nerves(3 ) . although a single case can not prove or rule out any of the proposed mechanisms , reviewing the history of this case could be informative . the reversibility of vitiligo in this case shows that the involved mechanism should not be a totally irreversible cellular change like apoptosis of melanocytes or permanent damage in sympathetic nerves at least in the early stages of medication - induced vitiligo . on the other hand , non - recurrence of vitiligo with re - challenge of carbamazepine suggests that some kind of immunity or desensitization has developed . the latter point is against the direct cytotoxic effects of medications on induction of vitiligo . we would like to emphasize that the patient also used other medications when the skin condition appeared . therefore , it is theoretically possible that these medications ( such as propranolol or antipsychotic drugs ) would be related to vitiligo . however , the temporal relationship of vitiligo appearance and the used medication suggests that carbamazepine is the most probable culprit for inducing vitiligo . the other limitation of the current report is that we did not use additional methods to confirm the diagnosis , like skin biopsy or wood s lamp examination . because the patient did not show any other signs or symptoms of other autoimmune diseases finally , we would like to suggest that the appearance of carbamazepine - induced vitiligo is not an absolute contraindication for the prescription of carbamazepine if other choices fail to respond or are not tolerated .

objectivevitiligo is a rare side effect of carbamazepine whose exact mechanism is unknown . the aim of this report is to describe a single case of vitiligo induced by carbamazepine.methodsthe case was a patient with bipolar i disorder whose medications were changed from valproate to carbamazepine and who developed vitiligo after a short while . we followed the case for about four years when he was rechallenged with carbamazepine.resultswhen depigmentation occurred , we immediately discontinued carbamazepine after which the depigmented areas improved gradually . about three years later , he received carbamazepine again , but depigmentation did not recur.conclusioncarbamazepine-induced vitiligo is not an absolute contraindication for the prescription of carbamazepine if other choices fail to respond or are not tolerated . the case has implications for the mechanism of medication induced vitiligo .

burning mouth syndrome ( bms ) is a burning or itching sensation in the normal oral mucosa . the pain is persistent , ranges from moderate to severe , and occurs particularly often in postmenopausal women . the international association for the study of pain defines bms , which is also called stomatodynia , glossodynia , oral dysesthesia , and persistent idiopathic orofacial pain , as any form of burning or stinging sensation in the mouth in association with a normal mucosa in the absence of local or systemic disease.1 in the literature , antidepressants and benzodiazepines have shown beneficial effects in the treatment of patients with bms.2,3 however , there has been no report on treatment with a dopamine partial agonist for bms . herein , we report a case of treatment - resistant bms successfully treated with low - dose aripiprazole . a 66-year - old female with no systemic disease or trauma history visited our clinic after being referred by her family physician . she complained of a chronic burning sensation in her tongue without taste alteration and dry mouth , which had lasted for 13 months . at first , she went to her family physician and was evaluated by laboratory screening tests . though antibiotics , topical dexamethasone , and kampo were administered at various clinics , the burning sensation was not ameliorated . based on the medication effects , a candidal infection was excluded . at the first examination , she came to our clinic by herself . no orientation disturbance or any verbal fluency problem was observed . no abnormality associated with the burning sensation based on the clinical , laboratory , and anamnestic data , we established a diagnosis of bms . because she was anxious about several side effects of antidepressants , she was started on escitalopram at 5 mg / d . however , the burning sensation was not reduced , even at 10 mg / d . therefore , duloxetine was administered instead of escitalopram . though duloxetine was initiated at 20 mg / d and gradually increased to 40 mg / d , her burning sensation was aggravated . the burning sensation was partially reduced at 7.5 mg / d , but dose escalation was not effective . therefore , low - dose aripiprazole powder ( 1.0 mg / d ) was added to her treatment regimen . a month later , she said that she almost always forgot about the burning sensation during the day . six months later , the mirtazapine was stopped and there was no change for the worse . two months later , the aripiprazole was also stopped , which resulted in recurrence of the burning sensation . aripiprazole was restarted at 1.0 mg / d , and the burning sensation disappeared within a few days . with low - dose aripiprazole , she continued to live a healthy life without experiencing any side effect . we report a case of bms that barely responded to antidepressants and was successfully treated with aripiprazole . low - dose antidepressants , anticonvulsants , and benzodiazepines have been investigated and are the most accepted options for bms . tricyclic antidepressants are the most commonly prescribed drugs for bms.4 additionally , it has been reported that serotonin norepinephrine reuptake inhibitor5,6 and selective serotonin reuptake inhibitor7 are effective recently , the effectiveness of atypical antipsychotics was reported in these patients.8,9 to our knowledge , there is no report on the effectiveness of aripiprazole for bms . aripiprazole is an atypical antipsychotic that bears the properties of d2 and 5-ht1a partial agonists and is a potent antagonist of the 5-ht2a receptor.10 unlike other atypical antipsychotics that are dopamine d2 antagonists , aripiprazole has a unique effect on the dopamine system . because of its d2 partial agonist activity , some studies have mentioned that aripiprazole has a role as a dopamine system stabilizer . recently , neurotransmitter positron emission tomography data indicated that hypofunction of the dopaminergic system within the basal ganglia was related to bms.11 one possible explanation is that the efficacy of aripiprazole in treating bms is partly caused by stabilization of these dopamine receptors within the basal ganglia . especially as this case did not have any response to a selective serotonin reuptake inhibitor ( escitalopram ) or a serotonin norepinephrine reuptake inhibitor ( duloxetine ) , it is strongly suggested that the efficacy of aripiprazole is caused by dopamine stabilization . considering this dopaminergic hypothesis , other treatments including other antipsychotic drugs and electroconvulsive therapy might be options of treatment for bms . recently , the role played by the dopamine system of the brain in pain control has garnered attention . kasahara et al12 reported four cases of refractory chronic pain that improved with a low dose of aripiprazole . in their paper it is also believed that the dopamine and opioid systems interact in complex ways in the treatment of chronic pain . this report suggests that low - dose aripiprazole may be effective for patients with treatment - resistant bms .

we report a case of refractory burning mouth syndrome ( bms ) ameliorated with low dose of aripiprazole . the patient was a 66-year - old female who had suffered from chronic burning pain in her tongue for 13 months . no abnormality associated with the burning sensation was detected in the laboratory tests and the oral findings . considering the clinical feature and the history together , we diagnosed the burning sensation as bms . the bms pain was decreased by aripiprazole ( powder ) 1.0 mg / d , though no other antidepressants had satisfying pain relief . it could be supposed that the efficacy of aripiprazole is caused by dopamine stabilization in this case , and bms might have a subtype that is reactive to aripiprazole . further studies are needed to confirm the efficacy of aripiprazole for bms .

although uterine rotation due to a myoma is a rare complication of pregnancy 1 , obstetricians should be alerted to avoid making an incorrect uterine incision during cesarean section . a 35-year - old woman ( gravida 1 , para 0 ) was referred to our hospital for delivery because her pregnancy was complicated by a left - sided myoma that measured 15 cm in diameter during ultrasonography . the patient 's medical history included renal artery aneurysm , primary aldosteronism , and chronic hypertension . to determine the anatomical relationship between the uterus and the myoma , we performed magnetic resonance imaging ( mri ) at 30 weeks ' gestation ( fig.1a ) . mri revealed that the myoma was intramural and located on the left side . notwithstanding the myoma , the pregnancy had not been complicated by abdominal pain , placental abruption , fetal growth restriction , or preterm labor and birth . fetal growth was determined to be appropriate for the gestational age , and no major anomalies were revealed by ultrasound . because of breech presentation , the patient was scheduled to undergo an elective cesarean section . owing to her chronic hypertension , the cesarean section was scheduled for 37 weeks ' gestation . ( a ) magnetic resonance imaging revealed an intramural myoma on the left side ( its greatest dimension is not shown in this figure ) ; however , imaging did not reveal the degree of uterine rotation . ( b ) midline laparotomy showed multiple large vessels pressing on the uterus . from the first abdominal incision prior to delivery , the obstetrician was unable to accurately understand the anatomical relationship between the myoma and the intra - abdominally placed uterus . therefore , the abdominal incision was extended vertically around the umbilicus , and the uterus was brought outside the abdominal cavity ( fig.2a ) . as a result , we were able to evaluate the spatial relationship between the tumor and the uterus . we then located the vessels belonging to the utero - ovarian ligament and the uterine parametrium ; they were facing front because the myoma had rotated the uterus 90. a vertical uterine incision was made to open the uterus ( fig.2b ) . intraoperative bleeding was 1600 ml . we managed the section successfully , and the patient delivered a healthy male infant , weighing 2538 g with apgar scores of 4 and 9 at 1 and 5 min , respectively . the neonate had no feeding - related complications ; he gained sufficient weight and was discharged with his mother . six months after delivery , the patient 's myoma had decreased to approximately 11 cm in size . ( a ) by bringing the uterus outside the abdominal cavity during the cesarean section , we were able to determine that the uterus was rotated 90 by the myoma , and the right side of the uterus was anteriorly directed . in this figure , the rotation has been corrected ; the white arrow denotes the area shown in figure1b . ( b ) because a low transverse uterine incision was not feasible , a vertical uterine incision was made . at the time of incision , we monitored the surgical margin between the incision and the myoma to ensure that the myoma was maintained in order to prevent hemorrhage . the distance between the incision and myoma usually shortens because of postdelivery uterine contraction . in summary , if the obstetrician has difficulty in confidently identifying the anatomical landmarks required to properly orient the uterine incision during a cesarean section , he or she should bring the patient 's uterus outside the abdomen . if an ill - placed uterine incision is made to deliver the child , massive intraoperative hemorrhaging can result . the authors declare that they have no conflicts of interest or funding disclosures related to this study .

key clinical messagein pregnancies complicated by large myomas , obstetricians may face difficulties during cesarean section if they fail to notice the rotation of the uterus and could make an incorrect uterine incision . this error might cause massive intraoperative hemorrhage . obstetricians must exercise extreme caution during cesarean sections complicated by large myomas .

the treatment of complicated crown - root fractures in many cases is compromised by tooth fractures that are well below the gingival margin or bone . after root canal obturation , proper isolation for a dry operation field is critical for successful restoration of traumatized teeth . in this respect , a wide range of treatment options have been advocated for traumatic permanent teeth include ; orthodontic extrusion1 osteotomy / osteoplasty2 intentional replantation3 re - attachment of fragments4 re - attachment of a tooth fragment should be preferable to restoring fractured teeth . there are several advantages in this treatment such as obtaining esthetic in a single appointment , being more conservative procedure , obtaining healthy periodontal attachment and it maintains the original tooth contours and translucence as the patient s own.5,6 the present case report describes the re - attachment of an original tooth fragment using a glass fiber post . a 32-year - old male patient was referred to the endodontic clinic of the faculty of dentistry , s. demirel university , with the complaint of a fractured right maxillary incisor tooth because of a trauma . the patient related pain during breathing and bleeding through the line of fracture ( figure 1 ) . the clinical and radiographic examinations showed an oblique crown - root fracture in the maxillary right central incisor involved the enamel root canal pulp extirpation and canal preparation was performed using the standard step - back method . the prepared teeth were dried with paper - points ( dentsply maillefer , tulsa , ok ) and filled with laterally condensed gutta - percha ( diadent group international inc . , chongju , korea ) and sealapex ( kerr , manufacturing co. , romulus , mi ) root canal sealer . after sealer set , the gutta - percha was partially removed from the root canal using heated instrument , leaving 5 mm of the filling material at the apex to maintain a good sealing . a post hole within root and coronal fragment was prepared using a drill recommended by manufacturer ( carbotech , ganges , france ) . a glass fiber post ( snowpost , carbotech , ganges , france ) was cemented within root canal with dual adhesive cement ( variolink ii , ivoclar vivadent , schaan , liechtenstein ) . after taking his consent , full thickness buccolingual mucoperiostal flap was raised with an intrasulcular incision ( figure 3 ) . after all debris on the fractured root surface was scaled , washed away with sterile serum physiologic and dried . a primer ( kuraray co. ltd , osaka , japan ) was applied onto the fractured surfaces of the tooth fragments for 20 second . clearfil se ( kuraray co. ltd , osaka , japan ) bonding agent was applied to the primed surfaces of the fragments and light cured for 10 second . the fragments were reattached with a composite resin ( clearfil ap - x , kuraray co. ltd , osaka , japan ) . the excess resin was removed with an excavator and the crown was light cured for 40 seconds from both buccal and palatal aspects . final polishing of the crown - root interface was made with ultrafine diamond burs and polishing disks . one week later , the sutures were removed and clinical examination was revealed proper healing ( figure 5 ) . one month later , the clinical and radiographic examinations revealed a stable reattachment of the crown fragment ( figure 6 ) . after 12 months , clinical and radiographic examinations showed a stable reattachment of the fragments , good aesthetics and periodontal health ( figures 7 and 8) . the present case report described that tooth fragment reattachment is an alternative to composite resin build - up for restoring esthetics and function of oblique fractured teeth . up to date , a lot of different approaches were proposed for treatment of fractured teeth depending on location of the fracture.7 one of the options for managing coronal tooth fractures is the reattachment of the dental fragment.4 re - attachment of fragments offers a good esthetic , less time - consuming and cost - effective restorative option . contemporary resin adhesives and resin post systems that allow strong durable bonds to dentin enhance this option.8,9 in this case , a glass fiber post was used to retain the coronal segment and reduce the stress on the luting material . the post interlocks the two separate fragments and minimizes the stress on the remaining tooth structure.7,10 the re - attachment of a tooth fragment is a viable technique that restores function and esthetics with a very conservative approach , but for each trauma case should be attempted to restore on an individual basis .

this case report presents a 32 year old male with an oblique fractured maxillary right central incisor . the procedure used to repair the fracture included flap surgery with an intrasulcular incision and endodontic treatment . the root canal was filled with a root canal sealer and gutta - percha . after root canal obturation , fragments were luted with composite resin using a glass fibers post . after 12 months , clinical and radiographic examinations showed a stable reattachment of the fragments , good aesthetics and periodontal health . ( eur j dent 2008;2:138141 )

advances in biological and medical research and their translation into diagnostic , prognostic and therapeutic tools are relying increasingly on partnerships between the academic ( government and university ) and industrial ( biotechnology , pharmacy and technology ) sectors , with essential participation and support from patient organizations and charities . despite these concerted efforts and the promises of genomics and systems biology , over the past two decades the cost of research and development has continuously escalated , while the number of truly novel drugs coming to market has constantly declined . to a large extent , this has been addressed in the private sector through mergers and outsourcing , with downsizing of the research and development workforce . these trends can not be sustained further as they threaten the economic viability of the healthcare system worldwide . at a time of a global crisis , it is crucial to identify how we can overcome these hurdles . i argue here that we should look again at how knowledge can be shared between all the stakeholders , redefining the frontier between what can be the subject matter of valuable intellectual property rights and what is the basic knowledge that should be made freely available to all . it was hotly debated at the beginning of the human genome project ( hgp ) , and for its entire duration in relation to competition between the public and private sectors . i suggested early on that the nucleic acid sequences collected on a genome scale should be considered as elements of description insufficient to warrant property rights by themselves in the absence of a genuine invention and should thus be placed in the public domain . a similar attitude was taken by the participants of the hgp in 1996 , as expressed in the ' bermuda rules ' , with the result that the openly accessible reference human genome sequence is now the common basis for current research . these proposals contributed to the ' universal declaration on the human genome and human rights ' adopted by the united nations and its educational , scientific and cultural organization ( unesco ) in 1997 - 1998 , which stated about the human genome : " in a symbolic sense , it is the heritage of humanity " , and " the human genome in its natural state shall not give rise to financial gains . " the issue was , and remains to a large extent , how best to balance general and particular interests to sustain basic research while promoting efficient healthcare product development . this has been discussed extensively on ethical , legal and social grounds , and the counterproductive underuse of scarce resources when they are protected by excessive intellectual property rights ( the tragedy of the ' anticommons ' ) has been pointed out ; these discussions have led to proposals to establish patent pools to facilitate development of diagnostic tests . the recent advent and rapid development of new generations of very - high - throughput dna sequencing methods makes it now possible to foresee that in the next few years the sequencing and assembly of thousands of human genomes ( and transcriptomes ) will be achievable at a cost of $ 1,000 each or less , which is a projected decrease of almost a million - fold in less than ten years . without the availability of the reference sequence , run delivering information on the scale of the entire genbank , it is clear that data quality assessment and analysis are becoming the limiting steps , beyond the capability of single individuals or groups . similar trends can be anticipated for proteins and metabolites when reference proteomes and metabolomes also become available in the coming years . public electronic repositories for these large - scale datasets , together with standards and open access publications for their description , have been important developments in the past decade for ensuring that they become available for further studies . however , despite requirements by prominent journals and funding agencies for submission of primary data as a condition for publication and financial support , recent surveys indicate variable compliance with these rules in both academia and industry . there is clearly room for significant improvements in this area if researchers are to take the best advantage of the large datasets produced . the same issues of data quality and availability are becoming prominent in the assembly of ever - increasing patient cohorts for the purpose of clinical trials and genome - wide genetic association studies , now often reaching tens of thousand of samples . great efforts have been made , initially in developed countries , to establish standards of good practice for informed consent , clinical trial registration and sample collection and storage in biobanks , and these are now being enforced in newly industrialized countries such as china and india . although these are welcome developments , much also remains to be done to ensure that these essential resources are used to the best advantage of the patients themselves , and to use genomics and bioinformatics to sustain the development of systems biology and medicine . the issues are many and complex , given the sensitive status of human material with respect to legal rules and practices that can vary substantially from country to country . international harmonization of health regulations and intellectual property rights is ongoing ; this is necessary but insufficient to overcome the major bottlenecks in the development of healthcare products , and it will take time to mature and adapt to the rapid pace of technology development . all stakeholders should work together to identify topics and areas in which joint actions would improve the situation significantly in the short term . i would like to suggest that one such topic is the status and availability of large amounts of underexploited experimental and clinical data in public and private laboratories . in many cases , these existing databases have been developed for a specific purpose , with a focus on a small number of biological elements . with the shift from targeted to global analyses , most of the data collected are not exploited at all , although they could be relevant in another context . it must be recognized that the high potential value of these datasets relies to a large extent on the quality of the biological and clinical annotations , which becomes significant only if the experimental data are properly collected and described . when that is the case , the added value will come from provision of the combined data for further analysis by other experts addressing related and complementary questions . recognizing this as a topic for sharing of knowledge between academic and industrial partners and establishing data warehouses with agreed open access rules would be a significant step in this direction .

to help overcome the bottlenecks that limit the development of diagnostic and therapeutic products , academic and industrial researchers , patient organizations and charities , and regulatory and funding institutions should redefine the basis for sharing the knowledge collected in large - scale clinical and experimental studies .

an elderly woman with a history of high blood pressure and hypercholesterolemia was admitted to the emergency room for suspected stroke due to abruptly starting vertigo , blurred vision and difficulties in speech . neurological examination within 3 h from the symptom onset revealed high blood pressure ( 206/130 mm hg ) , dysarthria and impaired right hand coordination . twelve hours after symptoms onset , the patient could not speak or swallow but was able to move all extremities . the patient was intubated because of breathing difficulties and co2 retention . at 24 h , the patient could not open her eyes , here pupils were dilated , and there were no spontaneous respiration or movements . yet a brain mri revealed an old hemorrhage in the pons , and csf findings were normal . nerve conduction study findings showed that motor amplitudes were negligible , but sensory responses were preserved . in electromyography of the finger 1 ) was observed , but no increment was found in repeated nerve stimulation ( rns ) . serum , gastric fluid and stool samples were taken , and botulinum antitoxin was given 3 days after symptom onset . despite all treatment efforts , the patient died 13 days after admission to the hospital due to septic shock and multiorgan failure . three days after the arrival of the first patient , a previously healthy 29-year - old woman was admitted to the emergency room for suspected stroke . it was soon found that the patient was a relative of patient 1 , and that they had eaten the same food . although she received the antitoxin treatment the same evening , her neurological symptoms deteriorated during the first 2 days . she needed assisted ventilation for 3 weeks , although other symptoms were resolved within 1 week . those olives were strongly positive for botulinum neurotoxin type b , and the gastric content and fecal samples of both patients tested by multiplex pcr were positive for clostridium botulinum type b. in addition , the serum of patient 1 was weakly positive for botulinum neurotoxin . botulinum toxin binds irreversibly to presynaptic plates of the neuromuscular junction causing dysfunction of voluntary muscles , inner organs and the autonomic nervous system . in addition , the toxin may modify the inhibition in the central nervous system , possibly due to altered afferent input from the muscle spindles . botulinum antitoxin neutralizes only the circulating toxin and should thus be administrated as soon as possible before completing the laboratory tests . the diagnosis must therefore be based on clinical findings ; bulbar palsy with descending paralysis is the most typical finding . especially the combination of bilateral drooping of the eye lids , absent gag reflex and respiratory failure with relatively well preserved limb forces and a clear mental status are suggestive of botulism and , more uncommonly , of other possible diagnoses like brainstem ischemia or polyradiculitis . botulism diagnosis is further supported by normal mri and csf findings . according to the literature , as a sign of presynaptic transmission defect , high - frequency rns shows often an incremental motor response in botulism patients . however , no increment was observed in our two cases , possibly due to largely destroyed endplates . neurological symptoms may develop acutely in food - borne botulism and may therefore be misinterpreted as brainstem ischemia .

botulism is a muscle - paralyzing disease caused by neurotoxins ( types a g ) produced by the bacteria clostridium botulinum . symptoms of food - borne botulism most commonly appear 1236 h after eating contaminated food , but the earliest neurological symptoms may in some cases start abruptly . here , we report the cases of two patients with food - borne botulism who were admitted to the neurological emergency room as candidates for intravenous thrombolysis for acute stroke .

we present the case of a 60-year - old female with a history of polycythemia , chronic obstructive pulmonary disease ( copd ) on home oxygen , and obesity who presented to the emergency room complaining of mild dyspnea on exertion that had been experienced for 3 days . she denied any medical history , including autoimmune disease , connective tissue disorders , or vasculitis . as part of her general workup in the emergency department , she was found to have elevated troponin levels at 0.182 ng / ml ( 0.00.045 ng / ml ) . ecg revealed sinus tachycardia ; there were no st segment changes suggestive of ischemia ( fig . the patient was admitted to the floor and managed as a possible case of copd exacerbation . twelve - lead ekg at the time of emergency department presentation with sinus tachycardia and no acute st segment changes . a nuclear stress test was performed that revealed a large inferior wall reversible defect with associated inferior wall hypokinesis . coronary angiography of the right coronary showed a spontaneous dissection throughout the mid - right coronary artery ( fig . due to poor distal thrombolysis in myocardial infarction ( timi ) 1 flow , it was decided to stent the lesion . a prowater wire was steered toward the mid - right coronary artery which initially went into the false lumen ; we then pulled the wire back and redirected it to the true lumen which was confirmed by the ability to advance the wire into the distal vessel . follow - up angiography showed excellent results with complete tacking of the dissection plane and excellent timi 3 flow at the end of the procedure ( fig . 3 ) . coronary angiography showing a dissection plane throughout the mid - right coronary artery . follow - up angiography showing complete tacking of the dissection plane and timi 3 flow . while a few of the reported cases have been called idiopathic , a predisposed arterial disease or underlying cause has been identified in most of the cases ( 3 ) . intimal tear or bleeding of vasa vasorum with intramedial hemorrhage has been proposed ( 4 ) . younger females in the pregnancy and peri - partum periods are thought to be at a higher risk than the general population which is believed to be secondary to increased hemodynamics during labor and delivery , and possibly hormonal effect on the arterial walls affecting the tunica media ( 5 , 6 ) . it is unclear whether this inflammatory reaction is reactive or causative of dissection by weakening the coronary architecture ( 5 ) . connective tissue disorders such as ehler danlos type iv and marfans have also been implicated in scad ( 8 , 9 ) . our patient did not have any clinical features usually associated with such connective tissue diseases . she is an elderly female presenting with symptoms initially thought to be suggestive of a copd exacerbation with a troponin elevation secondary to a supply demand mismatch . this presentation serves as a reminder to include scad in the differential diagnosis even among elderly patients with comorbidities presenting with unexplained troponin elevation . other management strategies have been proposed in the literature ranging from percutaneous coronary interventions to heart transplantation . revascularization in scad is challenging and should be considered in patients with ongoing ischemia ( 10 ) . intravenous ultrasound ( ivus ) may be helpful in confirming the diagnosis . in our patient , ivus was not used due to her narrow lumen coronaries with a concern of possible worsening of the dissection with the bulky ivus catheter . none of the authors have any financial or personal bias that would inappropriately compromise the publication of this work . the authors have not received any funding or benefits from industry or elsewhere to conduct this study .

spontaneous coronary artery dissection is a poorly understood phenomenon that usually affects women during pregnancy or the immediate post - partum period . we present the case of a 60-year - old female with chronic obstructive pulmonary disease who presented with vague complaints of shortness of breath , dizziness , and weakness with a mildly elevated troponin . she denied any anginal symptoms . as part of her initial workup , a nuclear stress test revealed inferior wall reversible changes . coronary angiography revealed spontaneous right coronary artery dissection which was treated with a drug - eluting stent .

a 38-year - old male patient with a 40-pack - year history of smoking initially presented with sudden - onset right - sided facial nerve palsy which was diagnosed as idiopathic bell 's palsy and was treated with prednisolone . three weeks later , the patient developed a new left - sided facial palsy in addition to his unrecovered right - sided facial palsy . outside of the facial nerve paralysis , he was neurologically intact , and his physical examination revealed no abnormalities . further workup was carried out to rule out autoimmune , metabolic , infectious , and vasculitic causes and revealed no abnormality . a chest x - ray showed a right upper lobe opacity which prompted a computed tomography ( ct ) scan of the chest which showed a well - defined , suspected soft - tissue lesion in the right upper lobe with extensive hilar and ipsilateral mediastinal lymphadenopathy ( fig 1a , b ) . at this point , the differential diagnosis included sarcoidosis and primary lung cancer given the presenting complaint was bilateral facial nerve paralysis . because the intrapulmonary lesion was too distal to be reached by flexible bronchoscopy , video - assisted mediastinoscopy was performed and revealed gross right - sided mediastinal invasion . multiple biopsies from lymph nodes were taken and revealed metastatic poorly differentiated adenocarcinoma of the lung . accordingly , positron emission tomography ( pet)/ct and magnetic resonance imaging ( mri ) of the brain were performed to stage the cancer . brain mri with contrast showed normal enhancement of the seventh and eighth cranial nerves with neither brain metastasis nor leptomeningeal metastasis . the pet / ct showed a metabolically active right upper lobe lesion with extension into the mediastinum and active ipsilateral mediastinal lymph nodes but no distant metastasis . due to our high clinical suspicion of leptomeningeal involvement , a lumbar puncture was performed . at this point , his clinical stage was considered ciiib ; ct4 ( extension ) n2 m0 . the patient received platinum - based doublet chemotherapy with a total of 60 gray of radiation . he tolerated the chemotherapy and radiation well but had no resolution of his facial paralysis . nine months later , the patient presented to the emergency department with severe headache and generalized tonic - clonic seizures . on physical examination an abdominal and pelvic ct scan was obtained which showed newly developed liver metastases as well . given the patient 's disease progression and deteriorating condition , palliative care was offered , and the patient succumbed to his disease . the incidence of leptomeningeal carcinomatosis has been reported to be between 0.8 and 8% in many autopsy studies , though the true incidence is difficult to determine . among all known solid tumors , leptomeningeal metastasis is more common in breast cancer , lung cancer , and melanoma [ 1 , 2 ] . with improvements in survival rates among patients who have cancer , the incidence of leptomeningeal carcinomatosis in patients with non - small - cell lung cancer is increasing . however , small - cell lung cancer is more likely to cause leptomeningeal metastases with a frequency of 1025% , as compared to 1% in the case of non - small - cell lung cancer . though leptomeningeal metastasis is a late complication of cancer , it can be the initial presentation in some cases . our case is one of these cases where neurologic symptoms were the first and only presentation of pulmonary adenocarcinoma . reviewing the literature , and to the best of our knowledge , our case is the only case describing bilateral facial nerve palsy as the only manifestation of lung adenocarcinoma . however , as demonstrated here , patients may present with a single isolated neurological sign or symptom . among these facial weakness was detected as a sign of an underlying leptomeningeal metastasis in about 13% of cases at the time of presentation . multiple cases in the literature reported unilateral peripheral facial nerve palsy as the first presentation of an underlying cancer . in some of these cases , the underlying cancer was pulmonary adenocarcinoma . csf analysis has been considered the gold standard method to diagnose leptomeningeal metastases ; however , the false - negative rate seen in many cases ( even after repeating the lumber puncture ) makes the diagnosis of leptomeningeal metastases challenging , causing many to suggest using mri as well as csf analysis . csf findings suggestive of leptomeningeal metastases are high cell count , high protein , low glucose , and high csf opening pressure . mri of the brain is considered more sensitive in leptomeningeal metastases from solid tumors , while csf analysis is superior to mri in cases of hematological malignancies [ 5 , 7 ] . csf samples are more likely to have positive findings from either clinically or radiographically diseased areas . our patient 's second csf sample was drawn directly from the ventricles after his disease had progressed , which may have played a role in the positive result of the csf cytology . all authors have been personally and actively involved in substantive work leading to the manuscript and will hold themselves jointly and individually responsible for its content . the authors declare that no financial or other conflict of interest exists in relation to the content of this paper .

leptomeningeal carcinomatosis is rare , and its precise incidence is unknown . it is associated with a wide spectrum of solid and hematological malignancies . to complicate its diagnosis , the clinical presentation of leptomeningeal carcinomatosis can be variable . we report a case of a 38-year - old male with bilateral facial nerve paralysis as first presentation of lung adenocarcinoma . to our knowledge , this is the only case describing bilateral facial nerve palsy as the first and only manifestation of lung adenocarcinoma .

acidithiobacillus ferrooxidans ( a. ferrooxidans ) is a gram - negative , extremely acidophilic , mesophilic , chemolithotrophic bacterium and the most well - studied acidophilic organism which is usually found in acid environments such as acid mine drainage , . due to its bioleaching capabilities , it is an important member of microbial consortia involved in the industrial recovery of metal under mesophilic conditions ( bioleaching or biomining ) . recently , a. ferrooxidans has played important roles in bioleaching and harnesse environmental contamination , . like in other acidophilic iron - oxidizing bacterium , it grows optimally at about 35 c in 9k inorganic medium at extremely low ph ( ph 1.02.0 ) and fixes both carbon and nitrogen from the atmosphere . a. ferrooxidans derives energy from oxidizing reduced sulfur compounds and fe ions to form sulfate and fe , respectively . dna was isolated from 1.01.5 g of cell paste using qiagen genomic 500 dna kit ( qiagen , hil - den , germany ) with a modified protocol , st / ft , for cell lysis , as described in valdes et al . . draft genome sequence of a. ferrooxidans type strain dlc-5 was obtained in illumina hiseq2000 sequencing technology by shanghai majorbio bio - pharm technology co. , ltd . ( shanghai , china ) , using the short oligonucleotides alignment program ( soap ) denovo alignment tool ( http://soap.genomics.org.cn/ ) processes reads assemble . a library containing 300-bp inserts was constructed . altogether , 6,372,268 paired reads ; 398,580 single reads ; total 1,079,535,272 bp bases with average coverage of 221.1 . reads were filtered to remove adapter sequences , low - quality bases ( phred score , < 20 ) , removing the 5 end that contains the bases of it is not a , g , c , t before shearing , remove reads with the containing 10% of n , giving up adapter and small fragments of length less than 25 bp after qualitative pruning . the reads were assembled into 881 contigs ( > 1000 bp ; contig n50 , 102 bp ; contig n90 , 569 bp ) and 573 scaffolds ( > 1000 bp ; scaffold n50 , 71 bp ; scaffold n90 , 333 bp ) . until now , two genome sequences of a. ferrooxidans strains atcc 23270 and atcc 53993 are available in the public databases , . these genomic data are useful for the experimental identification of unique proteins or estimation of the phylogenetic relationship among the related strains . strain dlc-5 ( cctcc - m 2014362 ) is the type strain of a. ferrooxidans , isolated from wudalianchi in heihe of heilongjiang province , and the type species of the genus acidithiobacillus , which currently contains five species . the draft genome sequences of strain dlc-5 have great importance to provide more explicit information for the physiology and metabolic potential of a. ferrooxidans . further analysis of the genome sequence via gene engineering might improve the oxidation of fe efficiency by strain dlc-5 . the genome includes two plasmids , for a total size of 3,142,890 bp , with one circular chromosome of 1,832,305 bp ( 58.3% gc content ) . for the main chromosome , 4299 bp genes 3250 bp of protein coding genes were assigned to a putative function with the remaining annotated as hypothetical proteins . the properties and the statistics of the genome are summarized in table 1 . the distribution of genes into cogs functional categories is presented in table 2 . extremely acidophilic bacteria and archaea with special emphasis on bioleaching microorganisms are widely distributed in the extreme acidic environment . in this study , we analyzed the genome sequence of a. ferrooxidans dlc-5 , which was isolated from acid mine drainage in northeast china . it may contribute to further studies on important process for bioleaching and acid mine drainage production , such as biofilm formation , energy resources utilization and quorum sensing that could play a role in a possible interrelationship of bioleaching heaps and other acidic environments . in addition , combining with genomes of other members in acidithiobacillus , will make an important advance in understanding of the ecological roles that acidithiobacillus species play in those acidic environments and their relationships with other extremely acidophilic microorganisms . the sequence of a. ferrooxidans dlc-5 under this whole genome shotgun project has been deposited at ddbj / embl / genbank under the accession jnnh00000000.1 .

acidithiobacillus ferrooxidans type strain dlc-5 , isolated from wudalianchi in heihe of heilongjiang province , china . here , we present the draft genome of strain dlc-5 which contains 4,232,149 bp in 2745 contigs with 57.628% gc content and includes 32,719 protein - coding genes and 64 trna - encoding genes . the genome sequence can be accessed at ddbj / embl / genbank under the accession no . jnnh00000000.1 .

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a 57-year - old woman developed multiple ( > 20 ) melanoma in - transit metastatic nodules on her right thigh ( figure 1a ) , as confirmed by histological examination , after removal , six months earlier , of a primary 2.2 mm thick ulcerated nodular melanoma on her right leg for which the initial sentinel lymph node biopsy was negative . after completion of fdg pet - ct to exclude distant metastasis and assessment of the extent of limb metastasis , the patient was referred to a specialized center to receive , isolated limb perfusion ( ilp ) of melphalan under hyperthermia , tnf- and interferon . superficial and deep pelvic ( ilioinguinal ) lymphadenectomy was simultaneously performed and revealed two additional deep pelvic lymph node metastases . one month after the end of this procedure , most in - transit lesions had undergone necrosis . four months later , all lesions had healed and persisted as non - changing pigmented macules ( figure 1b ) with no clinical or radiological ( including fdg pet - ct ) signs of relapse . in order to confirm that none of the residual pigmented macules was an active lesion , two of them were excised after being previously examined by rcm . histological examination showed a persisting melanophage granuloma ( nodular melanosis ) within areas of fibrosis in the upper dermis with no sign of tumor infiltration ( figure 2a and 2b ) . rcm imaging , performed using the vivascope 1500 ( vivascope systems , munich , germany ) showed ill - defined aggregates of dermal bright cells , typical of melanophages with almost no visible nuclei . sometimes a characteristic ring - shaped deposit was seen within the network of bright collagen fibers and bundles ( figure 3 ) . such deposits could be seen in many areas of the excised lesions and correlated with the histological images . we then performed rcm imaging on 12 other main persisting pigmented lesions ( figure 1b ) in which a similar characteristic image was consistently seen . the patient has now been followed for more than a year without any clinical or radiological sign of relapse and most lesions have slowly resolved ( figure 4 ) . around 510% of patients with malignant melanoma develop lymphatic dissemination and in - transit metastasis . even in the era of effective systemic therapies , isolated limb perfusion ( ilp ) with hyperthermia performed using various agents ( melphalan , with addition of chemokines , i.e. , tnf- and/or other chemokines ) is still a valuable therapeutic approach and is being used for patients who develop numerous limb lesions simultaneously and are therefore considered inoperable . according to the literature nevertheless complete response can be difficult to assess if post - necrotic persisting pigmented lesions occur , especially if numerous and unresectable . these residual lesions occur mainly in the more superficial and pigmented types of metastatic disease . as it was the case with our patient , these lesions can often correspond to the residual melanophage granuloma ( nodular melanosis ) that persists after tumor tissues undergo complete necrosis and can be difficult to distinguish from partial response and persisting in - transit metastasis . rcm is a non - invasive technique that allows the exploration of the superficial dermis . confocal - histopathological correlation has been shown to be possible for the visualization of some histologic features in the superficial dermis up to 300um deep [ 36 ] where , in the case of superficial in - transit metastasis , post - necrotic residual melanophage granuloma ( nodular melanosis ) might be localized . morphologic features of melanophages under in vivo rcm have been well characterized , allowing their distinction from melanocytes . here , using combined rcm and histological examination of two sample lesions , numerous unresectable post - ilp limb residual pigmented lesions were considered by rcm to be non - active as confirmed by the subsequent dermoscopy follow - up . although in the case presented here there was not any doubt at follow - up regarding the absence of residual disease , considering that the deeper reticular dermis can not be explored with rcm , biopsy remains mandatory in case of doubt during the subsequent needed close follow - up .

complete response can be difficult to assess after isolated limb perfusion ( ilp ) for metastatic in - transit melanoma , especially when numerous and unresectable post - necrotic persisting pigmented lesions occur . these residual lesions are mainly seen in the more superficial and pigmented types of metastatic disease and correspond to the residual melanophage granuloma that persists after tumor tissues undergo complete necrosis . reflectance confocal microscopy ( rcm ) is a non - invasive technique that allows the exploration of the superficial dermis . here , we present the case of a patient in whom numerous post - ilp limb residual pigmented lesions were explored using combined rcm and histological examination of sample lesions and could be characterized as non - active . this approach allowed us to avoid additional excisions .

a 66-year - old woman with sjgren syndrome presented with a 5-year history of bilateral shoulder pain that was significantly worse on the right than the left side . the pain was reported to be constant in nature but worse at night , and completely prohibited activities above shoulder height . examination of the right shoulder revealed a globally restricted range of movement at the glenohumeral joint . treatment with copeland resurfacing hemiarthroplasty was performed in june 2007 through an anterosuperior ( mackenzie ) approach . . 1 interrupted pds ( polydioxanone ) sutures were used for reattachment of the anterior border of the osteo - periosteal sleeve to the acromion . the sutures were placed transosseously through the acromium on one side and through the deltoid fascia , incorporating an osteo - periosteal sleeve , on the other [ figure 2 ] . preoperative radiograph demonstrating severe osteoarthritis radiograph taken in the immediate postoperative period showing a copeland resurfacing hemiarthroplasty passive elevation past 90 could be achieved on the first postoperative day . a shoulder immobilizer was worn for 3 weeks and the patient was instructed to avoid active elevation . the patient had returned to aerobics and table tennis by the 4 postoperative month , at which point increased pain in the shoulder was reported . at the end of the 1 postoperative year , significant impingement symptoms were reported and x - rays demonstrated a new bony spur on the anteroinferior edge of the acromion [ figure 3 ] . an ultrasound scan showed a bony spur indenting the supraspinatus , but there was no accompanying cuff tear . the pain continued to worsen and diagnostic arthroscopy was performed in july 2009 , which confirmed the presence of the spur at the anteroinferior edge of the acromion and excluded the presence of significant glenoid arthrosis or adhesions as contributing factors to the patient 's symptoms . radiograph at 1 year showing new bony spur on the inferior surface of the acromion appearance following arthroscopic resection of the spur the mackenzie anterosuperior approach has been popularized for several types of shoulder arthroplasties . in this approach the anterior deltoid is commonly reflected with an osteo - priosteal sleeve . we postulate that the bony spur responsible for the impingement symptoms in our patient was formed by malunion of the osteo - periosteal sleeve . at the time of arthroscopy , a sharp spur of bone of the same size and shape as the osteo - periosteal sleeve was found to be united in the correct position on the anterior acromion but , from its shape , it had apparently rotated 90 from the original orientation . the onset of symptomatic impingement along with new x - ray findings at 4 months postoperatively is consistent with rotation of the fragment during healing following the patient 's return to sporting activity . full resolution of the patient 's symptoms following resection of the spur also supports this conclusion . intra - articular factors are the most common reasons for postoperative pain following copeland resurfacing hemiarthroplasty and are due to the nonanatomical shape of the prosthesis , which does not accurately recreate the original shape of the humeral head . as our patient was initially pain free with good shoulder function , we carried out other investigations , including diagnostic arthroscopy , to look for other causes for her pain preoperative investigation to establish the diagnosis of subacromial impingement could include an injection of local anesthetic under aseptic technique into the subacromial space . immediate pain relief would be convincing evidence of the existence of subacromial impingement . to the best of our knowledge , this is the first report of formation of an acromial spur following the mackenzie approach . complications of the copeland resurfacing hemiarthroplasty have been reported as infection , aseptic loosening , periprosthetic humeral fracture , and osteolysis . subacromial impingement has been reported in patients after this procedure and the symptoms have necessitated subacromial decompression , but this has not previously been reported to be associated with a malunited osteo - periosteal sleeve . nonunion ( or fibrous union ) has been reported by previous authors following surgical access using the mackenzie approach but they did not report symptomatic subacromial impingement . in our patient , simple arthroscopic excision of the bony spur proved curative . this case has altered our practice , as we have now abandoned the osteoperiosteal sleeve technique and instead opt for subperiosteal dissection of the anterior deltoid . we conclude that the cause for the delayed , severe impingement symptoms in this patient following successful copeland resurfacing hemiarthroplasty was malunion of the osteo - periosteal sleeve that was made as part of the mackenzie approach . all symptoms resolved following arthroscopic resection of the spur .

we report the case of a patient with end - stage osteoarthritis who received a successful copeland resurfacing hemiarthroplasty through a mackenzie anterosuperior approach , which involves taking the anterior portion of the deltoid attachment from the acromion along with an osteo - periosteal sleeve . the patient went on to develop severe subacromial impingement symptoms 4 months postoperatively . x - rays revealed a large anteroinferior acromial osteophyte that had not been present preoperatively and was deemed to represent a malunited osteo - periosteal sleeve from the mackenzie approach .

curing type acetoxycolor tranlucentdensity approximately 1.04 g / ccskin formation time approximately 5 min at 30ccuring time approximately 24 h. for 2 mm thickness at 30cmovement accommodation approximately 0.61 n / mmelasticity approximately 260% curing type acetoxy density approximately 1.04 g / cc skin formation time approximately 5 min at 30c curing time approximately 24 h. for 2 mm thickness at 30c movement accommodation approximately 15% tensile strength step 1 : ear impression is made using irreversible hydrocolloid alginate and custom made wax rim . wet gauze pieces are layered on top of alginate for the interlocking of the plaster to be poured [ figure 1 ] . making of ear impression step 2 : undercut areas in the impression are identified and blocked out with wax . block out impression partly with wax and cast poured step 3 : working model is retrieved from the impression carefully and base trimmed [ figure 3 ] . working model with dental stone prepared step 4 : custom tray using soft tray sheets prepared . a complete ear cast is made by making alginate impression for a person related to the patient [ figure 4 ] . custom tray using soft tray sheets prepared for complete ear cast step 5 : skin tone of the patient is matched to the oil colors . colors mixed with silicone step 6 : silicone material is dispensed to the under cuts of the custom tray and filled completely [ figure 6 ] . material dispensed with syringe into soft sheet custom tray step 7 : separating medium is applied on working cast and under cuts are filled with silicone material . it is pressed against the soft sheet custom tray carrying the silicone material [ figure 7 ] . silicone filled tray pressed against working cast step 8 : prosthesis is tried on the model and trimmed before insertion on patient [ figure 8 ] . over a period of time various materials such as wood , leather , polyurethane and polyvinyl chloride have been used to produce esthetic properties to prosthesis , but silicone has proved to be the material of choice because of its lifelike effects and flexibility . methyl methacrylate resin is been used as a maxillo - facial material , but its use is limited due to its rigidity . the flexibility and staining of silicone this industrial silicone has the property of elasticity and has the potential to incorporate stains and colors to mimic natural skin . the advantage of this industrial silicone material is the low cost factor , soft nature , flexibility , easy to fabricate and having good esthetic property and it will be an ideal material for the post - graduate student to train himself and acquire skills during his pre - clinical training program , which includes maxilla facial prosthodontics . since the cost is not prohibitive and as this material is quite similar to the current silicones , it can be recommended .

this article describes an industrial elastic silicone as a material for the laboratory fabrication of ear prosthesis . it has been tested for toxicity in lab animals by the sgs india pvt . ltd and approved as a material to pass the parameter of abnormal toxicity . this material therefore can be safely recommended for laboratory exercise to fabricate facial prosthesis . the high cost of the maxillo facial silicone materials prohibits their use for facial prosthesis in pre - clinical training of post - graduate students in maxillofacial prosthodontics . for this reason , pre - clinical laboratory exercise in facial prosthesis is inadequate . a few institutions use polymethyl methacrylate resins which are rigid and do not have elastic characteristics of silicone , which is used for facial defects . this cost - effective industrial silicone material which mimics the elastic and color characteristics of the conventional silicones can be recommended for preclinical exercises .

since first described by quain in 1847 ( 1 ) , hundreds of abdominal aortic stenosis have been reported with many variations in anatomy . though , the complete absence of infrarenal aorta , seen in presenting case , is extremely rare . this represents the first case of complete segmental occlusion of infrarenal aorta in the old patient . a 52-yr - old woman presented to the clinic with one - year history of bilateral claudication . blood pressures were measured at 130/80 mmhg in the forearms and at 100/70 mmhg in the thighs , with ankle - brachial indexes of 0.77 . serologic tests including c3 , c4 , venereal disease research laboratory test ( vdrl ) , and immunoglobulin g were unremarkable except mild elevated erythrocyte sedimentation rate ( esr ) of 22 mm / hr and low tittered anti - nuclear antibody of 1:80 with speckled pattern . the abdominal aorta was reconstituted by multiple collaterals , such as marginal artery of drummond , the anastomosis between superior mesenteric and inferior mesenteric artery , bilateral epigastric , lumbar , and abdominal superficial arteries . for her knee joint arthritis , she was started with methylprednisolon of 8 mg and non - steroid anti - inflammatory drug . after negative results of pathergy test and human lymphocyte antigen b 51 , we ruled out the behcet 's disease and she tapered the medications to stop successfully . she wanted the antihypertensive drug for mild hypertension and was taken amlodipine at 5 mg daily . one year later , computer tomographic angiography showed patent bypass graft and decreased collateral vessels ( fig . the etiology of abdominal aortic stenosis is poorly understood . the young age of most of the patients suggests that the malformation may be congenital . maycock first proposed an unequal fusion of embryonic aorta as a congenital mechanism ( 3 ) . in first month of fetus , the paired primitive dorsal aortas fuse to form the abdominal aorta . incomplete fusion due to infection or inflammation may lead of kinking of the aorta resulting to permanent constriction . congenital rubella has been suggested for such vascular anomaly ( 4 , 5 ) . in alagille syndrome which is congenital pulmonary artery stenosis transmitted in an autosomal dominant inheritance , deletion of the jagged 1 gene has been suggested to relate with abdominal aortic coarctation ( 6 ) . acquired etiologies were also suggested in fibromuscular dysplasia or takayasu 's arthritis ( 7 , 8) . in our patients , several inflammatory presentations including the knee joint arthritis , recurrent oral ulcers , and elevation of esr have suggested the possibility of behcet 's disease . we ruled out the behcet 's disease after the negative study of pathergy test . considering the complete absence of infrarenal aorta without any inflammatory reaction , in review of cases for abdominal aortic stenosis , coarctation , hypoplasia , and middle aortic syndrome were used diversely . the term " coarctation " implies a congenital anomaly , and " hypoplasia " is to longer segment of coarctation . middle aortic syndrome is the clinico - anotomical entity involving long segmental narrowing of the abdominal aorta and various visceral arteries irrespective of its etiology . we described this case by " hypoplasia " , because 4 cm of infrarenal aorta was absent . presenting symptoms of abdominal aortic hypoplasia are related to the extent of the stenosis , and the collateral system . in case involving renal artery , patients typically present with uncontrolled hypertension . infrarenal aortic hypoplasia can give rise to claudication when the collateral circulation is not sufficient . an abdominal aortogram via angiography , ultrasound , computed tomography , or magnetic resolution imaging can be used to diagnose and to determine the location and extent of disease . in case of renal artery stenosis claudication and aneurysmal dilatation also need surgical correction . in this case , as the subrenal aortic stump was too short , graft was interposed between the subdiaphragmatic thoracic aorta to the iliac bifurcation . careful physical examination for inspection of dilated superficial arteries and auscultation of bruits can provide a clue for rare causes of ileofemoral insufficiency such as abdominal aortic stenosis . when suspected , an angiography is mandatory to diagnose , and surgical intervention is necessary to improve severe claudication .

we describe a case of infrarenal aortic hypoplasia in a 52-yr - old woman who presented with claudication . computed tomographic angiography revealed an abrupt absence of the infrarenal aorta , with collateral flow reconstituting the iliofemoral systems . after a polytetrafluoroethylene graft was interposed between the aortic stump and the iliac bifurcation , the patient 's claudication resolved .

focal choroidal excavation ( fce ) was first described by jampol et al1 as an anomalous excavation of the choroid ; it is typically observed by optical coherence tomography ( oct).1,2 this condition has been associated with several chorioretinal conditions and asymptomatic status , including central serous chorioretinopathy , choroidal neovascularization , and best vitelliform macular dystrophy.112 however , the mechanisms underlying the formation of fce remain uncertain . wakabayashi et al2 described two patterns of fce : 1 ) conforming pattern excavations that involved the outer retinal layers up to and including the external limiting membrane ; and 2 ) nonconforming pattern excavations that involved only the retinal pigment epithelium ( rpe ) . here we describe a clinical course of fce associated with vkh before and after treatment and compare the morphological findings of fce in four subjects with different chorioretinal conditions . a 55-year - old man ( subject 1 ) presented with bilateral metamorphopsia , with decimal visual acuity being 0.8 in the right eye and 1.2 in the left . the clinical diagnosis of vkh was made based on bilateral panuveitis and multifocal exudative retinal detachments at the posterior poles ( figure 1 ) . the serous detachments entirely resolved within 9 weeks and visual acuity in the right eye improved to 1.2 . serial spectral - domain oct ( sd - oct ) images obtained at the preuveitic , uveitic , and posttreatment phase are shown in figure 2 . sd - oct obtained 1 year before the onset of vkh due to symptoms of a floater and visual disturbance demonstrated a dome - shaped posterior protrusion of the rpe and outer retinal layers into the choroidal cavity ( ie , conforming fce ) at the fovea in the right eye . sd - oct at the uveitic phase identified multiple bilateral sensory retinal detachments , with the fce now involving only the rpe ( ie , nonconforming fce ) . the fce pattern and its alteration during treatment of the case with vkh were compared to those of four subjects with fce associated with other chorioretinal conditions ( figure 3 ) . this comparison group consisted of two patients with age - related macular degeneration ( amd ; subjects 2 and 3 ) , one subject with a macular hole ( mh ; subject 4 ) , and one asymptomatic individual ( subject 5 ) . treatment with intravitreal ranibizumab injections and vitrectomy had been performed in the patients with amd and mh , respectively . a conforming fce was observed in three patients ( subjects 2 , 3 , and 5 ) and pattern alteration from nonconforming to conforming fce was found after treatment in one subject ( subject 4 ) . in the two subjects with amd , a hyperreflective material around the fce was absorbed , resulting in a well - demarcated conforming fce after treatment . a detailed clinical course of fce at the fovea was documented in a case with vkh disease . sd - oct images obtained at the preuveitic , uveitic , and posttreatment phase suggested the preexistence of fce before the onset of vkh , and the fce pattern change ( from conforming to nonconforming pattern during the period of choroidal inflammation ) was observed during the follow - up . the fce identified at the preuveitic phase in our case supported the congenital / acquired posterior pole malformation , as previously suggested.1,4,9,11,12 on the other hand , hashida et al13 speculated that a direct pressure effect on the choroidal layer by subretinal fibrin disrupted the choroidal integrity and focal choroidal atrophy / thinning following inflammation and resulted in a formation of fce in a case with vkh.4,7,13 although such a remarkable subretinal fibrin was not detected , the association between the fce formation and choroidal inflammation could not be excluded entirely in our case , considering other possible subclinical inflammatory events prior to this disease history . an alteration of the fce pattern observed in vkh was also found in the comparison group with other chorioretinal conditions . in subject 4 with an mh , the nonconforming fce at presentation associated with vitreomacular traction changed to conforming fce after treatment . in two subjects with amd , a hyperreflective material around the fce was observed to be absorbed , resulting in a well - demarcated conforming fce via treatment ( subjects 2 and 3 ) . these findings suggest that the cavity within fce can be filled with subretinal fluid associated with inflammation and exudative material generated by choroidal neovascularization . given the proximity between the fce and the active lesions , the involved retina around the active lesion may well show disorganization of retinal layers in the pathological process ; then the conforming pattern is hard to maintain , unsurprisingly . in addition , it is possible that the cavity within fce may potentially accelerate the pathophysiological changes of chorioretinal disorders .

we describe focal choroidal excavation ( fce ) in a case of vogt koyanagi harada ( vkh ) disease and compare the findings with different chorioretinal conditions . a 55-year - old man was diagnosed with vkh based on panuveitis and exudative retinal detachments . spectral - domain optical coherence tomography demonstrated a dome - shaped protrusion with a nonconforming pattern at the fovea , which had been detected as a conforming pattern 1 year before the onset . the fce pattern returned into a conforming pattern following corticosteroid therapy . these findings suggest that the natively existent fce could be affected by pathophysiological changes of vkh as well as other chorioretinal conditions .

the content of each peptide flask was dissolved by addition of hepes buffer 100 mm , ph 7.4 ( a128 ) or ultrapure milli - q water ( a116 ) . the concentration of a was then measured by uv - visible spectroscopy ( 276 nm ( tyr10)=1410 m cm ) . uv - visible spectra were recorded on a biochrom libra s50 or a specord 205 spectrophotometer ( analytik jena , germany ) . fluorescence spectra were recorded on a flsp920 spectrometer ( edinburgh instruments ltd , uk ) , with bandwidth for excitation and emission=2 nm . a complex was first prepared by mixing equimolar amounts of a128 and cucl2 in hepes buffer 50 mm , ph 7.4 . the metalation of a was monitored by the decrease of fluorescence ( see ref . ) . a solution of ligand 1 or cq in dmso was then added ( 1 or 2 mol equiv , respectively ) , and the reaction was monitored by uv - visible spectroscopy . final concentrations were [ a128]=[cu]==20 m , [ cq]=40 m ; dmso / hepes buffer=5:95 v / v . the 50 % decrease of fluorescence of a upon metalation by copper was confirmed in buffered mixture containing up to 10 vol % of an organic solvent , namely ch3cn ( ref . ) , or dmso ( present report , data not shown ) . x - band ( 9.525 ghz ) esr spectra were recorded in quartz tubes at 120 k , using a bruker elexsys - ii e500 spectrometer . for experiments with ligand 1 , the solvent was hepes buffer 100 mm , ph 7.4 , containing 13 vol % of dmso . [ a116]=185 m ; a116/cu molar ratio=1:1 ( figure 3 a ) , a116/cu/1=1:1:0.5 ( figure 3 b ) , a116/cu/1=1:1:1 ( figure 3 c ) , cu/1=1:1 ( figure 3 d ) . the addition of 16 vol % of dmso in hepes did not induce modification of the spectrum of cu the solvent was dmso / hepes buffer 100 mm , ph 7.4 , 90:10 v / v . a116/cu molar ratio=1:1 ( figure 5 a ) , a116/cu / cq=1:1:1 ( figure 5 b ) , a116/cu / cq=1:1:2 ( figure 5 c ) , cu / cq=1:2 ( figure 5 d ) . the sample solutions were injected ( 7.5 l ) using a mobile phase ch3oh / h2o ( 90:10 v / v ) , flow rate=0.15 ml min . the cone voltage was 15 v , and spectra were acquired in the positive ion mode , in the m / z range 1002500 . the mixture of a116/cucl2/cq ( 1:1:1 mol ratio ) was prepared in ultrapure milli - q water ( ph 5.8)/meoh ( 1:1 v / v ) . final concentration was 100 m , injected volume was 7.5 l . the series of multicharged patterns at m / z=1161.9 , 774.9 , and 581.4 was not detected in the absence of cu . as a service to our authors and readers , this journal provides supporting information supplied by the authors . such materials are peer reviewed and may be re - organized for online delivery , but are not copy - edited or typeset . technical support issues arising from supporting information ( other than missing files ) should be addressed to the authors .

the accumulation of redox - active metal ions , in particular copper , in amyloid plaques is considered to the cause of the intensive oxidation damage to the brain of patients with alzheimers disease ( ad ) . drug candidates based on a bis(8-aminoquinoline ) tetradentate ligand are able to efficiently extract cu2 + from copper - loaded amyloids ( cu a ) . contrarily , in the presence of a bidentate hydroxyquinoline , such as clioquinol , the copper is not released from a , but remains sequestrated within a acu clioquinol ternary complex that has been characterized by mass spectrometry . facile extraction of copper(ii ) at a low amyloid / ligand ratio is essential for the re - introduction of copper in regular metal circulation in the brain . as , upon reduction , the cu+ is easily released from the bis(8-aminoquinoline ) ligand unable to accommodate cui , it should be taken by proteins with an affinity for copper . so , the tetradentate bis(8-aminoquinoline ) described here might act as a regulator of copper homeostasis .

coronary artery disease ( cad ) is a leading cause of death throughout the world ( 1 ) . over the four decades , a significant decrease has been observed in age - related mortality caused by cardiovascular disease ( 2 ) . people in developing countries suffer from cad at a relatively younger age and about half of mi occurs under the age of fifty years ( 3 ) . inadequate perfusion due to atherosclerosis is a common cause of coronary artery stenosis ( 2 ) . ischemic chest pain in young adults is usually related to hypertrophic cardiomyopathy , congenital coronary abnormalities , tachyarrhythmia , myocarditis , aortic stenosis , dissection , or coarctation ( 4 ) . age , male sex , hypertension , smoking , diabetes , dyslipidemia , and obesity are considered as established risk factors for cardiovascular diseases and atherosclerosis ( 5 , 6 ) . these agents are used currently to increase protein synthesis and muscle growth in athletes ( 7 ) . since the use of anabolic steroids has been increased , different types of them produced ( 8) . mostly people start using the anabolic steroids in school time ( 9 ) . according to the statistics , 0.9% of men and 0.1% of women use these agents during their lifetime ( 10 ) . in this review , a 23-year - old male body builder was reported , with myocardial infarction ( mi ) following use of trenbolone acetate . a 23-year - old man was referred to the emergency room with epigastric pain since the last day . he was an athlete and his previous medical history was negative . due to the absence of risk factors for heart disease , symptomatic treatment was done for him and his pain decreased so that the patient was discharged from the emergency department . but , after 3 days the patient presented again to ed with the same complaint . the pain radiated to the left arm accompanied by nausea . after obtaining the accurate history of the patient , it was elucidated that he has been using the trenbolone acetate in his daily diet since last year . the patient 's vital signs were included blood pressure 130/80 mmhg , heart rate 105 per minute , respiratory rate 25 per minute , o2 sat 98% ( on room air ) . in physical examination , the patient had sweated and the lungs were clear on auscultation . s1 and s2 sounds of heart were normal and s3 sound was heard , too . the abdomen was soft but not tender . according to the examination and history of the patient , electrocardiogram ( ecg ) cardiac markers ( creatine kinase mb and troponin i ) were elevated . due to the unavailability of a heart center for doing an emergency percutaneous coronary intervention ( pci ) , he was admitted to the cardiology ward and after a week , angiography was done for him . the patient s angiography showed the stenosis of the left anterior descending ( lad ) and left circumflex ( lcx ) arteries ( figure 2 ) . anabolic steroids can cause many problems such as cardiovascular which includes hypertension , left ventricular hypertrophy , impaired diastolic filling , polycythemia and thrombosis ( 7 ) . the effects of steroids on lipid profile include decreasing in high density lipoprotein ( hdl ) , increasing in low density lipoprotein ( ldl ) and total cholesterol . these changes are leaded to increase the risk of atherosclerosis in the coronary arteries ( 11 - 13 ) . indirectly , increasing the concentrations of ldl due to the abuse of anabolic steroids may lead to an increase in sensitivity of platelets ( 14 ) . long - term effects of these agents can be observed in the cardiovascular system , mental health , and increasing the risk of neoplasm ( 15 ) . a ) upper arrow : left anterior descending artery ( lad ) , lower arrow : left circumflex artery ( lcx ) ; b ) after percutaneous coronary intervention ( pci ) and stent placement in lad . trenbolone acetate is a synthetic anabolic steroid ( 16 ) often referred to as " fina " by users , because the injectable trenbolone acetate was originally adapted for use by bodybuilders from the dissolution of finaplix h pellets ; it is an ear implant used by cattle ranchers to maintain the weight of cattle during shipping to slaughter . trenbolone is a steroid used by veterinarians on livestock to increase muscle growth and appetite ( 17 , 18 ) . because these drugs have remarkable effects on bulking muscle mass and burning fats , illicit use of them therefore , it seems to be necessary that a comprehensive history of steroid consumption in young patients present to the emergency department with the chief complaint of chest pain or its equivalents should adjunct to other cardiac risk factors . when young athletes with a history of anabolic drugs refer to the emergency department with complaints of chest pain , ischemic heart issues must be considered . without the necessary symptoms to exclude ischemic heart problems , lower all authors passed four criteria for authorship contribution based on recommendations of the international committee of medical journal editors .

over the four decades , a significant decrease has been observed in age - related mortality caused by cardiovascular disease . people in developing countries suffer from cad at a relatively younger age and about half of mi occurs under the age of fifty years . abuse of anabolic steroids is one of the less common causes of atherosclerosis . in this report , a 23-year - old body builder male referred to emergency department ( ed ) with myocardial infarction ( mi ) following chronic trenbolone acetate consumption . it seems that a comprehensive history of steroid consumption in young patients referred to ed with the chief complaint of chest pain or its equivalents is necessary in adjunct to other cardiac risk factors .

as an occupation or past - time , fishing is widely practiced around the world . however , surprisingly , despite such extensive prevalence , injuries to the eyes from fish - hooks are rarely reported . a literature search provided less than a dozen cases of fish - hook injuries to the ocular structures . so far , there has been no reported case of actually using the cut - it - out technique for fish - hook removal , even though , it is reported in the literature as an option . thus , this case report focuses on a rather uncommon , though devastating form of injury and on an unusual technique to manage this situation . when they do occur , they can be associated with corneal lacerations and scars , traumatic cataracts , choroidal hemorrhage , vitreous hemorrhage , retinal detachment , and even endophthalmitis . once the fish - hook is impaled in the ocular structures , extraction can become a challenge for the attending surgeon . however , we report a case where the uncommon technique of cut - it - out was successfully employed to remove the fish - hook out of the patient 's eye . an 11-year - old boy was referred to our clinic with a history of injury to the right eye while fishing the day before . apparently while pulling the line , the fish - hook flew to the patient 's right eye and got embedded in the cornea . someone had cut the line and the dangling hook was taped to the cheek [ figures 1 and 2 ] . patient on initial presentation the fish - hook on closer inspection on examination , the visual acuity was a perception of light ( pl ) ; with projection accurate in all quadrants in the affected right eye and 20/20 unaided vision in the left eye . the fish - hook had penetrated the cornea and impaled itself in the deeper tissues . there was a settled hyphema and the deeper tissues were obscured by it and corneal edema . a written consent was obtained from the parents for the removal of the fish - hook under general anesthesia . we planned to use the commonly employed method of advancement of the fish - hook tip outside the cornea and snipping off the tip with wire cutters . however , during the surgery , we found the hook stuck firmly to the underlying tissues . the hyphema and edema were making visualization impossible . on trying to pull the fish - hook out , it got stuck in the cornea . was then pushed in along the shank until the barb could be felt near the cornea . this was then used to guide the fish - hook out of the eye [ figure 3 ] . a subconjunctival injection of dexamethasone and gentamicin was given to complete the procedure [ figure 4 ] . the fish - hook on extraction immediate post - extraction appearance at 1 week of follow - up , the eye showed minimal inflammation [ figure 5 ] . the vision was still pl ; with accurate projection of light in the affected eye . the damage to the iris at the pupillary margin and a traumatic cataract was visible . fishing is an occupation or hobby for a vast number of individuals across the globe . the coastline of malaysia itself extends for 4675 km and fishing is widely practiced here . however , surprisingly , ocular injuries from fish - hooks are rarely seen . a recent search of the literature provided just one report of two cases from malaysia . fish - hook injuries to the eye can involve the eyelids and the anterior- or posterior - segments . there are five methods mentioned for fish - hook removal from the para - ocular or ocular tissues . these include the following : ( i ) advance - and - cut technique . the hook is pushed forward until the tip and barb are outside the eye . subsequently , wire cutters are used to cut the hook between the barb and the bend . ( ii ) back - out or retrograde technique . in this method , the hook is removed by simply backing it out through the entrance wound . as the barb can inflict more ocular damage during this maneuver , it is employed only for barbless fish - hooks . this method is employed for removal of the fish - hook from non - ocular tissues . the barb is covered with the needle to prevent it from entangling in the tissues and both are then withdrawn together . ( subsequently , the hook is backed out of the eye similar to the back - out technique . patients with fish - hook injuries usually have corneal lacerated wounds and penetration of the lens , leading to traumatic cataracts . in our patient also , the hook had entered the cornea and was stuck in the deeper structures . we also wish to perform a cataract extraction for our patient , once the inflammation subsides . as the cornea is usually involved in these injuries , the usual sequel is corneal scarring . unless extensive , other complications reported include choroidal and vitreous hemorrhages , retinal tears , and retinal detachments . endophthalmitis is also known to occur . in one patient , the endophthalmitis developed after three weeks of the injury . the cut - it - out technique for fish - hook removal is an ideal option in cases where visualization is difficult . ultimately , prevention is the key .

an 11-year - old boy was involved in an injury with a fish - hook to his eye . the hook had impaled itself to the cornea and deeper structures . there was associated corneal edema and hyphema , making visualization difficult . in this case , we performed the unusual cut - it - out technique to remove the hook from the eye .

hughes - stovin syndrome is an exceedingly rare disorder characterized by the combination of multiple pulmonary artery aneurysms and deep venous thrombosis ; the cause is unknown . we present a case of a middle - aged man in whom multiple pulmonary aneurysms were demonstrated by ct angiography and dsa . a 40-year - old man was evaluated for bilateral hilar opacities on radiographs by a contrast - enhanced ct of chest . the patient had significant history of bilateral femoral vein thrombosis two years back for which the patient was hospitalized ; the patient also had recurrent episodes of hemoptysis for last one and a half year for which he did not undergo any imaging and which was managed conservatively with antibiotics . the patient had also developed aphthous ulcers in the past year that lasted for two to three weeks . there was no evidence of genital ulcers , uveitis , or skin lesions either historically or on physical examination . cect chest revealed large saccular aneurysms involving the segmental branches of lower lobe arteries bilaterally and right upper lobe artery [ figure 1 ] . the bronchial arteries were hypertrophied ; eccentric , organized thrombi were seen in few aneurysms [ figure 2 ] . pulmonary angiography was performed through the jugular vein as transfemoral cannulation was not possible because of bilateral femoral vein thrombosis which confirmed the presence of multiple pulmonary artery aneurysms . the possibility of incomplete behcet was also considered as the patient did not fulfill the diagnostic criteria of the complete behcet 's syndrome . the patient was put on topical steroids ( as mouth paint for oral ulcers ) , systemic steroids , and azathioprine ( in view of pulmonary vasculitis and aneurysm formation ) ; the steroids were subsequently tapered and withdrawn after 6 months ( aspirin and anticoagulation were avoided in view of hemoptysis ) . more than one year of follow up has documented the resolution of oral ulcers without any fresh recurrence ; there has been no fresh episode of deep venous thrombosis . there was no evidence of enlargement of the pulmonary artery aneurysms on follow - up ct scans . contrast - enhanced ct chest shows bilateral pulmonary artery aneurysms arising from lower lobe arteries with organized eccentric thrombi in the wall mip images of ct angiography demonstrate the hypertrophic bronchial artery collaterals in the mediastinum hughes and stovin first described cases of deep vein thrombosis and multiple pulmonary artery aneurysms with mural thrombi . the typical clinical syndrome is characterized by a stage of thrombophlebites , followed by formation and enlargement of pulmonary aneurysm , and the final stage of aneurysmal rupture with massive hemoptysis and death . many investigators considered this entity as an incomplete form of behcet 's syndrome.[13 ] the pulmonary artery aneurysms develop at the sites of prior thrombosis ; weakening of the vessel wall due to inflammation is the likely mechanism . in our patient , eccentric organized thrombi were well visualized in few of the aneurysms on ct angiography . some authors have reported dilated bronchial arteries on dsa ; they believe that hemoptysis likely results from rupture of angiodysplastic bronchial arteries rather than due to rupture of pulmonary arteries . enlarged , hypertrophied bronchial vessels were clearly visualized in our case as well , which we believe was the cause of recurrent hemoptysis in this case . one author has described multiple bronchial artery aneurysms with a hepatic artery aneurysm in one patient . ct angiography not only allows visualization of vessel lumen ( like pulmonary angiography ) , it also depicts the mural thrombus , vessel wall , and mediastinal structures including the hypertrophied bronchial circulation . the treatment of hughes - stovin syndrome is similar to behcet 's disease - steroids alone or in combination with immunosuppressants . our case emphasizes the overlap between the clinical and radiological features of behcet 's disease and hughes - stovin syndrome .

multiple pulmonary artery aneurysms are seen along with venous thrombosis in hughes - stovin syndrome , which many investigators believe is an incomplete form of behcet 's disease . we present a case of hemoptysis with multiple pulmonary artery aneurysms , femoral vein thrombosis , and oral ulcers with emphasis on its ct features .

regional odontodysplasia ( ro ) is a rare developmental dental anomaly that involves ectoderm and mesoderm derived tissues . the prevalence of this condition is still not clear since the studies reported till date have mainly been based on case reports . although several factors such as local trauma , infection , ischemia , neural damage , and somatic mutations of neural crest cell migration have been advocated , the underlying pathophysiology of the condition remains unclear . the maxillary teeth are more commonly affected , and only four cases of mandibular involvement have been reported so far . the teeth with ro often display a brownish or yellowish discoloration and most frequent clinical symptoms accompanied by this anomaly are failure of eruption and gingival enlargement . radiologically , the affected teeth illustrate hypoplastic crowns and lack of contrast between enamel and dentin is usually apparent . enamel and the dentin are very thin , displaying a ghost - like appearance . the other pathognomic radiological characteristics are enlarged pulp chambers , short roots , and open apices . a 33-month - old boy reported to the department of pediatric and preventive dentistry of our college with the complaint of delayed eruption of teeth in the lower jaw . there was no history of abnormal tooth anomalies on both maternal and paternal part of the family . extra - oral examination revealed normal symmetrical face and normal skin , hair , and nails . the intra - oral examination revealed an adequate maxillary arch with fully erupted primary teeth . all the maxillary teeth appeared normal except for some carious involvements in molars [ figure 1 ] . although all the primary teeth were visible in the mandibular arch , they were not fully erupted and seemed to be embedded in abnormal , slightly hyperplastic alveolar mucosa . furthermore , these teeth revealed abnormal crown morphology with yellowish discoloration and hypoplastic enamel [ figure 2 ] . normal maxillary arch with a full complement of teeth hypoplastic crowns and gingival hyperplasia in entire mandibular arch the orthopantomogram ( opg ) [ figure 3 ] which was taken after detailed clinical examination , showed a full complement of maxillary deciduous teeth and follicles of permanent teeth except for tooth germs of premolars , which was normal for his age . however , in the mandibular arch , all the primary teeth showed a striking ghost - like appearance [ figure 3 ] . the demarcation line between enamel and dentin in these teeth was not clear , and enamel was hypoplastic . none of the permanent tooth germs were seen except for the follicles of first molars . the laboratory investigations showed that the serum calcium , phosphorus , sodium , and potassium levels were in normal range . since the child was only 33 months old , and none of the teeth showed any abnormal mobility , extraction was not carried out . however , gingival biopsy examination revealed the odontogenic tissue in the epithelium and intramesenchymal calcifications . on the basis of clinical and radiographic findings , a diagnosis of ro was proposed . maintenance of proper oral hygiene and regular follow - up examinations for monitoring the developing dentition was advised . considering the age of the child , conservative treatment was preferred over extractions because it preserves oral functionality and esthetics until the end of growth period after which a more definite treatment could be planned . regional odontodysplasia is a rare tooth anomaly that affects maxillary teeth more often with very less tendency to cross the midline . however , cases have been reported with bilateral or multiquadrant involvement . in the present case , clinical and radiographic findings were consistent with that of ro , but this case was notable due to involvement of the entire mandibular arch . till date , only four cases of mandibular involvement have been reported . the cause of the present case also remains unknown because the patient 's past medical history and family history was noncontributory , and no congenital or acquired diseases were reported . regional odontodysplasia has shown to affect both primary and permanent dentition . in the present case , delayed development of permanent tooth germs in the mandibular arch was also evident which was in agreement with some cases reported previously . typical clinical features in this case included gingival hyperplasia and teeth embedded in the gingival overgrowth ; both of these features were consistent with the majority of cases reported previously . a differential diagnosis of ro includes hereditary conditions , such as dentin dysplasia , dentinogenesis imperfecta , and amelogenesis imperfecta as these conditions may present similar features of enamel hypoplasia , abnormal pulp morphology , and calcification . hereditary developmental anomalies of enamel and dentin , however , usually affect the entire dentition rather than segments . dentinogenesis imperfecta type iii bears close resemblance to ro but can be excluded if opalescent dentine , bell - shaped crowns , and family history are absent . factors such as the patient 's age , extent of the lesion , and eruption of the teeth , medical history , and esthetics need to be carefully considered . for the present case , in addition , since the opg showed absence of developing permanent tooth germs , it was thought appropriate to preserve the primary teeth . follow - up examinations were planned to observe the development of mandibular permanent tooth germs . the case presents unique clinical and radiographic features of ro involving the entire mandibular arch in a child aged 33 months . a dental professional must be prepared to manage cases of dental anomalies , providing an opportunity for early diagnosis , adequate monitoring and treatment plans that minimize the after - effects on the patient 's development . in cases of ro , treatment depends on the age of the patient , extent of the involvement of the teeth , and the individual functional and esthetic needs .

regional odontodysplasia ( ro ) is a rare developmental anomaly involving both mesodermal and ectodermal components in primary or permanent dentition . it affects the maxilla and the mandible or both ; however , maxilla is more commonly involved . this article reports the case of 33-month - old boy who came with the chief complaint of delayed eruption of mandibular teeth . findings of clinical and radiographic examination were consistent with those of ro . maxillary dentition was unaffected . clinical and radiographic features and treatment options are discussed .

this manifestation , widely known as post - operative cognitive dysfunction ( pocd ) , is characterized by disordered thinking and impaired consciousness with later onset and fluctuating course [ 24 ] . pocd , as mentioned above , is common in elderly patients , and probably has a pathogenesis similar to that of ad and may even evolve into ad . unfortunately , it has been demonstrated that 41.4% of aged patients have pocd at hospital discharge . leptin is synthesized and secreted by adipocytes , and has been recognized as having an important role in coordinating the peripheral and central signals , ultimately regulating food intake and body weight [ 811 ] . although the biological effects of leptin are thought to regulate eating behavior and energy expenditure , a prospective clinical study with 785 participants showed that higher circulating levels of leptin contribute to reduce ad incidence . a preclinical study has shown that leptin can reduce pathology and improve memory in a transgenic mouse model of ad . collectively , these findings indicate that leptin has unique therapeutic effects on cognitive dysfunction , which is the primary pathological feature of ad . in addition to pocd , it is also characterized by cognitive dysfunction and shares similar pathogenesis with ad . we hypothesized that leptin has prophylactic and therapeutic effects on pocd , and that the leptin signaling pathway may be involved in the pathogenesis of pocd . a previous study by doherty et al . indicated that leptin prevents hippocampal synaptic disruption and neuronal cell death induced by amyloid- ( a ) . a study by marwarha et al . has shown that leptin treatment reversed the 27-hydroxycholesterol - induced increase in a and tau phosphorylation ( p - tau ) . ad , a progressive neurodegenerative disease , is characterized by the accumulation of a peptide - containing neuritic plaques and neurofibrillary tangles composed of p - tau . in this regard , pocd is also characterized by abnormal deposition of a and p - tau . these findings strongly support the hypothesis that leptin may have beneficial effects for the treatment of pocd by down - regulation of a and dephosphorylation of p - tau . amp - activated protein kinase ( ampk ) , a ser / thr kinase , has a critical role in the maintenance of energy metabolism at cellular and body levels . furthermore , leptin is capable of decreasing the levels of tau phosphorylation by activation of ampk in rat cortical neurons . it is widely known that pocd and ad are both aging - related diseases , and slowing the aging process may have therapeutic effects . ampk is a major regulator , which can activate the autophagic pathway , while activation of ampk inhibits mtor , an inducer of autophagy . our recent study proposed a hypothesis that inhibiting mtor activates the autophagic pathway , thereby leading to therapeutic effects for pocd . leptin probably has prophylactic and therapeutic effects in pocd , and the leptin signaling pathway may be involved in the pathogenesis of pocd . further investigations are needed to determine whether leptin has unique effects in the treatment of pocd , and to make certain whether leptin signaling pathway is involved in the pathogenesis of pocd .

leptin plays a critical role in neuronal development and also promotes structural and functional activities in the central nervous system . recent studies have demonstrated that leptin could produce therapeutic effects for cognitive impairments of patients with alzheimer s disease ( ad ) . post - operative cognitive dysfunction ( pocd ) , defined as a significant dysfunction in cognitive performance for several weeks after surgery , probably has a pathogenesis similar to that of ad . specifically , they are both characterized by cognitive impairment . in this regard , we hypothesized that leptin probably has a therapeutic benefit of alleviating symptoms of patients with pocd , and the leptin signaling pathway may be involved in the pathogenesis of pocd .

it is not a specific disease entity , but a gingival response associated with a variety of conditions . these desquamative lesions represent oral manifestations of various dermatoses which include lichen planus , pemphigus vulgaris , erythema multiform , lupus erythematosus , dermatitis herpetiformis , and chronic ulcerative stomatitis . hence , it is of paramount importance to ascertain the identity of the disease responsible of desquamative gingivitis to establish the appropriate therapeutic approach and management . this paper reports a case of rare desquamative lesion involving the gingiva in a 66-year - old female patient . a 66-year - old female patient reported to department of periodontics , m. s. ramaiah dental college and hospital , bangalore , with the complaint of burning sensation in the gums for the past one month which had started spontaneously , not associated with pain and aggravated on taking spicy foods and relieved gradually . she also had bleeding gums for the past one month , while brushing and eating . her personal history reveals that she had no habits and she used medium brush with non - medicated paste . on extra oral examination , she had some obvious skin lesions on the scalp which appeared as depigmented scarring alopecia of the scalp [ figure 1 ] . depigmentation with scarring alopecia of the scalp hyperkeratotic plaque present on the posterior auricle of the left ear intra oral examination of gingiva revealed reddish marginal , attached gingiva and interdental papilla in both maxillary and mandibular anterior region [ figure 3 ] . desquamation of epithelium was present in the attached gingiva of maxillary right first molar region , mandibular right canine , between maxillary right premolars on the palatal aspect . desquamation of the gingiva -maxillary and mandibular anterior region desquamation present palatal desquamation present lingual nikolsky 's sign - positive the h and e section showed parakeratinized stratified squamous epithelium overlying the connective tissue . the connective tissue showed dense inflammatory infiltrate beneath the basement membrane chiefly composed of lymphocytes [ figure 7 ] . hematological examination included total white blood cell ( wbc ) count , differential white blood count , erythrocyte sedimentation rate , hemocrit , bleeding time , and clotting time were within normal limits . based on the clinical and histopathologic features , lesion was suggestive of discoid lupus erythematosus . hand scaling was performed as gently as possible in order to minimize tissue disruption . hand scaling was preferred , soft tooth brush and mouth rise ( chlorhexidine 0.12% ) was recommended . triamcinolone acteonide oral paste was advised to apply two to three times per day and was recalled for follow - up and maintenance . lupus erythematosus is an auto immune connective tissue disease which consists of discoid lupus erythematosus ( dle ) and systemic lupus erythematosus ( sle ) . one to five percent of patients with discoid lupus may develop systemic lupus erythematosus and 25% of patients with sle may develop typical chronic discoid lesions at some time during their illness . early classic dle lesions typically evolve into sharply demarcated , coin shaped ( i.e. , discoid ) erythematous plaques covered by a prominent , adherent scale that extends into the orifices of dilated hair follicles . the lesions typically expand with erythema and hyperpigmentation at the periphery leaving hallmark atrophic central scarring , telangiectasia , and hypopigmentation . when the adherent scale is lifted from more advanced lesions , keratotic spikes similar in appearance to carpet tacks can be seen to project from the under surface of the scale ( i.e. , the carpet tack sign ) . affected sites include face , scalp , ears , v area of the neck , and extensor aspects of the arms . a symmetric , hyperkeratotic , butterfly - shaped plaque is occasionally found over the malar areas of the face and bridge of the nose . the margins of the lesions are not sharply demarcated , but frequently show the formation of narrow zone of keratinization , hyperemia with edema , superficial , painful ulceration may occur with crusting , but no actual scale formation as is seen on the skin . it is important to remember that patients with lupus erythematosus may be taking , systemic steroids or / and other anti - inflammatory medications such as aspirin or non steroidal anti inflammatory drugs ( nsaid 's ) consequently these patients may be at risk of developing adrenal insufficiency and bleeding disorders . in these situations , oral lesions may be the first clinical manifestation of dle , so proper diagnosis is required for early detection and to establish better treatment planning , this promotes resolution of established lesions and prevents scarring of skin lesions and also , alleviates discomfort of the patients .

desquamative gingival lesions are non - plaque induced inflammatory gingival lesions . it is a clinical description and not a diagnosis . these desquamative lesions represent oral manifestations of various dermatoses . discoid lupus erythematosus is one of the rare dermatoses which show desquamative lesions as oral manifestations . this article presents a rare case report of discoid lupus erythematosus with oral lesions involving gingiva of a 66-year - old female patient .

i wish to point out a need for clarification concerning the methodology utilized in the study eye tracking detects disconjugate eye movements associated with structural traumatic brain injury and concussion by samadani et al . , 2015 . the authors state that binocular eye movements were recorded using a single - camera infrared - based video - oculography technique ( eyelink 1000 , sr research , ontario , canada ) and that binocular disconjugate characteristics were analyzed without calibration of eye orientation . the authors claim that their variance - based disconjugacy metric was sensitive to the severity of a concussive brain injury and to the status of recovery after the original injury . the eyelink 1000 system is an excellent eye tracker with a single high resolution camera and an infrared light source affixed to the camera . a typical recording setup consists of a computer monitor with which the visual stimuli are presented to the subject and the camera unit ( placed in front of the monitor base ) with which the eye movement is recorded monocularly or binocularly . the system has an option of easily outputting image - based , uncalibrated eye coordinates with hundreds of units representing 1 of eye rotation . the concern i would raise with samadani et al.s paper is the unclearness of the relationship between their metric and binocular disconjugacy . logically , for an identical amount of eye rotation , any asymmetry in the spatial relationship that the camera or the infrared light source has with the two fellow eyes would result in different extents of relocation of the images of the pupils or corneal reflections . asymmetries exist because there is a physical separation between the two eyes as well as between the camera and the infrared light source . in addition , although the biometric characteristics of eyes are highly symmetrical within individuals , they are not perfectly symmetrical and a 12% non - conformity in corneal curvature or axial length is not uncommon , which further confounds the relationship between eye rotation and changes in pixel coordinates . moreover , each of the two fellow eyes has its own function that maps pixel movement to the eye rotation , and this mapping is not linear . thus , the arithmetic difference between the uncalibrated coordinates of the two eyes is quite removed from a physical representation of gaze misalignment . beyond the factors associated with the raw data , the analytic methods in the paper it is puzzling why the disconjugacy metric is represented by the variance of the left - right differences after independently averaging for each eye the uncalibrated coordinates over several cycles for a given stimulus position , as opposed to the straightforward variance of the left - right differences at all sample points . furthermore , the ranges of outcome values presented in the series of figures run from 0 to at most 0.25 , but how the value 0 could have been obtained is not clear . the question arises because in the two eyes uncalibrated coordinates there must be a constant bias related to the interocular distance . lastly , what the high end of the outcome range represents is not clear . since one unit in eyelink s uncalibrated data output is smaller than 0.01 of eye rotation , being able to report differences in 0.25 square units or less seems implausible . if the raw data were numerically centered or scaled , the procedure should have been noted in the text . however , these points can be directly addressed by implementing a calibration procedure under monocular viewing 3 , 4 . a comparison between the results from thus calibrated and uncalibrated data , and a demonstration of test - retest reliability could have improved the paper . in summary , the reproducibility of the paper s findings may be challenged simply by the paucity of details in the methodological description . more importantly , however , from the information supplied or cited in the paper it is difficult to evaluate the validity of the potentially interesting conclusion that deficits in conjugacy of eye movements may quantitate physiologic impact of brain injury .

this correspondence points out a need for clarification concerning the methodology utilized in the study eye tracking detects disconjugate eye movements associated with structural traumatic brain injury and concussion , recently published in journal of neurotrauma . the authors of the paper state that binocular eye movements were recorded using a single - camera video - oculography technique and that binocular disconjugate characteristics were analyzed without calibration of eye orientation . it is claimed that a variance - based disconjugacy metric was found to be sensitive to the severity of a concussive brain injury and to the status of recovery after the original injury . however , the reproducibility of the paper s findings may be challenged simply by the paucity of details in the methodological description . more importantly , from the information supplied or cited in the paper , it is difficult to evaluate the validity of the potentially interesting conclusions of the paper .

a gastrointestinal duplication is defined as a spherical structure , with a muscular coat lined by a mucous membrane . they can be found anywhere in the gastrointestinal tract , from the base of the tongue to the anus , most commonly occurring in the ileum ( 35% ) . gastric duplication cysts are a rare phenomenon and account for only 29% of all gastrointestinal duplications . the majority is circular , non - communicating and surrounded by a smooth muscular coat . they can be found anywhere in the stomach , with the majority being located on the greater curvature . gastric duplication cysts are rarely diagnosed in the adult population and occur more commonly in young children , who may present with symptoms of abdominal pain , gastric outlet obstruction or a palpable abdominal mass [ 2 , 3 ] . clinically important ectopic tissues can include gastric , duodenal and pancreatic tissues , as demonstrated in our case report . a 28-year - old female presented to our clinic with a 4-month history of right upper quadrant and epigastric pain exacerbated by food . the patient also reported increased postprandial fullness followed by multiple episodes of nausea and emesis . a computerized tomography ( ct ) scan of the abdomen and pelvis showed a large cystic mass within the antrum of the stomach ( fig . this was confirmed with an esophagogastroduodenoscopy ; however , there was concern that this mass was extending past the pylorus into the duodenum , causing a gastric outlet obstruction . figure 1:an abdominal ct scan demonstrating a cystic mass within the stomach ( black arrow ) an abdominal ct scan demonstrating a cystic mass within the stomach ( black arrow ) the patient underwent an exploratory laparotomy . the mass was contained within the gastric antrum and did not involve other visceral or solid organs . histopathologic examination of the lesion demonstrated a 6-cm cyst with gastric mucosa and well - developed pancreatic tissue , including acinar and ductal elements ( fig . the cyst lumen was focally lined by duodenal type mucosa and was negative for dysplasia or malignancy . figure 2:histological section showing both gastric and duodenal tissues located within the cyst ( a ) , along with pancreatic tissue ( b ) histological section showing both gastric and duodenal tissues located within the cyst ( a ) , along with pancreatic tissue ( b ) . the stomach accounts for 29% of all duplications diagnosed , which is the least common site followed by esophagus colon and ileum . most cases will occur in females compared to males ( 8:1 ) , with the majority of cases being diagnosed in the pediatric population within the first 3 months of life and rarely after 12 years of age [ 2 , 3 ] . preoperative workup can include abdominal ultrasound , ct scan , magnetic resonance imaging and more recently endoscopic ultrasound [ 4 , 5 ] . although gastric duplication cysts are a rare entity , they can present with a multitude of symptoms . adult patients can present with abdominal pain , nausea , vomiting , dysphagia , dyspepsia , abdominal distention and potentially anemia . weight loss can also occur secondarily to abdominal pain and distention as seen in our patient . hemorrhage , perforation , malignancy or complete gastric outlet obstruction can occur based on location or type of ectopic tissue present . although gastric duplication cysts can be found anywhere in the stomach , the most common location is the distal greater curvature . they may communicate with the gastric lumen as described in our case ; however , the majority is non - communicating with a cystic configuration [ 1 , 7 ] . another distinctive feature is that any type of gastrointestinal mucosa can line the cyst . both gastric and pancreatic ectopic tissues can be seen , which are the most common and tend to be the most clinically significant , as patients can develop complications , such as bleeding , peptic ulcer disease or pancreatitis . even more rare are complex duplications , which can present with gastric mucosa and islands of heterotopic tissues such as respiratory , duodenal and pancreatic tissue . multiple treatment modalities have been reported in the literature including enucleation , formation of cystgastrostomy and even endoscopic removal . complete excision is recommended not only for symptomatic relief as seen with a gastric outlet obstruction , but also because of the risk of malignant degeneration . though rarely reported , there have been at least 14 cases of adenocarcinoma diagnosed in gastric duplication cysts after resection in the english literature . if malignant transformation is suspected , surgical resection is the treatment of choice . gastric duplication cysts are a rare entity , which can contain various heterotopic tissues , such as duodenal or pancreatic islands . surgical excision should be performed in patients who can tolerate surgery when the cyst is symptomatic or the risk of malignant degeneration is of concern . gastric duplication cysts should be contained in the list of differentials when evaluating submucosal gastric masses . verbal and written informed consent was obtained from the patient for publication of this case report and any accompanying images . a copy of the written consent is available for review by the editor of this journal .

gastric duplication cysts are an uncommon finding , especially in the adult population . presenting symptoms can be non - specific , but can include abdominal pain , nausea and emesis . in this report , we present a 28-year - old female diagnosed with a communicating gastric cyst with both gastric and duodenal mucosa , along with pancreatic tissue and no evidence of dysplasia or malignancy . the clinical picture , diagnosis and treatment are described and compared to findings in the literature .

ventilator - associated pneumonia ( vap ) develops commonly in mechanically ventilated patients and is a major cause of morbidity and mortality in the intensive care unit . in a study published in this issue of critical care , pelekanou and colleagues investigated the differences in innate and adaptive immune responses in 36 septic patients with vap and 32 patients with sepsis due to other infections , like pyelonephritis , bacteremia , intra - abdominal infection , and community- and hospital - acquired pneumonia . there was evidence of a more pronounced immunoparalysis in patients with vap than in those with other bacterial infections . this was supported by the decreased number of cd3/cd4cells , the increase in monocyte apoptosis , and the lower release of pro - inflammatory cytokines , namely tumor necrosis factor - alpha and interleukin-6 , from monocytes after stimulation with lipopolysaccharide ( lps ) in the group of patients with vap . it is known that anergic monocytes from patients with septic shock showed increased susceptibility to apoptosis when compared with monocytes from normal hosts . patients with vap are more compromised due to various factors like critical illness , malnutrition , invasive interventions , and the loss of anatomic defense mechanisms , some of which may contribute to monocyte unresponsiveness or lymphocyte depletion . the authors report that endotracheal intubation in septic patients without vap was not independently associated with similar numeric and functional alterations in lymphocytes and monocytes . nevertheless , the study might have been underpowered to detect such differences . additionally , one important finding of the study was the observation that septic patients with vap whose monocytes failed to adequately respond to monocyte stimulation had decreased survival rates when compared with those with an increased cytokine release from monocytes . a similar trend was observed in non - vap - related sepsis , but it was not statistically significant . previously published work from this group had demonstrated that early monocyte apoptosis was linked to survival advantage in patients with sepsis due to vap . what remain to be determined are whether a separate mechanism associated with monocyte anergy and enhanced apoptosis exists in vap - related sepsis and how is it related to mortality . one concept that may be useful in trying to answer monocytes exposed to low doses of lps exhibit a decreased responsiveness to subsequent stimulation by endotoxin . endotoxin tolerance has been considered a paradigm of immunoparalysis , which is present not only in sepsis but also in systemic inflammatory response syndrome and other diseases like cystic fibrosis and acute coronary syndrome . endotoxin tolerance could support the theory of vap pathogenesis that is embraced by the authors . gradual exposure of the host to increasing bacterial inocula originating from aspiration of oropharyngeal flora may contribute to a state of immunoparalysis through the mechanism of endotoxin tolerance . although endotoxin tolerance has been implicated in increased susceptibility to secondary infections , a number of studies in experimental models of sepsis have exhibited a protective role of endotoxin tolerance [ 9 - 11 ] . using a model similar to that of endotoxin tolerance , the authors attempted to mimic vap pathogenesis by using augmenting concentrations of gram - negative bacteria to sequentially stimulate ex vivo peripheral blood mononuclear cells ( pbmcs ) isolated from healthy volunteers and assessed their apoptosis parameters . an increase in cd14 monocyte apoptosis was observed when compared with non - stimulated pbmcs and pbmcs that had only a unique bacterial challenge with the highest concentration of bacterial inoculum used . moreover , a question raised by the results is whether lymphocyte depletion , monocyte apoptosis , and monocyte anergy in vap are immunoparalysis markers that could be used as prognostic factors or are underlying dysregulations that contribute to the pathogenesis of vap . although patients in the two groups did not differ significantly in terms of age , disease severity , underlying conditions , diabetes mellitus , corticosteroid use , the presence of other recent infections , or additional factors that may affect the immune response to sepsis , the duration of critical illness prior to enrollment was not reported . it would be reasonable to expect a more frequent occurrence of longer hospitalization , surgery , trauma , neurosurgical conditions , or other critical illness prior to the development of septic shock in the group of patients with vap . thus , it is hard to know whether any of the above - mentioned factors independently contributed to the observed monocyte unresponsiveness , monocyte apoptosis , and cd3/cd4 cell decrease in this group , and more work is needed before those changes are attributed solely to vap . lps : lipopolysaccharide ; pbmc : peripheral blood mononuclear cell ; vap : ventilator - associated pneumonia .

current evidence regarding potentially different host response mechanisms in sepsis according to the type of initiating infection is sporadic . it is possible that alterations in cell populations , variations in effector molecules , and the degree of apoptosis differ between sepsis caused by ventilator - associated pneumonia ( vap ) and non - vap sepsis . vap is one of the most common infections and leading causes of sepsis in the intensive care unit , and mortality remains high . a better understanding of the unique pathophysiologic features of vap is needed in order to develop interventions that target those specific pathways .

chinese food and soups contain monosodium glutamate ( msg ) as the main addictive ingredient . a sensitive individual may suffer from headache , giddiness , sweating , abdominal pain , and urticaria within a few hours of consumption of msg . angioedema may be delayed up to 816 h after the consumption of msg and it may persist for 24 h. this delayed life - threatening effect in the form of angioedema makes diagnosis difficult . a 23-year - old male was brought to the general hospital at mahad , with complaints of difficulty in speaking , inability to swallow saliva , and continuous spitting . he strongly rejected taking sips of water and was afraid of water like a hydrophobic patient . the posterior pharynx could not be visualized , even after repeated attempts with depression of the tongue with a spatula . the uvula was touching the base of the tongue [ figure 1 ] . on arrival , swelling of the uvula and surrounding tissues , almost closing the entry to the pharynx , touching the base of floor of the mouth . his blood pressure was 120/80 mmhg ; pulse was 88 beats / min and regular . his extremities were warm , the electrocardiograph was within normal limits , and spo2 was 98% on ambient air . the patient said that he ate only chinese triple fried rice for dinner the previous night 10 hours earlier . within an hour of eating , he had giddiness , sweating , and itching all over the body which subsided without any medication . two hours earlier he had woken up due to difficulty in swallowing and speaking out a few words . he communicated with his family with hand gestures regarding his inability to speak and swallow . the hemoglobin was 14 mg / dl , white cell count 13,000 per cu mm ( normal 500010,000 ) , eosinophils 1% ( normal 19 ) , neutrophils 90.9% , random blood sugar 135 mg / dl ( normal 70140 ) , and serum ige 917.021 iu / ml ( normal 3188 ) . the patient was admitted and given intravenous crystalline solution of 40 mg methyl prednisolone and was monitored continuously for oxygen saturation . there was no improvement over half an hour , so 0.30 mg of adrenaline was administered as a deep intramuscular injection over the lateral side of the thigh . the patient no longer had drooling of saliva and was able to speak a few words . his throat looked angry around the uvula and surrounding . on account of raised leukocyte count with neutrophilia , the patient was treated with oral amoxycillin with clavulinate . at 16 h after the initiation of treatment , he started normal oral communication and was able to swallow liquid . on the following day , there was a gradual reduction in the size of the uvula and surrounding inflammation . 2 days from admission , the uvula and surrounding structures including the palate returned to normal and he could swallow solids [ figure 2 ] . chinese food contains msg as the main additive ingredient and flavor enhancer . in a graded challenge , in addition to msg , many other food additives , including preservatives such as meta - bisulfate , soya sauce , coloring agents , such as , carmoisine , sunset yellow , tartrazine , scombroidosis , and seafood may stimulate allergic reactions . angioedema of the uvula after ingestion of msg can be fatal unless patients and physicians are aware of unusual reaction to msg . it is prepared by fermentation of carbohydrate sources , such as sugar beet molasses by acid hydrolysis , by the action of micrococcus glutamicus on a carbohydrate and subsequent partial neutralization , or by hydrolysis of vegetative proteins . large amount of msg is used in japanese , chinese , and south asian food preparation . even free glutamate that exists in tomatoes , mushrooms , and parmesan chinese is responsible for chinese restaurant syndrome . the exact etiology of the chinese restaurant syndrome is not known but , animal studies have shown neurotoxic and neuroexcitatory properties of msg in the hypothalamic region of the central nervous system . the delay of uvular swelling for > 8 h such as in our patient can be explained by the time taken for the synthesis and release of hormonal factors from the hypothalamic - pituitary region . a systematic review by obayashi and nagamura evaluating causal relationship between msg and headache was inconclusive and suggested the need of more blinded studies . however , consumption of msg in high concentration without solid food ( as in soups ) was found to be associated with higher incidence of headache and other symptoms . severe reaction to msg , a common active ingredient in chinese cooking may result in fatal outcome if not treated in time . delayed occurrence of serious symptoms are to be expected .

in india , eating chinese food has become very popular . we hereby report a case who presented with angioneurotic edema of the uvula and the surrounding structures , after eating chinese food , which resulted in severe difficulty in swallowing saliva and inability to speak .

a life in science is not a journey you take alone . at all stages , mentors are key . no one in my family had ever been to university , let alone undertaken a ph.d . it was a female high school teacher who encouraged me to set my sights high and apply to oxford university for undergraduate studies , and it was the sister of a school friend who first showed me by example that it was possible to have a career in research . at oxford i attended lectures by john gurdon on frog development and became fascinated by developmental biology . chris graham had just joined the faculty in the department of zoology and gave some lectures on new approaches to studying the mammalian embryo . i joined richard gardner 's lab in the marshall laboratory of reproductive physiology at the university of cambridge for my ph.d . in 1972 . richard had made a big impact with his studies on lineage development in the early mouse embryo , performed by injecting cells into blastocysts , and i became his first graduate student . the marshall lab at that time was really at the center of new developments in technologies to study mammalian development and reproduction , and it was a very exciting and challenging place to work . matt kaufman was developing parthenogenesis in mice , and richard gardner and martin johnson were making interspecies chimeras to follow cell fate in situ . all this was carried out in a very collegial environment where a shy new graduate student was able to mingle and learn from the experts . so when i am asked how students should choose a lab for their graduate studies , i tell them to find an area of science that fascinates them and find a leading lab working in the area , but also to make sure that they will be in a collegial working environment where people are ready and willing to talk and share expertise . after my ph.d . and postdoc in cambridge and oxford , i married a canadian , whom i met through a college rowing team in cambridge , and moved to canada . this was a leap into the unknown . moving to canada without a job was probably a dangerous way to start a marriage and definitely not the best way to start my academic career . however , i rapidly obtained my first faculty appointment and over the years have built and sustained my research program in the canadian system ( and remained happily married ! ) . success was not without its challenges , especially in the early years , and the importance of mentors comes to the fore again . richard gardner introduced me to verne chapman in buffalo and tom wegmann in the university of alberta , both of whom were inspiring scientists and fantastic mentors . they went out of their way to forge collaborations with me and to get me invited to meetings , grant panels , etc . this enabled me to expand my network of contacts and allowed my science to thrive and make an impact . verne and tom both died too young , but their selflessness in promoting me has been my touchstone as i try to guide the careers of people around me . so far this look back at some of the role models and mentors in my career has largely focused on men , but men who were completely gender - blind in their support of good science . but developmental biology in general and mammalian development in particular have always had a very high quotient of successful women at all levels , who provide role models for aspiring young scientists . there is no question that this preponderance of females makes it easier for a woman to feel that this is an area of research where she can be accepted and made welcome . a positive feedback loop is thus set up , and the field continues to recruit strong females to its ranks . joining the research profession is challenging and tough , and it is important to have support networks where problems can be shared and worked through . i have always tried to give back to the community of science that has been so good to me and am continuing this path in my current endeavor as head of one of canada 's biggest and most successful hospital - based research institutes at the hospital for sick children . it is a very challenging opportunity to try to make a difference for a broad range of scientists and clinician - scientists and to have real impact on major problems in children 's health and , of course , one more challenge in terms of being able to balance science , administration , outside commitments , and family . i was told the other day that i should write a book on how to be a successful scientist and leader who gets things done . of course , that immediately made me think of all the things i have not managed to get done ! in the end , nobody can do it all , but i have a few small pieces of advice in lieu of an entire book . first , compartmentalize your activities as much as possible and try not to let one activity take over the time set aside for the other . we are extraordinarily privileged to work in this era of great scientific discovery and to be able to contribute in whatever way we can . i can go anywhere in the world and immediately have a point of contact and common values with people there . i strongly believe that science has a major role to play in promoting harmony and peace across the world . educating world citizens in common cause to pursue knowledge and apply it to human welfare

a career in science is a journey of wonder and discovery . to succeed in science requires curiosity , perseverance , a good dose of luck , and wise guidance from those who have taken the journey ahead of you . we also need to use our science skills to contribute to public debate on complex issues of the day .

the transcriptome of medullary stromal cells of patients suffering from primary myelofibrosis was studied by agilent oligo microarray technology . the primary myelofibrosis is a chronic myeloproliferative syndrome . to invest the role of bone marrow stroma in the pathophysiology of this disease , we isolated primary cultured of bone marrow stromal cells from these patients . the osteo - medullary biopsies for the diagnosis of the disease were implanted in dmem medium with 10% fetal calf serum . stromal cells during their proliferation adhere to plastic and they were trypsinized between each passage ( 35 passages ) when cultures came to confluence . a cytometric control was carried out on the cells prior to performing molecular biology experiments . cd105 , cd73 and cd90 marker positivity was verified to validate the mesenchymal cell phenotype . the negativity of the cd45 marker was also carried out to prove the absence of residual hematopoietic cells in culture . each culture of mesenchymal stromal cell is isolated from the bone marrow of an individual . in total , the bone marrow samples were studied individually from 6 healthy donors ( 6 controls : gsm1084994 , gsm1084995 , gsm1084996 , gsm1084997 , gsm1084998 , gsm1084999 ) and from 6 patients with primary myelofibrosis ( 6 pmf : gsm1085000 , gsm1085001 , gsm1085002 , gsm1085003 , gsm1085004 , gsm1085005 ) . concerning the enrollment of control samples : subjects are negative for alcohol abuse and hcv , hbv and hiv virus infections . the choice of control subjects was conditioned by access to bone marrow from subjects having a hip prosthesis surgery : indeed the subjects have an average age similar to that of patients with primary myelofibrosis ( between 60 and 80 years ) . each sample was treated individually for the extraction of nucleic acids and the achievement of microarrays . rna was isolated using rna extraction protocols ( nucleospin rna ii , macherey - nagel ) on the miltenyi plateform . rna samples were quality - checked via the agilent 2100 bioanalyzer ( agilent technologies ) . total rna sample ( 1 g ) rna samples were amplified and labeled using the agilent quick amp labeling kit / low rna input linear amp kit ( agilent technologies ) . the hybridization procedure was performed using agilent gene expression hybridization kit ( agilent technologies ) . briefly , 1.65 g cy3-labeled fragmented crna in hybridization buffer was hybridized overnight ( 17 h , 65 c ) to agilent whole human genome oligo microarrays 4 44k using agilent 's in hybridization chamber . the fluorescence signals were detected using agilent 's microarray scanner system ( agilent technologies ) . the agilent feature extraction software ( fes ) v9.1 was used to read out and process the microarray image files . the signal intensities from single experiment raw data lists are normalized by dividing the intensities values by their median . normalized data were accessible on public database : ( geo submission number gse44426 , http://www.ncbi.nlm.nih.gov/geo/query/acc.cgi?acc=gse44426 ) , online on jan . 10 , 2015 ) , .

primary myelofibrosis ( pmf ) is a clonal myeloproliferative neoplasm whose severity and treatment complexity are attributed to the presence of bone marrow ( bm ) fibrosis and alterations of stroma impairing the production of normal blood cells . despite the recently discovered mutations including the jak2v617f mutation in about half of patients , the primitive event responsible for the clonal proliferation is still unknown . in the highly inflammatory context of pmf , the presence of fibrosis associated with a neoangiogenesis and an osteosclerosis concomitant to the myeloproliferation and to the increase number of circulating hematopoietic progenitors suggests that the crosstalk between hematopoietic and stromal cells is deregulated in the pmf bm microenvironmental niches . within these niches , mesenchymal stromal cells ( bm - msc ) play a hematopoietic supportive role in the production of growth factors and extracellular matrix which regulate the proliferation , differentiation , adhesion and migration of hematopoietic stem / progenitor cells . a transcriptome analysis of bm - msc in pmf patients will help to characterize their molecular alterations and to understand their involvement in the hematopoietic stem / progenitor cell deregulation that features pmf .

a.t . , a 43-year - old nigerian lady , presented to the eye clinic of the university college hospital , ibadan with gradual blurring of vision in both eyes for 4 years . ocular examination showed unaided visual acuity of 6/12 ou corrected to 6/9 in both eyes . dilated fundoscopy showed a well circumscribed cystic fovea lesion with a fluid level and an atrophic center in the right eye , while the left eye showed a scar in the macula ( fig . 1 and fig . the patient was prescribed with spectacles , counseled and placed on close observation with amslers grid for the development of choroidal neovascular membrane that will require urgent treatment . this is probably the first case of best macular dystrophy to be reported in ibadan , nigeria and possibly sub - saharan africa to the best of the author 's knowledge . it has also been reported in caucasians and asians , the other cases seen in african americans were associated with sickle cell trait . the pathophysiology of best 's disease is explained by abnormality in the retinal pigment epithelium ( rpe ) with resultant abnormal ionic transport leading to the accumulation of lipofuscin in the rpe cells and sub - rpe space in the macular area . the vitelliruptive stage may herald visual deterioration which becomes worse in the atrophic stage due to the presence of choroidal neovascular membrane . our patient presented with the early stages of the disease , hence the good visual acuity . the patient will be under observation and follow - up so as to detect changes amenable to treatment . choroidal neovascular membrane ( cnvm ) from best 's disease has been reported to respond well to intravitreal antivegf [ 7 , 8 ] . fundus flourescein angiography is essential in confirming the presence of cnvm and should be done when patient presents with sudden deterioration in vision . if a cnvm develops , then a corresponding area of hyperfluorescence with leakage will be found . since the disease is an autosomal dominant disorder , other family members will benefit from regular fundus examinations , and amslers grid is a valuable tool for monitoring central vision . an electrophysiologic test such as electrooculogram ( eog ) is specific for confirming the presence of the disease in relatives even in the absence of clinical signs and symptoms . a severe decrease occurs in light response , reflected by an arden ( light - peak / dark - trough ) ratio of 1.11.5 ( the normal arden ratio is 1.8 ) . the full - field electroretinogram ( erg ) result is normal in best 's disease . a focal erg or multifocal erg , concentrating on macular function , reveals abnormal function corresponding to the area of anatomical disruption . the pathophysiology of best 's disease is explained by abnormality in the retinal pigment epithelium ( rpe ) with resultant abnormal ionic transport leading to the accumulation of lipofuscin in the rpe cells and sub - rpe space in the macular area . the vitelliruptive stage may herald visual deterioration which becomes worse in the atrophic stage due to the presence of choroidal neovascular membrane . our patient presented with the early stages of the disease , hence the good visual acuity . the patient will be under observation and follow - up so as to detect changes amenable to treatment . choroidal neovascular membrane ( cnvm ) from best 's disease has been reported to respond well to intravitreal antivegf [ 7 , 8 ] . fundus flourescein angiography is essential in confirming the presence of cnvm and should be done when patient presents with sudden deterioration in vision . if a cnvm develops , then a corresponding area of hyperfluorescence with leakage will be found . since the disease is an autosomal dominant disorder , other family members will benefit from regular fundus examinations , and amslers grid is a valuable tool for monitoring central vision . an electrophysiologic test such as electrooculogram ( eog ) is specific for confirming the presence of the disease in relatives even in the absence of clinical signs and symptoms . a severe decrease occurs in light response , reflected by an arden ( light - peak / dark - trough ) ratio of 1.11.5 ( the normal arden ratio is 1.8 ) . the full - field electroretinogram ( erg ) result is normal in best 's disease . a focal erg or multifocal erg , concentrating on macular function , reveals abnormal function corresponding to the area of anatomical disruption . this is a report of best macular dystrophy in ibadan , sub - saharan africa .

best macular dystrophy is reported to be rare in africans . it is a hereditary disease that starts in childhood and progresses through some stages before visual symptoms occur . this case report presents a 43-year - old nigerian with the disease and stresses the importance of regular eye exams of patients and relatives to detect changes such as choroidal neovascular membrane amenable to treatment .

a healthy 60-year - old male with a 30 pack - year smoking history presented with 2 months of right eye pain , headache , and photophobia . his ocular history was significant for pterygium excision with mitomycin c in the right eye 5 years ago as well infiltrative infectious scleritis secondary to enterobacter cloacae and curvularia fungus species 2 years ago . his first episode of the right eye infectious scleritis presented as scleral thinning 3 mm nasal to the limbus with overlying infiltrates that resolved with topical moxifloxacin and natamycin over the course of 3 months . however , a residual calcified plaque remained in the area of scleral thinning . two years later , the patient returned complaining of several weeks of worsening pain and photophobia in the right eye . on examination , his best - corrected visual acuity was 20/25 in the right eye and 20/20 in the left eye . examination of the right eye revealed soft , yellow - white purulent material overlying the existing calcified plaque with extensive scleral thinning [ fig . 1 ] . slit - lamp photograph of the right eye 2 years after initial presentation , now with an elevated mass of purulence overlying the area of scleral thinning a limbal peritomy was performed to define the areas of scleral thinning , and the purulent material was noted to be adherent to the calcific plaque . the plaque and purulent material were excised with gentle traction and sent for culture and histopathology . to avoid scleral perforation , a tutoplast scleral graft was sutured over the scleral defect and covered with a conjunctival flap . initially , the patient was treated with hourly topical moxifloxacin , fortified tobramycin , and natamycin . gomori methenamine silver stains of the purulent material eventually revealed a marked amount of branching fungal hyphae ( indicated by arrow ) with cultures growing pseudomonas aeruginosa and bipolaris fungus [ fig . 2 ] . gomori methenamine silver stain of purulent area demonstrating numerous branching hyphae ( arrow ) indicative of fungus nine days after the procedure , the patient developed moderate discomfort in the right eye . examination revealed a retracted conjunctival flap with further purulent material overlying the scleral patch graft . a decision was made to inject subconjunctival voriconazole , and qid dosing of topical cyclosporine 2% plus oral ketoconazole ( 400 mg daily ) was added to his treatment plan . after 10 weeks of treatment , the patient 's symptoms had resolved , and vision returned to baseline [ fig . 3 ] . once clinical evidence of infection resolved , medications were discontinued . slit - lamp photograph of the right eye on postoperative month 3 with epithelialization of the scleral patch graft and resolved infection in our patient , aggressive medical and surgical management resulted in complete resolution of the infection . surgical intervention was elected due to the chronicity and recurrence of the lesion and due to the possibility of impending scleral perforation . in vitro strains of dematiaceous fungi have shown to respond well to antifungals such as natamycin and amphotericin b. hence , a suggested clinical treatment modality includes topical natamycin 5% supplemented with oral ketoconazole . keratitis has been shown to resolve in over 70% of cases with dematiaceous fungi , but poor penetration of antimicrobials through the collagen - bound scleral layer poses a challenge for effective treatment . the persistence of organisms such as pseudomonas species and fungal hyphae in the sclera despite aggressive medical therapy has been reported . for such cases of infectious keratoscleritis that are refractive to medical treatment such as this particular case , the best option may be to include surgical management such as excision with lamellar corneoscleral graft and/or cryotherapy . infectious scleritis typically presents as an extension of keratitis resulting from ocular injury , often from trauma , foreign bodies , radiation , or surgery . infectious scleritis following pterygium removal has been reported , and is often related to postoperative beta irradiation and/or intraoperative antimetabolites . the events resulting in an infection involve direct pathogenic invasion of the sclera that triggers an immune - mediated vasculitis and necrosis at the site of pterygium excision . hence , if a presumed inflammatory scleritis does not respond as expected to steroid treatment , an infectious etiology should be considered . such cases can be diagnosed through smear / culture , scleral biopsy , or anterior chamber aspirate . poor prognosis in infectious scleritis includes corneal involvement , inappropriate antimicrobial therapy , and presence of fungal infection . thus , mycotic scleritis should always be considered in the differential diagnosis , and prompt diagnoses and treatment are critical to salvage vision . to our knowledge , this is the first report of infectious scleritis without corneal involvement caused by the bipolaris species .

we report an interesting case of infectious scleritis from coinfection of pseudomonas aeruginosa and bipolaris with no corneal infiltrate . a healthy 60-year - old man with a history of infectious scleritis following pterygium excision presented with purulent material growing p. aeruginosa and 1 + colonies of bipolaris species of fungus . broad spectrum treatment was initiated with hourly topical moxifloxacin , fortified tobramycin , and natamycin along with a subconjunctival injection of voriconazole and topical cyclosporine , with po ketoconazole . after 10 weeks of aggressive empiric treatment , the patient 's symptoms had resolved , and his vision returned to baseline although a scleral patch graft was utilized to stabilize scleral thinning .

extrarenal calyces are rare anomalies of the upper urinary tract . only 20 cases were reported so far in the literature . the rarity of this anomaly and the complexity of possible associated anomalies often makes the preoperative diagnosis difficult . they are known to be associated with other anomalies , including bifid kidney and renal dysplasia . to the best of our knowledge , only 3 cases have been reported in the literature wherein , it was associated with pelviureteric junction obstruction ( pujo).[24 ] when associated , management options depend on the renal function and presenting symptoms . herein , we are reporting a case where routine preoperative imaging did not throw any light on the diagnosis and was an intraoperative surprise . a 9-year - old male child presented to us with complaints of pain in the left flank for 1 month . an ultrasound of the abdomen showed severe hydronephrosis of the left kidney with a parenchymal thickness of 2 mm with upper ureteric dilation . a radionuclide scan showed a poorly functioning left kidney with a split renal function of 7% with obstructed curves . an ante grade dye study was done , which showed significant stasis in the hydronephrotic kidney and the ureter was not visualized . intraoperatively , a large dilated kidney with papery thin cortex was found with 5 pseudopodia - like projections ( extrarenal calyces ) arising from it and connected to the pelvis ; pujo was noted . the rest of the ureter appeared normal . in view of the complex nature of the lesion and poorly functioning kidney , a right nephroureterectomy was performed [ figure 1 ] . the presence of extrarenal calyces is a very rare anomaly of the upper urinary tract that was first described in 1925 . a few cases were reported in the literature and the exact etiology of this condition is unclear . it has been hypothesized that the anomaly could be due to a disparity resulting from slow development of the metanephric tissue or to a relatively rapid development of the ureteric bud . a kidney with extrarenal calyces is usually associated with other anomalies , such as bifid kidney , renal ectopia , horseshoe kidney , and renal dysplasia . it might be an incidental finding diagnosed at autopsy or may present with complications , such as hydronephrosis secondary to associated pujo , as in our case . because the incidence of this anomaly is quite low and the clinical presentation is very similar to classic pujo , a high degree of suspicion should be kept , especially when preoperative radiologic findings are not classical and show distorted calyceal appearance on preoperative imaging studies . this would safeguard against inadvertent injury of the calyces when operating on a well - functioning kidney . but , when they are associated with complications , such as pujo , as in our case , the management is not standardized . ( 2006 ) mentioned the successful management of pujo associated with extrarenal calyces in a pelvic kidney using transperitoneal laparoscopic reconstruction . the management options may include the following : ( 1 ) to do a classic pyeloplasty and follow the patient , risking failure of pyeloplasty and recurrent urinary tract infection ( uti ) ( as was done in the previous case ) ; ( 2 ) to resect the pelvis and do a ureterocalicostomy with fusion of the calyces ( again associated with extensive dissection and suturing ) ; or ( 3 ) to go ahead with a single - stage nephroureterectomy , thereby preventing the child from further recurrent uti . the management strategies are controversial and should be decided on individual merits and surgeon competence . however , in kidneys with well - preserved function , a renal sparing procedure is worth attempting .

extrarenal calyces are one of the rare renal anomalies associated with the collecting system . their association with renal ectopia or horse shoe kidney is known . but , here in , we are reporting an intraoperative surprise , where extrarenal calyces were associated with pelviureteric junction obstruction and routine preoperative imaging did not show any light on the diagnosis .

intracranial dermoid cysts and colloid cysts are rare congenital lesions generally occurring along the midline . dermoid cysts are known to be ectodermal in origin while debate still exists about the embryological origin of colloid cysts . here , we report a 22-year - old young male with suprasellar dermoid cyst associated with a colloid cyst of the third ventricle and discuss the possibility of their unified embryological derivation . to best of our knowledge , a 22-year - old male patient presented with on and off frontal headaches and occasional blurring of vision for 1 year . clinical examination was normal with visual acuity of 6/6 in both eyes without evidence of any field defects on perimetry . magnetic resonance imaging ( mri ) revealed mainly cystic lesion in the suprasellar region with small solid component anteriorly which was hyperintense on t1-weighted sequences without any contrast enhancement . a colloid cyst of 1.2 1.5 cm was seen at the foramen of monro , mainly occupying third ventricle with evidence of mild hydrocephalus [ figure 1a ] . an old bifrontal craniotomy was re - explored and complete excision of suprasellar dermoid cyst was achieved through bilateral subfrontal approach along with transcortical transventricular excision of colloid cyst in the same setting . intraoperatively , suprasellar cyst was containing a whitish fluid with fat globules with small solid vascularized fatty nodule . post - operative scans showed complete excision of both the lesions [ figure 1b ] . sagittal sections of contrast - enhanced magnetic resonance images of brain showing cystic lesion in the suprasellar area with small solid part anteriorly . post - operative scans ( 48 hrs ) scans showed complete excision of dermoid and colloid cyst with decreased mass effect on the optic chiasm ( b ) photomicrographs showing stratified squamous epithelium with underlying sebaceous glands ( a ) . colloid cyst wall comprising fibrocollagenous wall and is lined by columnar epithelium ( c ) with high - power view of lining epithelium ( d ) . ( a , b : hande 100 ; c , d : h and e 200 ) intracranial dermoid cysts generally occur along the midline and are derived from the trapped somatic ectoderm during embryological development during third to fifth week of gestation . these cysts are generally associated with dermal sinus tracts and other neural tube closure defects ; however , dermal sinus tract may be absent in suprasellar and cerebello - pontine angle dermoids . these lesions mainly presents with headache , local sings of mass effect , or neural compression . colloid cysts of the third ventricle are also rare benign midline lesions presenting during early adulthood . colloid cyst generally become symptomatic in early adulthood and mainly presents with headache and hydrocephalus . although controversy exists about timing of surgery of asymptomatic colloid cysts , symptomatic ones are excised surgically at the earliest . with advent of endoscopy , complete or partial resection of colloid cyst congenital origin of these cysts is accepted universally ; however , cell of origin is highly debated . initial reports suggested their neuroepithelial origin as the derivatives of paraphysis or ventricular ependyma and these were further supported later by other studies . however , mackenzie in 1991 suggested the endodermal derivation of these cysts on the basis of ultrastructural features which was supported by other authors . until recently endodermal derivation of these cysts enjoyed the favor when nagaraju et al . , in 2010 reignited the debate by claiming colloid cysts as paraphysis remnants in human . colloid cysts are associated with various developmental malformations like corpus callosal agenesis , anterior cranial fossa encephalocels , cavum veli interpositi , and multiple neuroepithelial cysts and aqueductal stenosis . these malformations represent a spectrum of neural tube closure defects and associated anomalies . although isolated sporadic form of colloid cyst is most common occurrence , these associations suggest disordered embryogenesis as a possible mechanism in their formation . cheng et al . , in 1999 reported a case of nasal dermoid sinus cyst associated with colloid cyst of third ventricle and proposed a fascinating theory of anterior neuropore corridor defects to explain the spectrum of these associated rare midline lesions . according to this theory , defective closure of situs neuroporicus promotes the neuroepithelium adjacent to commissural plate derivatives to form a vacuolated paraphysis growing slowly as colloid cyst of the third ventricle . similar derivation from paraphysial remnants was also reported by nagaraju et al . , in 2010 supporting cheng 's hypothesis . although lack of substantial evidence remained main drawback of this theory , disordered embryogenesis appears to be the most plausible explanation in our case rather than mere coincidence . suprasellar dermoid cyst associated with colloid cyst of the third ventricle is a rare presentation . this association appears more than coincidence and can be partially explained by anterior neuropore corridor defect theory .

intracranial dermoid cyst and colloid cysts of the third ventricle are rare benign congenital lesions of early adulthood . both lesions are thought to be congenital in origin however association is rare . only one case of this association has been reported . we report a 22-year - old male with suprasellar dermoid cyst and colloid cyst of the third ventricle presenting simultaneously . embryogenesis of this association has been discussed .

henoch schnlein purpura ( hsp ) belongs to the group of nongranulomatous small vessel vasculitis . intussusception , gangrene of the bowel , bowel perforation , and massive hemorrhage are the most common gastrointestinal complications of hsp . a comprehensive search of pubmed , embase , and web of science was performed for all relevant papers published before july 1 , 2015 . because the full text of six articles was unavailable , only 13 cases described in 12 full - text articles were included in this study . then , we investigated the clinical features , treatments , and prognoses of hsp - related pancreatitis cases . the main clinical characteristics of the 13 patients ( six males , seven females ) are summarized in table 1 . the patients ranged in age from 3 to 70 years , half of cases were 520 years old . pancreatitis presented as the initial manifestation of hsp in eight cases . in addition , pancreatitis and typical purpura occurred at the same time in the two cases . from the onset of pancreatitis to the diagnosis of hsp , the time elapsed ranged from 1 day to 75 days , and 62.5% ( 5/8 ) of the patients began to have typical purpura within 7 days of the onset of pancreatitis . in addition to abdominal pain in all cases , seven patients presented with vomiting , one patient had hematemesis , one patient had hematochezia , and one patient had poor appetite . iu / l ) in all cases . abdominal computed tomography ( ct ) revealed pancreatic edema , ascites , and a wide range of intestinal wall edema in seven cases . a pancreatic cyst was found in one patient , it appeared over the course of 35 days and disappeared in 55 days ; however , five patients did not demonstrate any morphological changes in the pancreas . all patients experienced relief through treatment with fasting , gastrointestinal decompression , nutritional support , antiacid drug , glucocorticoid , and somatostatin . the abdominal pain relief time ( 144 days ) was noted in six cases . patient prognoses were described in three cases , and these patients were cured without recurrence through follow - up . main clinical characteristics of 12 previously reported cases of henoch - schnlein purpura - related pancreatitis hsp occurs about twice as often in boys as in girls , half of affected patients are younger than 10 years of age . however , our study suggested that hsp - related pancreatitis usually occurs in adolescent girls . the reason remains unclear ; it may be associated with the immune state of adolescent girls . compared with other types of pancreatitis , hsp - related pancreatitis has the following characteristics . in our study , before the onset of hsp - related pancreatitis , there was no prominent cause , such as biliary tract disease , overeating , drinking , hyperlipidemia , viral infection , or drugs . we speculate that hsp - related pancreatitis may be associated with small blood vessel thrombosis , vasculitis , and intimal thickening . although the clinical manifestation was relatively mild , acute pancreatitis may be the initial manifestation of hsp . hsp - related pancreatitis is diagnosed clinically but requires ct evaluation or ultrasound imaging to differentiate mild acute pancreatitis from severe necrotic pancreatitis . in our study , approximately 41.7% ( 5/12 ) of the patients with pancreatic morphology were normal , and only 41.7% of the patients presented with pancreas swelling . the imaging changes in hsp - related pancreatitis were atypical , and pancreatic necrosis or pseudocysts and other local complications were relatively rare . in our study , all patients had elevated serum amylase and urine amylase levels , with an increase of at least three times the upper limits in blood and urine , but the level was not positively correlated with the disease severity . some researchers believe that elevated amylase creatinine clearance and serum lipase levels are appropriate for the early diagnosis of pancreatitis . it has been reported that the measurement of plasma factor xiii could be useful for the early diagnosis of hsp , particularly when the typical purpura is preceded by abdominal pain . in addition to the conventional treatment for pancreatitis , more attention should be paid to hsp . in our study , the symptom of pancreatitis was improved after steroid treatment in all patients . somatostatin can inhibit gastric and pancreatic secretions , thereby reducing enzymatic activity ; it can also reduce capillary permeability , open the sphincter of oddi , and promote the excretion of pancreatic enzyme . after a diagnosis of hsp - related pancreatitis in addition to the symptomatic and supportive treatment , the use of corticosteroids to control hsp helps to alleviate hsp - related pancreatitis .

to summarize the experience of diagnosing and treating patients with henoch schnlein purpura ( hsp)-related pancreatitis , a systematic review of previously published cases was conducted . among 13 reported cases , there were six males and seven females whose age from 3 to 70 years . the clinical features of these patients indicated that acute pancreatitis could be the initial manifestation of hsp , the radiological change was atypical , and most cases were alleviated with steroidal treatment . good outcomes can be achieved in patients who are diagnosed early with hsp - related pancreatitis , and it is vital to begin timely treatment of hsp - related pancreatitis with corticosteroid .

vitiligo is the most common depigmenting disorder , with a worldwide occurrence of 0.1 - 2% in general population . multiple conditions have been described colocalized in vitiligo patches , like psoriasis or lichen planus . however , actinic granuloma has not been described in association with vitiligo lesions so far . actinic granuloma was first described by o'brien in 1975 as an uncommon disease , most frequently presented in middle - aged women [ 6 , 7 ] . some authors consider actinic granuloma a subtype of annular granuloma and others a different entity . we report the case of a 50-year - old woman , who was referred to our dermatology department in 2006 with a diagnosis of lip - tip vitiligo . the milky patches were located in the face , on the dorsum of the hands , and on the forearms and legs . no diagnosis of lupus , sarcoidosis or other dermatologic diseases could be elucidated in her medical or family history . she was treated with topical steroids ( clovate cream ) and topical tacrolimus ( protopic 0.1% ointment ) , with poor response in achral lesions . oral puva therapy with 8-mop was started in 2007 , obtaining a moderate repigmentation in distal areas . in june 2009 , during a routine follow - up , the patient complained that non - pruriginous lesions had appeared in her hands 2 weeks ago . on physical examination , she presented with multiple asymptomatic , erythematous , annular plaques and solitary papules 5 - 10 mm in diameter , located on the dorsum of both hands , strictly confined to vitiliginous lesions . full blood count , liver and renal function and urine analysis were all in normal ranges . based on the clinical picture , presumptive diagnosis of coincident vitiligo and annular granuloma was made . fibers phagocyted by the histiocytes were seen , too . besides , absence of melanocytes in fontana - masson staining was demonstrated . several articles have suggested that annular and actinic granuloma are different conditions based on histopathological characteristics . different terms have been used to designate this disorder : annular elastolytic giant - cell granuloma ( aegcg ) , o'brien granuloma , multiple [ 10 , 11 ] , actinic [ 7 , 8 ] or elastolytic granuloma . multiple granuloma was coined by leiker in 1964 , and it is clinically similar to actinic granuloma , being considered the same entity in some studies . a photo - mediated etiology has been proposed in the literature for this subtype [ 10 , 11 ] . o'brien suggested the role of infrared and ultraviolet actinic radiation [ 6 , 7 , 13 ] . the importance of a granulomatous chronic inflammation in association with another systemic illness such as sarcoidosis or diabetes mellitus was proposed by hanke . cd4 lymphocytes would act against the solar - damaged elastic fibers , which could be recognized as antigenic products [ 6 , 7 , 13 ] . histological characteristics like sarcoidal granuloma or multinucleated giant cells suggest an inflammatory response to small products , like altered elastic fibers . the confinement of the inflammatory reaction to the superficial dermis , corresponding to the area of solar elastosis , supports the actinic theory . this photo - mediated damage could be considered as a trigger factor in our patient and could explain the strict colocalization of elastolytic granuloma on vitiligo patches . vitiliginous skin is more photo - sensible and the actinic damage is more intense than in the rest of the body . in this case , the affected skin on the dorsum of the hands was not only prone to photo - damage as a result of sun exposure , but it was also selectively exposed to puva therapy over the last 2 years in our patient . in this case , we present an uncommon clinical feature with strict colocalization of elastolytic granuloma in vitiliginous areas . the location of the lesions strongly supports the photo - damage hypothesis in our patient . moreover , the patient received puva and sun therapy as an important part of her treatment . to our knowledge , this is the first patient with coexistence and strict anatomical coincidence of actinic granuloma and vitiligo reported in the literature thus far . we conclude that the actinic radiation played an important role in this form of granuloma in our patient . however , further studies to clarify pathogenetic links between vitiligo and actinic granuloma would be necessary .

vitiligo is the most common depigmenting disorder , with a worldwide occurrence of 0.1 - 2% in the general population . multiple conditions have been described colocalized in vitiligo patches , like psoriasis or lichen planus . however , actinic granuloma has not been described in association with vitiligo lesions so far .

a coronary artery fistula ( caf ) is an abnormal communication between one of the coronary arteries and a cardiac chamber or vessels , including the coronary sinus , pulmonary artery , or superior vena cava.1)2)3 ) caf is involved in 0.002% of general population and accounts for 0.4% of all cardiac malformation.4 ) caf mostly drains into the venous structures of circulation , such as the right - sided chambers , pulmonary artery , coronary sinus and superior vena cava , but drainage into the left - sided chambers is less frequent.5 ) we present a case of caf which is originated from left coronary artery and drained into left ventricle . a 48-year - old male presented with abdominal pain that had lasted for 2 months . he had no cardiovascular risk factor , and no cardiovascular symptom such as chest pain or dyspnea . preoperative electrocardiography was within normal limit , and chest x - ray showed no pathologic abnormality . he was consulted to cardiovascular department because surgeon heard a continuous cardiac murmur and ordered echocardiography for a preoperative evaluation . the mitral inflow e / a ratio was 1.63 , and e / e ' ratio was 12.0 . however , there was abnormal color flow within left ventricle predominantly during diastole ( fig . 1 , supplementary movie 1,2 , 3 , 4 ) . the maximal velocity of blood flow draining into left ventricle was approximately 3.0 m / s . in short - axis great arterial view , left coronary artery appeared dilated . multiple tortuous dilated vascular structures with internal blood flows disclosed by color doppler image were also found around left ventricular myocardium . especially , dilated large echo - free vascular structure was detected in the apical area . the connection between those dilated vascular structures and left coronary artery was suspected , but clear visualization of the connection was limited in 2-dimensional echocardiography . coronary artery computed tomography ( ct ) scan was performed to confirm the pathologic anatomy of these abnormal findings . coronary ct revealed markedly dilated ( up to 16 mm ) and serpentine whole left coronary arteries ( fig . 2 ) . the left anterior descending artery was communicating with left circumflex artery at the apical posterior epicardium , and was directly connected to the basal posterior side of left ventricular cavity ( fig . since the patient had no cardiovascular symptom , he underwent laparoscopic cholecystectomy without specific treatment of caf . caf communicating with the cardiac chambers are called coronary cameral fistula , as in our case . however , the incidence of coronary cameral fistula is very low and coronary cameral fistula involving left ventricle has been very rarely reported.4)5 ) this case is also unique because almost whole left coronary artery was dilated and looked like cystic lesion in the apical portion , which could be clearly detected by transthoracic doppler echocardiography . the majority of coronary cameral fistula ( up to 90% ) drains into the right - sided chambers of the heart , while only 10% of coronary cameral fistula shows communications with the left - sided chambers or both left and right - sided chambers.6 ) doppler echocardiography is a good screening imaging tool for the diagnosis of caf , but evaluation of whole anatomy of caf can be best visualized by coronary ct image . chest pain , exertional dyspnea and fatigue may develop , but many patients do not suffer from any symptoms.7 ) the mechanism of symptoms appeared to be coronary steal phenomenon and diastolic overload.8 ) the most common complication is myocardial ischemia due to coronary steal phenomenon which occurs in 15% of patients with caf.9 ) congestive heart failure and arrhythmia could be caused by excessive loading of cardiac chambers . intravascular thrombosis , infective endocarditis , and rarely hemopericardium due to rupture of aneurysmal coronary artery might be complications . surgical closure of caf may have benefit for the patients with large , hemodynamically significant caf.10 ) there were several reasons for not doing any surgical interventions for this patient . first , this pathology was incidentally found without any cardiovascular symptom , and may carry only mild risk in undergoing laparoscopic surgery . therefore , the first thing we had to do for this patient was to let the surgeon not to postpone this necessary surgery because of the cardiac problem . second , good functional status of this patient indicates low possibility of coronary steal causing myocardial ischemia due to this pathology . lastly , surgical treatment for this coronary cameral fistula was not technically easy , because almost entire portion of the coronary artery was dilated as noticed in coronary ct . the parasternal long axis view revealed abnormal color flow within left ventricle predominantly during diastole . the parasternal short axis view revealed diastoledominant abnormal color flow draining from posterior left ventricular wall . dilated large vascular structure was detected in the apical area . abnormal vascular structure was originated from basal posterior wall and drained into the left ventricular cavity .

we present a case of 48-year - old male who presented with coronary artery fistula draining into left ventricle . transthoracic echocardiography showed abnormal blood flow draining into left ventricle , with enlarged coronary arteries and multiple vascular structures around ventricular myocardium . coronary computed tomography revealed dilatation of entire left coronary artery which was wrapping around left ventricle , and draining into the posterior side of left ventricle . he did not undergo any invasive treatment , because he was not symptomatic .

simultaneous cardiac and pericardial rupture secondary to blunt chest trauma is a highly lethal combination with a rarely reported survival . we present a young patient with a right atrioventricular groove injury , pericardial rupture and a unique and previously undescribed coronary sinus avulsion . a 17-year - old male presented to our hospital after a high - speed motor vehicle crash that required prolonged extrication followed by rapid - sequence intubation . he required one unit of packed red blood cells for hypotension . on arrival to the trauma bay , he was normotensive with a heart rate of 70 beats / min . a focused abdominal sonogram for trauma ( fast ) was negative for fluid in the abdominal and cardiac windows . full - body computed tomography ( ct ) scan revealed a retained left hemothorax and subtle irregularity of the proximal descending aorta concerning for aortic disruption [ figure 1 ] . following imaging , cardiothoracic surgery was called to the operating room due to concern for aortic disruption . upon exploring the left chest through a thoracotomy the pericardium was traumatically ruptured , but the avulsed and skeletonized left phrenic nerve was intact [ figure 2 ] . chest computed tomography scan image showing aortic irregularity ( arrow ) and retained left hemothorax traumatic rupture of the pericardium with exposed left phrenic nerve ( arrow ) control of bleeding was attempted by placing a large sheet of surgifoam along the atrioventricular groove at the base of the heart . shortly thereafter , the heart distended and the patient arrested requiring emergent cardiopulmonary bypass achieved by cannulating the left apex and right atrium . further , inspection revealed avulsion tears of the coronary sinus extending to the inferior vena cava . there was a separate avulsion of the right atrial appendage near the right coronary artery . post - operatively the patient recovered without further cardiac events and has been discharged from rehabilitation . autopsy studies show that cardiac injury in up to 32% of blunt trauma deaths with 64% of those injuries being cardiac rupture . reported only twenty - two pericardial ruptures out of over 20,000 blunt trauma admissions with a mortality rate of 63.6% . in the setting of cardiac rupture , cardiac rupture carries a high mortality rate of 50 - 86% and when combined with pericardial rupture , the mortality is nearly 100% . the clinical signs of these combined injuries are often subtle , but rapid diagnosis is paramount as exsanguinating hemorrhage can quickly ensue . isolated cardiac rupture often presents with cardiac tamponade , but with simultaneous pericardial rupture , the pericardium is decompressed preventing these classic signs . ultrasonography and ct scan can be misleading since pericardial fluid may be absent . in our case , fast revealed no pericardial fluid and chest ct led to a misguided concern for aortic injury . may et al . proposed an algorithm to aid in an early diagnosis of combined pericardial and cardiac rupture . ct scan and/or pericardial window with lavage are indicated for patients with no pericardial fluid on fast with otherwise unexplained hypotension or enlarging mediastinal hematoma . if no blood is initially identified in the pericardial window , pericardial lavage is performed by instilling 100 - 200 ml of saline into the pericardial sac . , median sternotomy is preferred allowing for excellent mediastinal exposure and potential placement on cardiopulmonary bypass or extension to laparotomy . we selected a posterolateral thoracotomy due to concern for an aortic injury since the remainder of the ct was unremarkable . historically , a cardiopulmonary bypass is rarely required and is reported in only 10% of cardiac repairs for blunt trauma ; however , in our case the patient arrested in the operating room requiring emergent cardiopulmonary bypass . for cardiac repair , we report a rare survival of combined pericardial and cardiac rupture and a unique coronary sinus avulsion following blunt chest trauma . in addition , we propose an algorithm for its diagnosis and management [ figure 3 ] . traditional diagnostics such as fast and ct are often unhelpful due to the unique combination of injuries repair of this spectrum of injuries requires early involvement of cardiac surgery expertise and possibly placement of patient on cardiopulmonary bypass .

simultaneous cardiac and pericardial rupture from blunt chest trauma is a highly lethal combination with rarely reported survival . we report of a case of young patient with a right atrioventricular groove injury , pericardial rupture and a unique description of a coronary sinus avulsion following blunt chest trauma . rapid recognition of this injury is crucial to patient survival , but traditional diagnostic adjuncts such as ultrasound , echocardiography and computed tomography are often unhelpful . successful repair of these injuries requires high suspicion of injury , early cardiac surgery involvement of and possible even placement of the patient on cardiopulmonary bypass .

benign schwannoma is a slow growing encapsulated tumor arising from the neuroectodermal sheath of schwann . approximately , 25 - 30% of all reported schwannomas occur in the head and neck and most of these in the eighth nerve . schwannoma of the facial nerve is a rare entity with very few cases reported in the literature . in our case a 7-year - old female was brought to ent out - patient department with right - sided parotid swelling of 1-year duration . initially , the swelling was small in size that gradually and painlessly increased up to its present size [ figure 1 ] . the swelling was 10 cm 9 cm 7 cm in size , firm in consistency , had bosselated appearance , was mobile horizontally and vertically and the skin over the swelling was mobile . cytology was advised , and it came out to be a benign parotid tumor . since the mass was large in size and patient was a child , a computed tomography ( ct ) scan ct scan suggested a well - defined , lobulated , homogenous , mildly enhancing soft tissue lesion with loss of interface with the masseter muscle and multiple small lymph nodes [ figure 2 ] . a provisional diagnosis of pleomorphic adenoma was made , and superficial parotidectomy planned [ figure 3 ] . peroperatively , the facial nerve trunk was found deeper and inferior due to pushing effect of the tumor . however , on further anterior dissection the tumor was found to originate from the buccal and zygomatic branches of the facial nerve . the patient had postoperative ( after 3 months of surgery ) facial weakness of grade three ( house brackmann classification ) [ figure 4 ] . female patient with right parotid swelling ( preoperative ) computed tomography scan showing the parotid mass line of incision of the planned surgery postoperative photograph showing facial weakness the gross appearance of the tumor was like a brain tissue [ figure 5 ] and histopathologically , it was confirmed to be an intraparotid shwannoma , showing antoni type a and b cells [ figure 6 ] . the lymph node showed reactive changes . resected tumor resembling brain tissue histopathological slide showing salivary and nervous tissue among 802 parotid tumors reported eneroth and hamberger could demonstrate two cases with neurogenic origin and in a review of 700 parotidectomies nussbaum et al . found only one case of neurilemma of the facial nerve . the intraparotid facial nerve shwanommas are mostly ( 75% ) asymptomatic . the tumor grows eccentrically pushing the nerve fibers away as observed in the present case . the ability of the parotid to accommodate the expanding tumor results in facial nerve palsy in only 20 - 27% of cases . in some cases , radiology may provide preoperative information about facial nerve schwannoma but in many cases it mimics pleomorphic adenoma . the ct scan although not the preferred method of imaging shows a smooth and sharply defined mass in the parotid . fine - needle aspiration cytology is also unreliable for diagnosing these lesions as in most cases results are inconclusive or suggest a pleomorphic adenoma . the diagnosis is , therefore , generally made peroperatively when the surgeon finds difficulty in identifying the facial nerve . in our case , the size of the tumor was quite big and could have complicated into facial nerve palsy very soon . moreover , the patient 's preference to get the tumor operated was the main indication for a surgical intervention . any parotid mass in children should be viewed with suspicion and adequately investigated before any intervention is planned . intraparotid schwannoma should be suspected if the facial nerve can not be found intraoperatively , if the tumor is intimately associated with the facial nerve and if the gross appearance of the tumor is like that of a brain tissue . in cases where schwannoma is suspected , biopsy is recommended while complete resection is postponed to obtain imaging studies to evaluate the extent of disease and to discuss possible outcomes with the patient .

pleomorphic adenomas are the most common tumors which present as parotid masses . shwannoma ( peripheral nerve sheath tumor ) is a rare entity in this region . very few schwannomas originate from the facial nerve and in the majority of these cases the tumor involves its intratemporal part . the following case is reported because it presented as an asymptomatic parotid swelling with normal seventh nerve function , which masqueraded as pleomorphic adenoma clinically , radiologically and cytologically . however , it turned out to be peripheral nerve sheath tumor on histopathological examination .

ultrasound is routinely used to evaluate small breast masses . a large proportion of these small masses are benign but unfortunately many go to biopsy or excision despite benign imaging characteristics . utilizing our case of adult male pilomatrixoma , we discuss the work - up of small breast masses , focusing on ultrasound characteristics , and stress conservative management if the typical benign sonographic features are present . despite a very low propensity for malignant degeneration , surgical excision is the treatment of choice for pilomatrixomas , mainly for symptomatic and cosmetic reasons in a pediatric population . given the age , location , and low chance of malignant degeneration , conservative management may be considered in rare case of pilomatrixoma of the adult breast . a 53-year - old male presented with a painless hard palpable pea - sized lump , in the right breast , near the nipple . a referral for mammography was made as the man had a strong family history of breast cancer with two sisters diagnosed at ages 42 and 50 . diagnostic mammography showed a well - circumscribed , oval - shaped mass of the medial right breast , with smooth borders , and multiple small punctuate calcifications [ figure 1 ] . gray - scale ultrasound examination revealed a small superficial isoechoic well - marginated oval mass parallel to the skin , with smooth borders , and multiple hyperechoic foci corresponding to calcifications seen on mammogram [ figure 2 ] . the lesion appeared benign on ultrasound examination , but given the patient 's concern and a strong family history of breast cancer , a core biopsy was performed . the patient remained anxious about malignancy despite this benign diagnosis , so the mass was excised two months later with an unchanged pathologic diagnosis . mediolateral oblique view of mammogram demonstrates a small well - circumscribed oval mass just underneath a radiopaque skin marker in the medial right breast near the nipple . a gray - scale ultrasound image of the lesion shows a well - defined isoechoic oval mass , parallel with the skin measuring 7 mm 3.5 mm , containing the small hyperechoic foci of calcification observed on the mammogram . pilomatrixoma , originally named calcifying epithelioma of malherbe , was initially described in 1880 by dr . chenantals malherbe as a calcified epithelioma of sebaceous glands . developing in the subcutaneous tissue and arising from the matrix cells of the hair bulb , these benign tumors usually develop during the first two decades of life and have been very rarely described to degenerate to pilomatrixcarcinoma . the tumors are commonly found on the head with the cheek being a frequent location , followed by the neck and upper extremities in decreasing frequency . pilomatrixomas are more seldom found on the trunk and lower extremities and are very rarely found in the breast . despite a very low chance for malignant degeneration surgical excision is the treatment of choice as these tumors do not regress and occur in cosmetically unpleasant locations , usually the head and neck in a pediatric population . , there have been only five reports of pilomatrixoma of the adult male breast in the english literature . the tumors were removed in two cases without undergoing medical imaging , most likely due to the size of the tumor . two other reports described typical benign features of the tumor ; one with mammography and sonography and the other with mammography alone . sonography is routinely used to evaluate palpable masses as well as incidental masses found on screening mamography . traditionally , ultrasound was used for differentiation of cystic from solid masses but its role has evolved to serve as a guide in characterization of masses into benign , indeterminate , or malignant categories . the negative predictive value for small breast masses with benign ultrasound features has been quoted to be greater than 99% in several studies.[710 ] features that have been described to most reliably characterize masses as benign are a round or oval shape , circumscribed margins , and width - to - anteroposterior dimension ratio greater than 1.4 . , if agreed upon by three different readers , these three features predicted benignity with 100% accuracy . in fact , pilomatrixomas on imaging are commonly well - defined subcutaneous nodules . also , most are heterogeneously hyper- to isoechoic with acoustic shadowing and hyperechoic calcifications , as seen in our case . in general , a differential diagnosis of a superficial breast lesion includes seborrheic keratosis , dermal nevus , epidermal inclusion cyst , and basal cell carcinoma . although the majority of superficial tumors are benign , management is frequently based on results of the triple test ( physical exam , imaging , and biopsy ) and biopsy is performed despite benign imaging characteristics . we suggest a conservative approach of interval follow - up for any small superficial breast mass with benign imaging characteristics . such masses can be assigned into a bi - rads 3 category without a pathologic diagnosis [ figure 4 ] or following a benign biopsy result to ensure stability over time . also , conservative management can be considered in cases of pilomatrixoma of the adult breast . although , most advise surgical excision of the tumor , especially in a pediatric population , an asymptomatic pilomatrixoma cosmetically acceptable to the patient seems safe to monitor with imaging as there is very low chance of malignant degeneration . given the location and lack of symptoms , conservative management was discussed with our patient but excisional biopsy was ultimately performed due to anxiety resulting from a strong family history of breast cancer . a well - circumscribed breast lesion on gray - scale ultrasound parallel to the skin surface has not been biopsied but is likely to be benign given its stability on sonography for nearly two years .

pilomatrixomas are uncommon benign skin neoplasms arising from the hair follicle matrix . they occur more commonly in children than adults . most originate on the head , neck , or upper extremities , less commonly on the trunk or lower extremities , and very infrequently in the breast . we present a rare case of pilomatrixoma of the breast in an adult male . as the patient had a strong family history of breast cancer , a full work - up of the breast mass was performed . ultimately , an excisional biopsy was carried out for patient reassurance .

spontaneous pseudoaneurysm of the femoral artery is extremely a rare entity.(1 ) it can be secondary to the rupture of a true aneurysm , but in most events the cause is unclear.(2 ) most patients with non - atherosclerotic pseudoaneurysms of sfa are asymptomatic initially and later present with a pulsatile , expanding mass along the anatomical course of sfa in the thigh and have a history of some trauma or surgical procedure.(3 ) due to their complications , large pseudoaneurysms require some kind of intervention . if not treated , they are at risk for rupture , infection , distal embolization or arteriovenous fistula formation . ( 1 ) we report a young patient with spontaneous femoral artery pseudoaneurysm without any history of trauma or vascular intervention successfully treated surgically . a 29-year - old healthy man presented with right thigh swelling since 2 months which is painful throbbing in nature and aggravated by walking . there was no history of local trauma and he had never suffered peripheral vascular disease . on presentation , he was hemodynamically stable and there was a tender swelling at the medial aspect of right mid thigh which was pulsatile . ct angiogram was done which showed a pseudoaneurysm of the lower third of femoral artery . ct angiogram showed a pseudoaneurysm of the lower third of femoral artery a surgical excision of the pseudo - aneurysm was done under general anesthesia . after evacuation of hematoma , there was a large defect in the wall of the artery . therefore , a greater saphenous vein interposition graft was used to maintain the vascular continuity . formation of a femoral artery pseudoaneurysm is not infrequent after femoral arterial access , and it occurs in up to 0.7% of the diagnostic procedures and in up to 8% of the interventional procedures . ( 4 ) recently , there has been a significant decrease in both major and minor vascular complications after diagnostic and therapeutic procedures with an incidence of pseudoaneurysm less than 0.3 % . ( 5 ) spontaneous pseudoaneurysm of the superficial femoral artery in the young age group is a rare occurrence . some of the false aneurysm may close spontaneously but rupture is a major concern followed by thrombosis , distal embolization and compression of adjacent structures . ( 3 ) atherosclerosis has been proposed by several authors as an etiologic factor in spontaneous perforation of the femoral arteries or its branches . ( 6 - 8 ) in these case reports , the patients were elderly with atherosclerotic changes in their peripheral arteries and weakening of the arterial walls could have led to spontaneous rupture . patients who have inherited connective tissue disorders such as ehlers - danlos have also been reported to present with spontaneous rupture of their arteries because of the fragility of their arterial walls . ( 9 ) . our patient , however , was young and there was no evidence of atherosclerotic changes in his arteries on the angiogram or during surgery . furthermore , he did not have any clinical features to suggest an inherited connective tissue disorder . there was also no history for any trauma and for which we labeled him to have a spontaneous femoral artery pseudoaneurysm . therapeutic options for femoral pseudoaneurysm include open surgical repair , ultrasound - guided compression , ultrasound - guided thrombin injection , coil embolization , and endovascular repair using stent - grafts . ( 10 ) particularly in young patients , an appropriate approach is surgical exploration with hematoma evacuation and arterial repair by means of arterial suture , patch angioplasty , or graft interposition . ( 2 ) femoral artery pseudoaneurysm has been a common complication after the wide use of femoral artery access for diagnostic and therapeutic procedures . however , spontaneous rupture of the artery without trauma remains a rare entity . as there are many evolving types of management , the surgical intervention may has superiority when there is a suspicious of underlying vascular pathology .

spontaneous femoral artery pseudoaneurysm is a rare disease and reported cases are very few . most of them are related to underlying pathology either atherosclerotic disease or connective tissue disease . we present a 29-year - old healthy man with two months history of a painful pulsating mass at the level of the lower right thigh without any previous history of trauma , surgery or puncture of the femoral artery . an angiogram revealed a right superficial femoral artery pseudo - aneurysm . it was treated surgically by resection of the aneurysm , reconstruction with inter - positional saphenous vein graft . we reported this case because of its rare incidence in the young patient with no underlying pathology .

primary cutaneous apocrine gland carcinoma , a subtype of sweat gland carcinoma , is an extremely rare malignant neoplasm [ 1 , 2 , 3 ] . most of these neoplasms involve the axilla , but lesions can also occur elsewhere on the skin . most often , they are indolent and slowly developing , but some are rapidly progressive and extremely aggressive [ 1 , 4 , 5 , 6 ] . the treatment of choice is wide local excision with clear margins , with or without lymph node dissection [ 1 , 3 , 5 , 7 ] . a 67-year - old man presented to our hospital with an ulcerated nodule in the right axilla measuring 1 0.8 cm . the nodule had been present for more than 3 years , but only grew in size over the past 6 months . bilateral breast examination , skin examination and complete lymph node survey were unremarkable . ultrasound examination and a mammogram revealed no significant lesions in either breast , consistent with the physical examination . microscopic examination revealed a well - to - moderately differentiated adenocarcinoma which contained ductal and glandular structures with obvious apocrine features . in addition , the cytoplasm of the tumour cells contained pas - positive diastase - resistant granules without iron - staining granules . the tumour tissue invaded the papillary and reticular dermis and ulcerated the epidermis without extension to the subcutaneous tissue ( fig . 1 , fig . the immunohistochemical study showed that the tumour cells were positive for ker7 , ema and e - cadherin and negative for cea , sloop and psa . no oestrogen or progesterone receptors were detected , making the diagnosis of male breast cancer metastaticto the axilla unlikely ( fig . cutaneous apocrine gland carcinoma , a subtype of sweat gland carcinoma , is a very rare malignant neoplasm arising in areas of high apocrine sweat gland density . to date , only few cases ( about 50 cases ] have been reported in the literature . these lesions occur primarily in the axilla , but can also occur elsewhere on the skin [ 1 , 2 , 3 9 ] . apocrine adenocarcinoma usually develops de novo but has also been observed to arise in association with other benign tumours such as apocrine adenoma and apocrine hyperplasia [ 10 , 11 ] . most of the neoplasms are relatively indolent and slowly developing over months to years , but some are rapidly progressive and extremely aggressive . they mostly present as nodules or masses 23 cm in size , without any additional symptoms [ 1 , 3 , 5 , 6 ] . one third of the patients have regional lymph node involvement at diagnosis . also , some patients have died from metastases to the lungs , liver , bone , brain and kidney [ 1 , 3 , 5 ] . the histologic picture likes as an adenocarcinoma that may be well , moderate or poorly differentiated [ 1 , 3 , 9 ] . the cytoplasm of the tumour cells contains pas - positive , diastase - resistant granules and often iron - positive granules [ 1 , 7 , 9 , 12 ] . the differential diagnosis of apocrine gland carcinoma from metastatic mammary adenocarcinoma is not possible on morphological grounds , and immunohistochemistry , except for a few cases , does not allow distinction between them . malignant sweat gland tumours are often positive for oestrogen and progesterone receptors , and these markers are therefore of limited usage in differential diagnosis [ 1 , 3 , 9 ] . features that favour the diagnosis of a primary apocrine gland carcinoma are the presence of neoplastic glands high in the dermis , apocrine glands near the tumour and intracytoplasmic granules of iron . the treatment of choice is wide local excision with clear margins , with or without regional lymph node dissection . postoperative radiotherapy and chemotherapy in patients with moderately or poorly differentiated tumours have been used as adjunctive treatments but have shown little benefit on mortality [ 1 , 3 , 5 , 13 , 14 ] . there is a high incidence of local recurrence ( 28% in one report ] , and prophylactic nodal dissection does not reduce the incidence of local recurrence . primary apocrine carcinoma of sweat glands is a very rare tumour , and there are no guidelines for the treatment of recurrent or metastatic disease .

cutaneous apocrine gland carcinoma , a subtype of sweat gland carcinoma , is a very rare malignancy , and only few cases have been reported in the literature . many of these carcinomas are indolent and slowly developing , but some are rapidly progressive . the treatment of choice is wide local excision with clear margins , with or without lymph node dissection . we report a case of a 67-year - old man who came to our hospital with an ulcerated nodule in the right axilla measuring 1 0.8 cm . histological evaluation showed features of an apocrine gland carcinoma arising in an area of high apocrine gland density .

healthcare - seeking behaviors declined markedly during the ebola epidemic ( 2 ) , which likely contributed to probable underreporting of measles cases , a fact further substantiated by the lack of fatalities reported to dps . although there are wide variances in estimates of measles case - fatality rates , a large study has suggested an average case - fatality rate of 3.7% for africa ( 13 ) , which would correspond with 26 deaths from the 702 suspected cases reported in lola during january june 2015 . the lack of reported fatalities may also be related to the effect of the ebola epidemic on burial practices ; any families notifying authorities about deaths would be required to conduct safe and dignified burials , a protocol that had been met with resistance by many local groups ( 2 ) . when the full shipment of vaccines arrived in lola in mid - april , logistical planning was challenged by shortages of personnel , fuel for automobiles , and appropriate vehicles for traversing difficult terrain during the onset of the rainy season . the campaign is estimated to have reached 92% of the target population , but persons in some urban areas and villages were reluctant to receive vaccinations . the launch of the campaign coincided with reduced measles transmission ( figure ) , but further modeling research would be required to assess its effect on the course of the outbreak . in the aftermath of the ebola epidemic , . the front lines of disease surveillance and outbreak detection often occur in rural settings that are understaffed and underresourced . as guinea transitions to a post - ebola phase this reduction would be unfortunate , because the technical and cultural expertise of the doctors from guinea in lola and other similar settings transcends ebola and could be harnessed to support a wide range of public health activities . strengthened investments in local public health systems will be essential to ensure the population of guinea can recover from the ebola epidemic and be better protected from future disease outbreaks . aside from personnel , the public health infrastructure , including surveillance , information and communications technology , and temperature - controlled supply chains , particularly requires attention . meanwhile , great efforts will also be needed to restore and enhance community trust in medicine and public health ( 2 ) .

during public health crises such as the recent outbreaks of ebola virus disease in west africa , breakdowns in public health systems can lead to epidemics of vaccine - preventable diseases . we report here on an outbreak of measles in the prefecture of lola , guinea , which started in january 2015 .

the introduction of osseointegration and the use of endosseous implants provide alternative treatment options to clinicians for all indications of edentulism.1 implant - supported , fixed restorations are usually classified as screw- or cement - retained.2,3 the advantage of screw - retained restorations is the combination of a rigid connection between the restoration - abutment complex and its retrievability . however , these restorations are usually more expensive than cement - retained restorations because of the use of extra components and laboratory costs.4 cement - retained restorations were introduced to compensate for problems of screw loosening and the lack of esthetics of screw - retained restorations.4 the lack of fastening screws in cement - retained restorations reduces the possibility of preload stress and screw loosening.5 the major advantages of cement - retained restorations are the passive fit of frameworks , enhanced esthetics resulting from lack of screw access holes , and reduced complexity of laboratory procedures and chair - side time.6,7,8 the disadvantages of cement - retained restorations include the requirement for extra time for cementation , removal of residual excess cement , limited design possibilities for superstructure , and the reduced possibility for modifying treatment in case of periimplantitis.9 the existence of residual excess cement in peri - implant sulcus is a common complication of cement - retained implant prostheses.10,11 if there is excess cement located in the soft tissue deeper than 3 mm , it might be difficult to observe and remove . insufficient removal of excess cement may result in swelling , soreness , exudation or bleeding on probing , and can initiate a local inflammatory process , which is evidence of peri - implant disease and can ultimately lead to implant failure.12,13,14 moreover , removal of excess cement may cause scratching and gouging on the implant surfaces when plastic and metal scalers are used.15 several authors have reported on techniques regarding procedures used to assist in minimizing residual excess cement extrusion.16,17,18 this article describes a method of controlling cement flow , using stock or custom implant abutments , when cement - retained implant - supported restorations are utilized . the method can be used easily and quickly at chair - side by the use of daily restorative and laboratory materials . the use of die spacers results in a uniform space between the crown restoration and the implant abutment . check the marginal fit of the crown restoration to the implant analog on the dental model . 1a ) according to the manufacturer 's recommendations ( siladent die spacer 12 m gold , dr . bhme & schps gmbh , germany ) . repeat applying the die spacer until the desired cement film thickness is achieved ( application of the die spacer 3 - 4 times forms a film thickness of approximately 45 - 50 m ) . completely fill the crown restoration with a bis - acrylic temporary restorative material ( luxatemp plus , dmg chemisch - pharmazeutische fabrik gmbh , germany ) and put a retention pin ( bredent gmbh & co. kg , germany ) with a smaller diameter tip into the uncured material to form a handle ( fig . 1d ) and secure the retention pin until the bis - acrylic material is cured . remove the crown restoration and check any discrepancies between the implant abutment and the bis - acrylic abutment . check that there are no voids on the duplicate abutment , and that the finish line has been duplicated accurately ( fig . 1f ) . clean the intaglio surface of the crown restoration with air and check any residual die spacer . mix a desired luting agent ( temp bond ne , kerrhawe s.a . , switzerland ) and apply to the intaglio surface of the crown restoration ( fig . 2b ) place the crown gently onto the bis - acrylic abutment and wipe off the excess cement with a cotton swab ( fig . if there is a lack of cement layer , line the intaglio surface of the crown restoration with a thin layer of extra luting agent . this article presents a method of minimizing the excess cement around implant - retained restorations . the advantage of the technique is allowing the control of cement flow by using a custom - made duplicate abutment that can be fabricated quickly , easily , and economically at the time of implant abutment / crown insertion.16 the major benefit of extraoral cementation is to allow the indirect removal of excess cement around the margins . this clinical procedure is extremely important for avoiding the potential of peri - implant disease caused by residual cement left in the gingival sulcus . it is important not to form an oversized cement space when duplicating the implant abutment . the use of a die spacer provides a space of approximately 50 m , which represents the ideal cement space , and may be used for both custom and prefabricated abutments . the disadvantage of the technique is that it is a time - consuming procedure for routine clinical processes . dumbrigue et al.16 stated that when the extraoral cementation technique is preferred , the luting agent must have a longer working time . when a custom abutment is to be used , the dental laboratory may be instructed to make an abutment analog using an acrylic resin , but this is time consuming for the technician and involves additional laboratory costs.18

the major drawback of cement - retained restorations is the extrusion of the excess cement into the peri - implant sulcus , with subsequent complications . insufficient removal of the excess cement may initiate a local inflammatory process , which may lead to implant failure . this article presents a method of controlling cement flow on implant abutments , minimizing the excess cement around implant - retained restorations .

in the field of dentistry , gingival hypertrophy ( gh ) is a common presenting complaint , especially among patients belonging to pediatric age group . gingivitis is a commonly occurring phenomenon since the oral cavity is a potential site for active infection as it is constantly exposed to pathogenic microorganisms . the prevalence of gh among school going children is nearly 14% , inflammatory origin being the most common cause followed by chronic drug intake . hence , there is a tendency among us to consider gh as a mere inflammatory response to local infection rather than considering systemic illnesses ; this can eventually lead to delay in diagnosis and initiating appropriate treatment . gh due to acute myeloid leukemia ( aml ) in a child is extremely rare . here , we report a case of aml ( subtype m5 of fab classification ) in a 3-year - old male child wherein the disease primarily presented as gh . a 3-year - old male child presented to us with fever and body pain for 2 weeks , gum swelling for 1-month duration , initially presented to a pediatrician and a dentist , for which symptomatic treatment was given . the child was again reviewed with the pediatrician after 1 week with persisting symptoms . no history of halitosis or dental caries . on general examination , he had pallor ; there was no icterus , cyanosis , clubbing , pedal edema , or generalized lymphadenopathy . oral examination revealed the enlargement of maxillary and mandibular gingiva covering two - third of the crown structure in buccal , lingual , and palatal aspects . gingiva was reddish with the loss of stippling , soft , spongy , bleeding on touch but was nontender and not warm [ figure 1 ] . on abdominal examination , the liver was palpable 2.5 cm below the right costal margin , and the spleen was palpable 2 cm beyond the left costal margin . g / l , total white blood cell count was 5.6 10/l with 90% blast cells and 10% lymphocytes , and platelet count was 0.9 10/l . his renal and liver function tests were within the normal range ; uric acid and lactate dehydrogenase levels were also normal . bone marrow aspirate smear showed blasts which are large with moderate amount of agranular to granular cytoplasm and round to irregular nucleus with fine chromatin and 13 nucleoli . few of the blasts showed auer rods [ figure 2 ] . in flow cytometric analysis , the blasts were positive for cd33 , cd117 , human leukocyte antigen - dr , and cd13 . parents were counseled about his condition , treatment , and outcome of the disease . due to long distance commuting issues , the parents opted for the child to receive treatment elsewhere ; the child was lost to follow - up . most common causes of gh in children are poor dental hygiene and chronic drug intakes such as phenytoin , cyclosporine , and calcium channel blockers . systemic illnesses such as wegener 's granulomatosis , sarcoidosis , crohn 's disease , infantile systemic hyalinosis , tuberculosis , and hereditary gingival hyperplasia should also be considered in the differential diagnosis . acute leukemia , which is the most common malignant disorder in children , is characterized by neoplastic proliferation of blast cells causing accumulation of > 20% blast cells in the bone marrow , interfering hematopoiesis resulting in fatigability , bleeding tendency , and infections . acute leukemia is subdivided into acute lymphoblastic leukemia and acute myeloid leukemia ( aml ) , based on the phenotype of the blast . aml in a child generally presents with fatigue , pallor , abnormal bleeding and infections with or without splenomegaly and lymphadenopathy ; however rarely , it can present with infiltration of blasts cells in tissues such as skin , mucosa , and gums . literature review has shown only few studies that have reported cases of aml presenting with oral lesion as a primary manifestation . oral manifestations in leukemia are gh , oral ulcers , pale mucosa , herpes , and candida infections . gingival infiltration in aml is rare , usually associated with subtypes m4 and m5 ( fab classification ) . it is due to infiltration of tissues with neoplastic precursors of myeloid cells or secondary to thrombocytopenia , neutropenia , or impaired granulocyte function . hence , gingival tissue , although unusual , is one of the sites that should be monitored for relapse . although confirmatory diagnosis and treatment of aml is primarily done by the pediatric hemato - oncologist , it is almost always a dentist or a pediatrician who first attend to cases of gh , and hence increasing awareness among them will aid in early detection and prompt referral and drastically improve the outcome of the disease . finally , any gh , particularly when associated with hepatosplenomegaly and abnormal blood counts should raise the suspicion of possible underlying hematological malignancy in the child .

acute leukemia is the most common malignant disorder of childhood . acute leukemia is characterized by marrow failure due to the replacement of marrow elements by leukemic blasts . gingival hypertrophy ( gh ) due to acute myeloid leukemia ( aml ) in a child is extremely rare . here , we report a case of aml ( subtype m5 of fab classification ) in a 3-year - old male child wherein the disease primarily presented as gh .

a 56-year - old caucasian male with polycythemia vera controlled with interferon alpha treatment for 2 years presented to our institution with decreased vision , greater on the right than left , scintillating scotomata , and floaters . intraocular pressures were 14 mmhg in the right eye ( od ) and 12 mmhg in the left eye ( os ) . fundus examination on the right showed nerve fiber layer swelling at 12 and 6 oclock , a very distended and tortuous superior branch retinal vein , one flame - shaped peripapillary hemorrhage , and one preretinal hemorrhage alongside areas of ischemia at the inferior pole ( figure 1 ) . the left eye showed one peripapillary hemorrhage with a white center ( roth spot ) with slight retinal nerve fiber layer swelling at the optic disc margin superiorly and inferiorly , but much less venous distention ( figure 2 ) . fluorescein angiography confirmed impending retinal vein occlusion with delayed transit time ( 20 seconds for the right superior hemi vein ) , more in od than os , but no choroidal infarction . interferon was stopped immediately , and the patient started pentoxifylline 400 mg every day to treat polycythemia vera , and prednisone 40 mg every day to counter any interferon - induced inflammation . within 3 weeks , his vision became clear , the afferent papillary defect resolved , and fundus examination revealed complete resolution of the aforementioned vascular abnormalities . he was slowly tapered down to prednisone 7.5 mg every other day and maintained on pentoxifylline 400 mg three times a day . here we describe the course and likely pathophysiology of interferon alpha and polycythemia vera - associated impending aion , retinal vessel ischemia , and hemorrhage . our patient s bilateral optic disc edema did not have the supporting signs or symptoms of elevated intracranial pressure or malignant hypertension . in fact , our patient s optic disc edema was concentrated at the superior and inferior poles , corresponding to the locations of venous compromise . thus , considering the associated retinal venous distention and hemorrhage , we attributed the patient s optic disc edema to impending aion and his delayed filling on fluorescein angiogram to retinal vein occlusion . we attribute these fundus examination findings to axoplasmic stasis and retinal hemorrhage resulting from optic and retinal vascular occlusion and venous backflow,1 all likely secondary to complications of interferon and polycythemia vera . interferon has been documented to cause occlusion directly , possibly from inflammation.2 interferon may produce autoantibodies and incite inflammation in the vessels of the optic disc,2 contributing to an impending aion . this can cause optic nerve ischemia and fiber swelling,1 which can progress further to retinal vein occlusion and hemorrhage , as well as to aion with permanent losses of visual field and visual acuity.3 the preretinal hemorrhages and roth spots seen in our patient have been documented as complications with both interferon4 as well as myeloproliferative disorders.5 polycythemia vera poses additional risk factors for retinal vein occlusion , including higher whole blood viscosity , reduced red cell deformability,6 and abnormal red cell adhesion . diminished membrane fluidity of red blood cells plays a role in microvascular occlusion in patients with polycythemia vera.7 in addition , jak2 kinase mutation in patients with polycythemia vera causes abnormal phosphorylation of cd 239 on red blood cells , which allows its ligation with the laminin alpha5 chain on the apical surface of endothelial cells.8 this adhesion has been demonstrated to be strong enough to withstand sheer stresses of postcapillary venules.8 thus , polycythemia vera - associated red blood cell adhesion may be another strong factor in the pathophysiology of venous occlusion.6 considering that both polycythemia vera and interferon are possible influences on vascular occlusion and optic disc edema and the pathophysiology described , we immediately attempted to treat the polycythemia vera empirically with pentoxifylline to increase red blood cell fluidity and the interferon - associated inflammation with prednisone . our patient also had a history of hypotension to 95/65 mmhg , which was monitored , given that hypotension is a risk factor for aion.9 our patient experienced complete resolution of fundus abnormalities and return of normal vision , which may be attributed to successful treatment of both etiologies , though there remains the possibility of spontaneous resolution . thus , further study is warranted to elucidate the treatment of both polycythemia vera and interferon - induced impending aion .

we describe the course and likely pathophysiology of impending anterior ischemic optic neuropathy ( aion ) and retinal vein occlusion in a 56-year - old man with polycythemia vera managed with interferon alpha for 2 years . our patient presented with decreased vision , scintillating scotomata , and floaters . fundus examination findings and results of a fluorescein angiogram led to the diagnosis of impending aion and retinal vein occlusion . considering that both polycythemia vera and interferon have possible influences on vascular occlusion and optic disc edema , we stopped interferon treatment and immediately attempted to treat the polycythemia vera empirically with pentoxifylline and any interferon - associated inflammation with prednisone . our patient experienced complete resolution of fundus abnormalities and return of normal vision within 3 weeks , which may be attributed to our successful treatment of both etiologies . thus , further study is warranted to elucidate the treatment of both polycythemia vera and interferon - induced impending aion .

a 60-year - old male was referred to the asan medical center for a nodule in the left lower lobe ( lll ) . the nodule was incidentally detected during the preoperative evaluation of gallstone , for which he underwent laparoscopic cholecystectomy at an outside hospital three months prior to his presentation to us . on his visit , he did not have any respiratory complaints such as productive cough or dyspnea . further , the patient had been a non - smoker for 10 years although he used to smoke half a pack a day for 30 years before quitting . we found that the patient 's physical examination was unremarkable except decreased breath sounds over the left lower lung field . chest x - ray and computed tomography scan revealed a mass having the following dimensions : 20105 mm . the mass obstructed the secondary bronchus entering into the lll , which resulted in a total collapse of lll ( fig . 1 ) . a flexible bronchoscopy showed an endobronchial mass filling the basal segments of the lll ( fig . the patient underwent a left lower lobectomy via left posterolateral thoracotomy through the 5th intercostal space . we divided the lll bronchus at the level of the left upper lobe spur and performed a left lower lobectomy . the medial side of the left main bronchus was repaired using an interrupted anastomosis of 3 - 0 vicryl . the resection margin of the bronchial stump was clear from the tumor on the frozen section . immunohistochemical staining demonstrated the positivity for s-100 protein , and the ki-67 labeling index was low ( 1% ) , supporting the current diagnosis . granular cell tumor ( gct ) , a rare benign neoplasm that most commonly occurs in the tongue , skin , subcutaneous tissue , and breast , was first described by abrikossoff in 1926 . pulmonary gct , known to comprise 6% to 10% of all gtcs , was first reported by kramer in 1938 , and since then , less than 80 cases of gct arising in the lung have been reported in the english - language literature . in korea , seo et al . it was traditionally termed ' granular cell myoblastoma ' until the late 1980s after abrikossoff suggested that gct had a myogenic origin . now , it is believed that gct has a neural cell origin , thus establishing the current nomenclature . although it has been known that most pulmonary gcts behave in a benign fashion , our review of the literature suggests that they have no unique clinical features . pulmonary gcts can be associated with synchronous extrapulmonary gcts occurring in various organs , such as the tongue , kidney , or esophagus . pulmonary gcts may occur metachronously in a single lung and thus , can be multicentric in 10% to 20% of the patients , although a majority of the gcts tend to be solitary . pulmonary gcts can be associated with other malignancies or infectious diseases such as tuberculosis or human immunodeficiency virus . pulmonary gcts can be diagnosed at any age , but most cases of pulmonary gct present in the third or the fourth decade . previous clinical series showed that there is no gender predilection , and gcts are equally distributed over both lungs with a predilection for the upper lobe . however , another study found a slight predilection toward the left lung with a preference for the lower lobe , as presented in our case . there are no commonly agreed risk factors , although the association of smoking with pulmonary gcts was hypothesized in some studies . more than half of the gct patients are asymptomatic at the time of diagnosis , and respiratory symptoms such as cough , dyspnea , hemoptysis , and wheezing present as tumor erosion and bronchial obstruction progress due to the growth of the endobronchial mass . with the onset of the symptoms , the bronchial obstruction causes the suppuration and destruction of the lung parenchyma distal to the obstruction ; in this case , surgical resection is clearly indicated . advocated bronchoscopic resection in asymptomatic patients with a tumor having a diameter of less than 8 mm , and van der maten et al . suggested that endobronchial therapy be the primary treatment however , bronchoscopic removal can not be a safe treatment option as it does not guarantee complete removal when gcts infiltrate through the entire bronchial wall , which is evidenced by the fact that the incidence of recurrence was as high as 54% after bronchoscopic removal . in addition , although extremely rare , it is possible that pulmonary gcts can be malignant ; the first case of malignant pulmonary gct was reported in 2003 . being relatively young at the time of diagnosis can be a factor that favors complete surgical resection . in this regard , we believe that adequate surgical removal should be the preferred treatment option for all patients among whom gcts are amenable to surgical resection . based on the size , location , and number of masses , a surgical option , including either segmentectomy , lobectomy with or without sleeve resection , or rarely pneumonectomy , can be chosen with a lower incidence of recurrence and long disease - free survival .

we report a rare case of granular cell tumor arising in the left lower lobe ( lll ) bronchus with secondary obstructive change in a 60-year - old male . the patient was found to have a nodule in the lll on a computed tomography scan , three months prior to his presentation to the asan medical center . bronchoscopic biopsies revealed a granular cell tumor . after undergoing lll lobectomy with bronchoplasty , the patient has not experienced any tumor recurrence .

a 73 year - old patient with mild hemophilia b ( factor ix clotting activity 12% ) was referred to our department due to a left renal tumor incidentally discovered during ultrasonography performed as follow - up two years after prostate cancer brachytherapy . on admission abdominal and pelvic ct scans revealed a multilocular tumor in the lower left kidney pole ( 57x52x52 mm ) with a solid mass of 3.5 cm in diameter ( figure 1 ) . other abdominal and chest imaging showed no abnormalities . twelve hours before surgery , he was transfused with 4200 units of factor ix concentrate ( octanine , octapharma ag , switzerland ) , with the same dose administered one hour prior to the procedure . on october 3 of 2014 , the procedure was performed with the patient positioned on his right side . following urinary catheterization , a veress needle was introduced close to the umbilicus and an intraperitoneal pressure of 14 mmhg was created . the descending colon was dissected and the fat renal capsule subsequently cut for access to the left renal artery . the artery was then clipped with polymeric clips ( hem - o - lok xl ( teleflex , usa ) and cut in between . the same was done for the renal vein . the kidney was excised , placed into a hemobag ( covidien , usa ) and removed through a small incision between the two ports . a plastic drain was placed into the kidney bed and the abdominal wall was closed with layers of sutures . peritoneal drainage was kept in place for two days with no significant output of blood or serous fluid . substitution therapy was continued for 9 days with the level of factor ix clotting activity at 90130% of the normal value on days 13 , 6574% on days 46 and 3040% on days 79 . the postoperative course was uneventful and the patient was discharged on 10 day after surgery . histopathological investigation revealed renal carcinoma , clear cell type ( g2 ) , limited to the parenchyma , without capsule infiltration or angioinvasion . no abnormalities were revealed in ultrasonography and ct scans . computed tomography scan revealed a left kidney tumor mass . hemophilia b ( christmas disease ) is a congenital bleeding disorder resulting from factor ix deficiency . hemophilia b patients undergoing surgical procedures are at a high risk of severe bleeding complications unless surgery is performed after adequate correction of the diathesis . secure hemostasis in hemophilia b patients is achieved with transfusions of factor ix coagulation factor concentrate , either recombinant or plasma - derived . desirable preoperative activity is 6080% of the normal value and should be maintained until complete wound healing , usually through 710 days . on days 13 the recommended value is 6080% , on days 46 4060% and 3040% on days 710 . minimally invasive procedures in patients with hemophilia , apart from well - known benefits such as reduced postoperative pain , fewer complications , better cosmetic effect , shorter hospitalization , quicker convalescence and return to professional activity , also contribute to shorter duration of substitution therapy . the first laparoscopic nephrectomy was performed by clayman et al . in 1990 . since then , the procedure has been performed in an increasing number of urologic centers [ 4 , 5 ] . there are only few reports in the literature of urinary tract operations in patients with congenital coagulation disorders [ 6 , 7 ] ; nonetheless , there have been none of laparoscopic nephrectomy in a hemophilia patient . we present the first case of laparoscopic nephrectomy in a hemophiliac performed with an adrenal - saving , transperitoneal lateral approach using four trocars . no significant complications were observed either postoperatively or in the long - term , which can most likely be attributed to the adequate preparation of the hemophilia patient for surgery , as well as the effective cooperation between urologist and hematologist . perioperative and postoperative bleeding episodes are the most frequent complications reported in hemophilia patients . for patients with no coagulation disorders , tlrn intraoperative bleeds are observed at 2.22.8% , and are frequently a reason for conversion to an open procedure . there are no significant differences in the reported percentage of all surgical complications , hemorrhage included , between hemophilia patients with adequate substitution of deficient coagulation factor and patients with no coagulation disorders [ 8 , 9 ] . it is therefore crucial for hemophilia patients to undergo surgery in special reference centers , which provide expert health - care services and are equipped with a modern laboratory for careful daily monitoring of deficient coagulation factor activity [ 9 , 10 ] . in conclusion , laparoscopic radical nephrectomy in hemophilia patients is a safe and effective procedure for renal carcinoma . surgery should be performed by an experienced urologist in cooperation with a multidisciplinary team , providing both perioperative and postoperative care , supported by a specialized laboratory .

surgery in patients with hemophilia is a serious challenge . it requires a comprehensive approach , as well as careful postoperative monitoring . we present here the first case of a transperitoneal laparoscopic radical nephrectomy ( tlrn ) for renal cell carcinoma , of the clear - cell type , performed in a hemophilia b patient . the level of factor ix clotting activity before surgery and on postoperative days 16 was maintained at 65130% and at 3040% on subsequent days until healing of the post - operative wound was achieved . the intraoperative and postoperative courses were uneventful . tlrn can therefore be considered safe and effective for renal cell carcinoma . in hemophilia patients , the tlrn procedure requires proper preparation , as well as adequate substitution therapy for the deficient coagulation factor provided by a multidisciplinary team in a comprehensive center .

in critical care , as in other areas of health care , clinicians are faced with rising health care costs and aging and increasingly complex patients . furthermore , the rate of research knowledge production is outstripping our ability to incorporate this information into patient care . these factors , as well as the increasing awareness of the risks of medical error , have highlighted the potential benefits of information technology to clinical care . the paper by morrison and colleagues in the previous issue of critical care describes the impact of the introduction of an electronic patient record on interdisciplinary communication during intensive care unit ( icu ) ward rounds . critical care is a data - rich environment where it appears obvious that computing technology would be of benefit in managing the large amount of data generated by each patient , but few studies have formally evaluated the effects of introducing an information system into the icu . some studies have addressed the benefits of clinical information systems with automated data capture from icu devices , demonstrating a reduction in nursing workload , but this finding is certainly not uniform . furthermore , the reduction in common errors of omission and commission may be replaced by new errors facilitated by the technology itself . it is with this fairly limited background that the paper by morrison and colleagues provides an important insight into another potential problem introduced by computing technology in the icu . these investigators evaluated the effect of the introduction of an electronic patient record on team interactions and communication during icu rounds . in a before - and - after study of the implementation of a fully integrated electronic patient record into their 25-bed icu , they observed and video - recorded team interactions during daily rounds . in the physical setup after implementation , data were presented on a computer screen ( rather than on a large observation chart plus additional charts and folders ) and as a result were accessible to only a few team members . the attention of the group was no longer focused on the patient data and it was noted that team members had difficulty entering the conversation , impairing communication . one year after implementation , the process had improved ; the physician leading rounds stood further back from the screen and the team members reoriented themselves . staff reported preparing for the ward round by reviewing data that they would not have access to during the round . multidisciplinary communication and teamwork are essential to icu care , and impaired communication in high - intensity clinical settings has been documented . however , the paper by morrison and colleagues demonstrates that information technology may , in fact , introduce new barriers to communication . while these were overcome to some extent over a period of time by changing the format of the ward round , this is an issue that needs to be recognized , anticipated , and resolved . a single small screen may not be adequate to view the large amount of patient data generated daily , even with optimal software solutions . morrison and colleagues discuss the fact that the cost of larger screens was prohibitive and handheld devices discourage communication , while ironically a paper printout for each team member was beneficial . morrison and colleagues are to be congratulated for their foresight in evaluating an important component of their new information and communication technology . while information systems and electronic patient records may be a solution for many of the current problems in health care , this clinical intervention requires an evidence - based assessment similar to that to which other clinical innovations are subject . it is essential to identify and prevent the potential hazards and negative effects of information technology . the use of fully integrated icu clinical information systems is not yet widespread in many areas , providing the opportunity for preplanned , comprehensive , and continual evaluation during the full life cycle of implementation and use of such systems .

information and communication technology has the potential to address many problems encountered in intensive care unit ( icu ) care , namely managing large amounts of patient and research data and reducing medical errors . the paper by morrison and colleagues in the previous issue of critical care describes the adverse impact of introducing an electronic patient record in the icu on multi - disciplinary communication during ward rounds . the importance of evaluation and technology assessment in the implementation and use of new computing technology is highlighted .

a 76-year - old female presented to the emergency department with the main complaint of 7 days of dyspnea and 1 day of dysarthria and dizziness . auscultation findings showed an irregular heart beat with a diastolic rumbling murmur in the apical region and crackles in both lower lung fields . transthoracic echocardiography showed a moderate degree of aortic regurgitation with left ventricular ( lv ) ejection fraction of 70.8% and a 1.631.31-cm complex , echogenic , round , mass - like lesion attached to the left atrial side of the interatrial septum . transesophageal echocardiography revealed the same mass finding with prominent spontaneous echo contrast in the left atrium and decreased emptying velocity of the left atrial appendage ( fig . 2 ) . the diagnostic coronary angiogram revealed a round , movable mass lesion in the left atrium with feeding arteries originating from the conus branch and atrioventricular nodal artery of the right coronary artery and no significant stenosis in either coronary artery ( fig . 3 ) . the patient was transferred to cardiac surgery and underwent removal of the mass and a maze operation . macroscopic findings were compatible with myxoma , and the microscopic findings revealed an acid mucopolysaccharide - rich stroma composed of a myxoid matrix and polygonal cells with scant eosinophilic cytoplasm scattered throughout the matrix ( fig . 4 ) . after surgery , the atrial fibrillation was abolished and an electrocardiogram showed a normal sinus rhythm . follow - up transthoracic echocardiography was performed after surgery and no remnant mass was observed in the left atrium ( fig . , the patient is admitted to outpatient department regularly and has taken aspirin and angiotensin receptor blocker steadily without specific problems . the presence of possible tumor vessels originating from coronary arteries may be helpful in the decision on an operative strategy for cardiac myxoma . about 52% of cardiac myxoma is visualized by coronary angiograms according to previous reports , but catheterization of the chamber from which the tumor arises carries the risk of tumor embolization .

a 76-year - old female present to the emergency department with dysarthria , dizziness , dyspnea . the patient had hypertension and atrial fibrillation . brain mri revealed right cerebellar infarction . transthoracic echocardiography showed a large round mass in the left atrium . transesophageal echocardiography showed large complex echogenic round mass lesion attached on left atrial side of interatrial septum . coronary angiogram revealed round movable mass lesion in left atrium with feeding arteries originated from right coronary artery . she underwent removal of mass and maze operation , and pathologic finding was compatible with myxoma .

reagents were received from sigma aldrich chemical co. ( bangalore , karnataka , india ) and used without further purification . solvents were purchased from commercial sources from hyderabad , telangana , indiabased companies and purified by reported protocols . h and c nmr spectra were recorded on bruker 300 mhz and 500 mhz machines and calibrated against tetramethylsilane ( tms ) . mass spectrometric data were acquired by an electrospray ionization ( esi ) technique on a qtofmicro quadruple mass spectrometer . the stock solutions ( 110 m ) of ndin and ndina were prepared in chcl3 . an aliquot of it was transferred to various ratios of chcl3/mch in different volumetric flasks ( final volume of 2 ml ) and allowed to equilibrate for 2 h prior to the uv / vis absorption measurements . all experiments were performed upon excitation at 350 nm in a quartz cell with a 1 cm path length . the stock solutions were prepared in a similar manner as for the absorption study and employed for emission measurements . the stock solutions were prepared in chcl3 in a similar manner as for the uv / vis study . sem images of ndin and ndina were recorded on an fei nova nanosem ( hillsboro , or usa ) operating at high vacuum . for sem imaging , the samples of ndia and ndina were sputtercoated with gold for 10 s at 0.016 ma ar plasma after dropcasting the solutions on glass coverslip and solvent evaporation at rt . tem samples were prepared by the paper blotting method followed by solvent evaporation on a holey carboncoated copper grid . the liquid sample in chcl3/mch ( 5:95 , v / v ) was dropcasted and solvent was allowed to evaporate on the surface naturally . reagents were received from sigma aldrich chemical co. ( bangalore , karnataka , india ) and used without further purification . solvents were purchased from commercial sources from hyderabad , telangana , indiabased companies and purified by reported protocols . h and c nmr spectra were recorded on bruker 300 mhz and 500 mhz machines and calibrated against tetramethylsilane ( tms ) . mass spectrometric data were acquired by an electrospray ionization ( esi ) technique on a qtofmicro quadruple mass spectrometer . the stock solutions ( 110 m ) of ndin and ndina were prepared in chcl3 . an aliquot of it was transferred to various ratios of chcl3/mch in different volumetric flasks ( final volume of 2 ml ) and allowed to equilibrate for 2 h prior to the uv / vis absorption measurements . all experiments were performed upon excitation at 350 nm in a quartz cell with a 1 cm path length . the stock solutions were prepared in a similar manner as for the absorption study and employed for emission measurements . the stock solutions were prepared in chcl3 in a similar manner as for the uv / vis study . sem images of ndin and ndina were recorded on an fei nova nanosem ( hillsboro , or usa ) operating at high vacuum . for sem imaging , the samples of ndia and ndina were sputtercoated with gold for 10 s at 0.016 ma ar plasma after dropcasting the solutions on glass coverslip and solvent evaporation at rt . tem samples were prepared by the paper blotting method followed by solvent evaporation on a holey carboncoated copper grid . the liquid sample in chcl3/mch ( 5:95 , v / v ) was dropcasted and solvent was allowed to evaporate on the surface naturally . as a service to our authors and readers , this journal provides supporting information supplied by the authors . such materials are peer reviewed and may be reorganized for online delivery , but are not copyedited or typeset . technical support issues arising from supporting information ( other than missing files ) should be addressed to the authors .

abstractin the present work , two new naphthalene diimide ( ndi ) amphiphiles , ndin and ndina , were successfully synthesized and employed to investigate their selfassembly and optical properties . for ndina , which contains an amide group , aggregationinduced emission enhancement ( aiee ) was demonstrated in the presence of various ratios of methylcyclohexane ( mch ) in chloroform , which led to the visual color changes . this new amidecontaining ndina amphiphile formed nanobelt structures in chloroform / mch ( 10:90 , v / v ) and microcuplike morphologies in chloroform / mch ( 5:95 , v / v ) . the closure of these microcups led to the formation of vesicles and microcapsules . the structural morphologies gained from the solvophobic control of ndina were confirmed by various complementary techniques such as infrared spectroscopy , xray diffraction , and scanning and transmission electron microscopy . in the absence of the amide moiety in ndin , no selfassembly was observed , indicating the fundamental role of hbonding in the selfassociation process .

cysts related to the mullerian system usually occur in paraovarian ( 1 ) or paratubal regions ( 2 ) . an eleven year old girl presented to the emergency department with a twenty four history of abdominal pain , fever and nausea . on presentation she was clinically dehydrated , pyrexial , and the lower abdomen was full with tenderness in the right iliac fossa and hypogastrium . inflammatory markers showed elevated c - reactive protein of 331 mg / ml ( normal < 10 mg / ml ) and a total white cell count of 29.7 x 10/l ( range 5- 13.0 ) with a neutrophilia of 27.0 x 10/l ( range : 1.5- 8.0 ) . ( fig 1&2 ) ultrasound image of longitudinal view of multicystic pelvic mass ultrasound image of coronal view of multicystic mass following resuscitation she underwent laparotomy on gram stain only a few white blood cells were seen and fluid culture did not identify an organism . the histopathological analysis revealed both cysts to be lined focally by ciliated and non - ciliated epithelium forming plicae . however , for the major part , the cysts were lined by acute inflammatory exudate . in the paratubal and paraovarian regions the majority of the cysts are of mullerian origin and can account for up to 76% of all the cysts ( 3 ) . they are more common in adults and only occur rarely in adolescents ( 4% ) ( 3 ) . dilatation of these cysts could be anticipated after menarche , due to the secretory activity of the lining epithelium under the influence of the hormones ( 3 ) . large cysts could cause symptoms such as nausea , pain and vomiting ( 4 ) . some undergo torsion ( 5,6 ) with severe pain and other may also cause torsion of the fallopian tubes ( 2 ) . in large adult series pelvic pain and a neoplastic potential has been identified ( 6,7 ) with the incidence varying from 1.69% to 2 % . no case of inflamed paratubal cysts appears in the english literature . we can only postulate that contributory factors in our case could have included low grade infection due to previous meningitis and vp shunts . it shows a wide range of findings ( 8) , but the majority are simple cysts . multilocular cysts , as in our case are very rare accounting for only 4% of the cases ( 8) . occasionally a normal ovary abutting a cyst gives a clue to the origin of these cysts but accurate pre op diagnosis remains rare . mri may give more detailed three dimensional information but essentially features are the same as an ultrasound . kishimota et al suggested that most paraovarian cysts were homogenous , near the ipsilateral round ligament and uterus ( 9 ) . laparoscopy has been used successfully in paediatric practice to deal with large or complicated paraovarian cysts ( 4 ) . the indication for open surgery is malignancy or suspicion of malignancy , dense adhesions or large cysts ( 6 ) , as was the case in our patient . in our patient laparoscopic surgery would have been impossible due to the obliteration of the peritoneal cavity . in summary we report a very rare case of bilateral inflamed fimbrial cysts which were identified and excised during laparotomy for an acute abdomen .

paramesonephric duct remnants are an infrequent cause of abdominal symptoms in childhood . preoperative diagnosis is often difficult and diagnosis is usually made at surgery . we report a rare presentation of an acute abdomen in a child with bilateral inflamed fimbrial cysts . ultrasound revealed the presence of a multicystic lesion behind bladder . it was only at laparotomy the diagnosis of bilateral inflamed fimbrial cysts was establsihed . these were excised and the child made an uneventful post operative recovery .

a 59-year - old womon presented with an abnormal mass on chest x - ray that was discovered during a routine check - up . we obtained a biopsy though endobronchial ultrasound and pathologically diagnosed a thymoma ( type a ) . contrast - enhanced computed tomography showed a smooth , solitary homogeneously enhanced mass measuring 33.52 cm in the right paratracheal area , which had no calcification ( fig . the resected specimen was a firm light - gray tumor with a smooth capsule that measured 4.532.5 cm . postoperative follow - up proceeded without any problems , and the patient was discharged after 5 days . microscopy revealed that the tumor was a thymoma ( world health organization type a+b2 , masaoka staging i ) ( fig . the majority of thymomas are found in the anterior mediastinum . only a few thymic masses arising out of the anterior mediastinum have been described in the literature , and these have been found in an ectopic thymus location such as the neck , pulmonary hilus , or posterior mediastinum . the thymus arises embryologically from the third pharyngeal pouch and branchail cleft on each side . the thymic masses from each side then move toward each other and migrate from the midline to the anterior mediastinum and their final position . the incidence of ectopic thymic tissue is 3% to 5% in the retroinnomiate vein ( i.e. , a paratracheal site ) . surgical resection has been used for both a firm diagnosis and treatment in previously reported cases of a middle mediastinum thymoma . sakurai et al . reported that 18f - fluorodeoxyglucose positron emission tomography ( pet ) scan and 11c - acetate pet are useful for diagnosing a thymoma from the middle mediastinum as ectopic thymic tissue . castleman disease is an atypical lymphoproliferative disorder , and masses are commonly located in the chest . most patients are asymptomatic and lesions are accidentally found on chest x - ray as rounded mediastinal masses , often mistaken for a thymoma . thymomas rarely arise in the middle mediastinum , and thymoma is not considered in the differential diagnosis of middle mediastinum masses . but thymomas appear to have malignant potential , and the overall rate of thymoma recurrence is approximately 20% .

thymoma is a common anterior mediastinal mass , although thymomas have occasionally been found in the neck , pulmonary hillus , or posterior mediastinum . but a thymoma within the middle mediastinum has rarely been reported . we report a thymoma arising in the middle mediastinum with a review of the literature .

patent foramen ovale , which is necessary in fetal circulation , is a potential route for emboli arising from the venous system to enter the systemic arterial circulation , resulting in paradoxical air embolism syndrome . we report a case of paradoxical air embolism as a complication of percutaneous approach for treatment of calyceal diverticular stone . a 37-yr - old man in good health was referred to us for right calyceal diverticulum with milk of calcium . after induction of general anesthesia , the patient was placed in the lithotomy position . a 6-fr ureteral catheter with ureteropelvic junction occlusion balloon was passed through the right ureter , and the patient was then turned to prone position . after confirming the position of the ureteral catheter under fluoroscopy , the pyelogram was performed by injecting contrast and a small volume of air into the right pelvocalyceal system to locate the diverticulum ( fig . 1 ) . direct puncture of the calyceal diverticulum was attempted several times without success . the total volume of air used during the procedure was about 25 ml . the patient remained cardiovascularly stable throughout the procedure . six hours after the surgical procedure , the patient complained of weakness of his right leg . eight hours after this episode , he had sudden tonic seizure and became unconscious ; he was transferred to the intensive care unit and treated , accordingly . the patient underwent magnetic resonance imaging of the brain including diffusion - weighted imaging , which was negative . twenty - four hours after the procedure , he recovered fully without any neurological deficit and regained consciousness . neurologic evaluation showed that cryptogenic arterial air embolism was the mostly likely cause of the seizure . cardiac workup with transthoracic echocardiogram failed to reveal a cardiac source of emboli . however , transesophageal echocardiography with provocation testing showed air bubbles in the left atrium revealing that the patient had right - to - left shunt due to patent foramen ovale ( fig . 2 ) . in this case , the clinical features were strongly suggestive of paradoxical air embolism during air pyelogram . air embolism , the entry of gas into the vascular structure , is mainly a iatrogenic problem that can result in serious morbidity and even death . venous embolism occurs when air is introduced to the central venous intravascular space and embolizes to the right heart or pulmonary arterial system . arterial embolism results from the entry of gas into the left heart chambers , as with paradoxical air embolism across an intracardiac shunt or during cardiac surgery , or directly into the arteries of the systemic circulation such as during decompression barotraumas or penetrating trauma involving an artery ( 1 , 2 ) . this phenomenon was first described by lopez , who noted the passage of fluid from the calyces into the renal veins ( 6 ) . air embolism as a complication of retrograde pyelography , and air entering the hepatic veins after nephrolithotomy have also been reported ( 7 ) . the factors that influence morbidity and mortality of air embolism include the volume of air entrained , rate of entrainment , position of patient during the event , and the cardiac status of the patient . patent foramen ovale is necessary in fetal circulation to sustain intrauterine life . the closure of the foramen ovale is normally completed by the age of 2 yr . in case of incomplete closure , the foramen ovale is kept sealed by the left atrial to right atrial pressure gradient but may be opened if the right atrial pressure increases and exceeds that of the left atrium ( 8) . a persistent patent foramen ovale is thus a potential route for emboli arising from the venous system to enter the systemic arterial circulation . a patent foramen ovale and paradoxical air embolism syndrome has been increasingly implicated in cryptogenic embolic stroke . the most widely supposed mechanism for a patent foramen ovale related stroke is paradoxical air embolism of venous thrombotic material across the atrial right - to - left shunt ( 9 , 10 ) . the normal collecting system is known to have a capacity of approximately 5 - 10 cc . in this case , retrograde injection of 25 ml of air appeared to be the cause of physiologically significant air embolism , most likely via pyelovenous backflow phenomenon . in addition , clinical features of this case strongly suggested paradoxical air embolism associated with patent foramen ovale . a recent study reported that endoscopic operation under carbon dioxide pneumovesicum is effective and safe ( 11 ) . although gas embolism is usually air embolism , the medical use of other gases such carbon dioxide , nitrous oxide , nitrogen , and helium can also result in embolism . thus , endoscopic operation using carbon dioxide , which is highly water - soluble and relatively safe , carries potential risk of embolism . to our knowledge , this is the first report of paradoxical air embolism associated with patent foramen ovale during percutaneous nephrolithotomy . at present , there are no consensual guidelines on the optimal management of patent foramen ovale in patients who have suffered from cryptogenic embolic stroke ( 12 ) . urologists who prefer to use air during pecutaneous nephrolithotomy under general anesthesia should be aware of the potential risk of air embolism . injection of a smaller amount of air for pneumopyelography is strongly advisable , if necessary .

air embolism is a rare complication of percutaneous nephrolithotomy . patent foramen ovale , which is necessary in fetal circulation , is a potential route for emboli arising from the venous system to enter the systemic arterial circulation , resulting in paradoxical air embolism syndrome . a case of paradoxical air embolism during percutaneous nephrolithotomy is presented . to our knowledge , this is the first report of paradoxical air embolism associated with patent foramen ovale during percutaneous nephrolithotomy .

fundus examination revealed a round - shaped hypopigmented macular lesion including a few yellow spots in the fovea [ fig . fa showed a macular transmission hyperfluorescent ring in which patchy hyperfluorescent and normofluorescent areas existed [ fig . fundus autofluorescence imaging showed scattered patchy hyperautofluorescent areas matching the hypopigmented fundus lesion [ fig . spectral - domain optical coherence tomography ( sd - oct , spectralis oct , heidelberg engineering , heidelberg , germany ) demonstrated hyper reflective accumulations ( humps ) involving rpe inner band and photoreceptor outer segments ( oss ) . outer nuclear layer and inner segment / os ( is / os ) line was distorted because of the accumulations . multifocal electroretinogram ( mferg , roland - consult retiscan system , wiesbaden , germany ) showed decreased amplitude in the central 2.3. the p1 amplitude in the central response was 50.3 nv / deg [ fig . arden ratios in electrooculogram ( eog ) were 2134 in the right eye and 1650 in the left eye . total error score in farnsworth - munsell-100 ( fm-100 ) hue test was 35 in the right eye and 157 in the left eye . the patient reported a viral ( possibly ) upper respiratory tract infection 710 days prior to the visual loss . a probable diagnosis of arpe was suggested , and the patient was followed in next weeks . ( a ) fundus photograph demonstrating round - shaped hypopigmented macular lesion including a few yellow spots in the fovea . ( c ) fluorescein angiography showing a macular transmission hyperfluorescent ring in which pathcy hyperfluorecent and normofluorescent areas existed . ( e ) spectral - domain optical coherence tomography image showing hyperreflective accumulations ( humps ) involving rpe inner band and photoreceptor outer segments . ( f ) multifocal electroretinogram showing depressed central ring amplitude best - corrected visual acuity improved gradually up to 20/20 in the next 5 weeks with no treatment . there was almost no change in fa and fof imaging compared to the initial imaging [ fig . 2c and d ] . sd - oct showed almost entirely restored is / os line almost no distortion in the outer nuclear layer . irregularities and humps decreased , but there were still some persisting hyper reflective spots in the rpe inner band and photoreceptor oss [ fig . the p1 amplitude of the central response increased to 114 nv / deg in mferg [ fig . arden ratios were 2080 in the right eye and 2071 in the left eye [ fig . 3 ] . the patient reported significantly improved central vision in amsler grid testing when compared with the initial testing . total error scores in the fm-100 hue test was 42 in the right eye and 83 in the left eye . ( e ) spectral - domain optical coherence tomography image showing the decreased hyperreflective accumulations ( f ) . multifocal electroretinogram showing normalized central ring amplitude electrooculogram recordings in the right ( a ) and left eye ( b ) during the initial examination and at the 5th week examination ( c and d , respectively ) the diagnosis of arpe depends on the presence of a characteristic fine pigment stippling in the macular area , at the level of the rpe , surrounded by yellow - white haloes of hypopigmentation . spectral - domain optical coherence tomography findings suggest that the initial lesion in arpe is possibly located at the level of the photoreceptor os and rpe . the disruption of photoreceptor is / os line with a wider disruption of the rpe inner band is almost typical in the reported cases . the rpe inner band is considered to be related with the verhoeff 's membrane that is constituted by tight junctions of rpe cells or with basal infoldings and apical processes of rpe cells . in our case , however , photoreceptor is / os line is discernible throughout the lesion . the main finding in our case was humps in the inner band of the rpe and photoreceptor oss . our findings support the speculation that arpe is a postinflammatory response of the os of photoreceptors and the rpe . eog explores the response of rpe to light and is a function of all rpe cells in the retina . that is probably related with the increased metabolic need of rpe cells in the macula with respect to rpe cells in the peripheral retina . the localized accumulation in sd - oct images likely reflects undigested oss of the photoreceptors . the diminished central amplitudes with progressively increasing amplitudes toward periphery in mferg confirm the functional deterioration of cone photoreceptors in the central retina . the electrophysiologic findings may mean that rpe cells in the peripheral retina can overcome the increased metabolic load although the rpe cells in the macula can not due to the high concentration of photoreceptors . our case showed that mferg recording was more correlated to visual acuity than structural imaging modalities ( fa , fof , sd - oct ) . spectral - domain optical coherence tomography and mferg findings in our case support the hypothesis that rpe is possibly the initial site of involvement in arpe . mferg depression confirmed the functional involvement of cone photoreceptors in the disease process .

a 20-year - old man applied with vision loss in the left eye . right eye examination was unremarkable . best - corrected visual acuity ( bcva ) in the left eye was 20/200 . fundus examination revealed a few yellow spots within a round - shaped macular lesion . autofluorescence imaging showed hyperautofluorescence in the lesion . central amplitudes in multifocal electroretinogram ( mferg ) were depressed . the patient reported a rhinopharyngitis 710 days before the visual loss . the patient was diagnosed as acute retinal pigment epithelitis . bcva improved gradually up to 20/20 in 4 weeks . mferg amplitudes returned to normal . a slight pigmentary distortion was the only residual fundus finding .

it is caused by a deficiency of both hexosaminidase ( hex ) a and b , resulting in accumulation of glycosphingolipids and oligosaccharides in the brain . it has three clinical subtypes ( infantile , juvenile , and adult forms ) and represents around 7% of cases among all the lysosomal storage disorders . the infantile form presents in the first 618 months of age with regression of milestones , developmental delay , startle response , hypotonia , cherry red spots , and convulsions . we report this case as the infant presented with regression without hepatosplenomegaly and confirmed by gene testing . a 1-year - old second born male child , born to a third - degree consanguineously married couple with uneventful perinatal history , was brought with regression of milestones and seizures . the onset of clinical symptoms began at the age of 6 months with gradual loss of the milestones . the child had achieved response to sounds by 2 months ; head holding , social smile , and recognition of mother by 3 months of age . initially , he lost social smile and recognition of mother followed by control of neck . he lost all the milestones by 89 months of age . the child developed exacerbated startle response since 8 months of age and multiple episodes of right - sided focal seizures from 11 months of age . there was a history of sibling death at the age of 18 months with similar complaints . on examination , head circumference was 44 cm ( between 3 and 15 centile ) with coarse facies . the tone was increased in all the limbs with power of 3/5 ( medical research council grade ) . fundus examination showed cherry - red spot in the macula , and there was no hepatosplenomegaly . magnetic resonance imaging ( mri ) shows hyperintensity of the basal ganglia ( long white arrow ) and hypointensity of the ventral thalami ( open arrow ) on t2-weighted sequences [ figure 1 ] . based on above findings suspected to be a case of sandhoff disease , the enzyme assay in leukocytes for hexosaminidase total ( a + b ) revealed 61 nmhol / h / mg ( normal , 9052878 ) . gene testing was positive for homogenous missense substitution p. cys534tyr , c1601g > a , chr5:74016560g > a in exon 13 of hexb gene . axial t2-weighted imaging showing hyperintensity of the basal ganglia ( long white arrow ) and hypointensity of the ventral thalami ( open arrow ) our patient had presented with regression of milestones , exaggerated startle response , decreased vision , and seizures . the child had coarse facies , cherry red spot , and normocephaly without hepatosplenomegaly . in a study conducted among 18 gm2 gangliosidosis patients by karimzadeh et al . , seven patients had macrocephaly , three patients had microcephaly , and eight patients had a normal head circumference . a distinguishing feature in standoff 's disease from other gm2 gangliosidosis is presence of hepatosplenomegaly and n - acetylglucosamine - containing oligosaccharides in urine likewise , karimzadeh et al . also noted , and only two patients of sandhoff disease out of their nine cases had hepatosplenomegaly . similar observation was made by ozkara et al . in their study of 18 cases affected by sandhoff disease , and hepatosplenomegaly was not found in 11 out of 18 infantile sandhoff disease patients , while the remaining seven had mild hepatosplenomegaly . closest differential diagnosis of sandhoff disease is tay sachs disease , which is characterized by the absence of coarse facies , hepatosplenomegaly , skeletal deformities , and signs of peripheral nerve involvement . axial t2-weighted imaging on mri in our patient revealed hyperintensity of the basal ganglia and hypointensity of the ventral thalami . also reported as bilateral thalamic hyperdensity on computed tomography and hypointensity on t2-weighted mri images as the earliest diagnostic markers of sandhoff disease . the gold standard method for diagnosis of gm2 gangliosidosis is the measurement of -hex activity in plasma , serum , and/or fibroblasts . molecular characterization of the hexa and hexb mutations can also be performed for confirmation by direct sequencing of the entire coding region and intron / exon boundaries using genomic dna . the same mutation was reported previously in a japanese case which showed hepatosplenomegaly unlike our case . prenatal diagnosis advised for next pregnancy . sachs disease if any child presenting with regression of milestones and cherry red spot without hepatosplenomegaly .

sandhoff disease is a neurodegenerative disease caused due to deficiency of hexosaminidase ( hex ) a and b. a 1-year - old male child presented with regression of milestones , exaggerated startle response , decreased vision , and seizures from 6 months of age . the child had coarse facies without hepatosplenomegaly . serum levels of hexosaminidase total ( a + b ) were low . genetic testing for sandhoff disease revealed a homozygous missense variant on hexb gene . the case is presented to highlight that the absence of hepatosplenomegaly should not restrain in suspecting sandhoff disease .

although several surgical interventions have been developed , mortality and complications related to surgical management remains high , especially with respect to trans - ventricular approach . ventricular incision also has several disadvantages : increased postoperative bleeding , ventricular malfunction , and ventricular arrhythmia . in the present work , we report a successful case of repair of posterior ventricular septal rupture ( vsr ) via transatrial approach . a 73-year - old woman presented to a local hospital with shortness of breath and anterior chest pain . the electrocardiogram ( ecg ) demonstrated st - segment elevations in the inferior leads , but regional wall motion abnormality was not noted on the transthoracic echocardiogram . the coronary angiogram showed severe stenosis at the middle of the right coronary artery ( fig . 1 ) . the left coronary catheterization revealed mild stenosis in the middle left anterior descending coronary artery . percutaneous coronary intervention ( pci ) was performed at the right coronary artery by stent insertion . after pci , sinus tachycardia changed to ectopic junctional rhythm on the ecg , and severe hypotension was noted . the additional transthoracic echocardiogram revealed vsr at the basal inferoseptal wall and severe tricuspid regurgitation with right ventricular dilation ( fig . a median sternotomy was performed , and cardiopulmonary bypass ( cpb ) was established via ascending aortic and bicaval cannulation . vsr was noted at the basal inferior septum measuring approximately 1.5 cm in diameter ( fig . the septal and posterior leaflet and a few chordae were resected for more consistent access to the vsr . eleven stitches of pledget buttressed by 2 - 0 prolen were sutured by the method of interrupted horizontal mattress along the ventricular septal defect ( vsd ) margin . the stitches near the septal leaflet were sutured through the annulus of the septal leaflet , and the stitches for the inferior margin were sutured through the inferior free wall of the right ventricle . these stitches were passed through the trimmed teflon felt and tied into place without difficulty . tricuspid valve replacement was performed with a carpentier edward valve ( edwards lifesciences , irvine , ca , usa ) by using a supra - annular technique ( fig . although pci for right coronary artery lesion was done successfully , we decided to perform a right coronary artery bypass to be prepared for the possibility of acute thrombosis . the distal right coronary artery was bypassed with a saphenous venous graft in an end - to - side fashion . the immediate postoperative transthoracic echocardiogram ( tte ) revealed right ventricular dysfunction , and the iabp was removed from the patient on postoperative day 4 . there was no evidence of residual vsr , and right ventricular function was in the normal range on the tte . several risk factors are associated with post - infarct vsr : first infarction , inferior location , transmural infarction , complete and sudden occlusion of a coronary artery , poor collateral blood flow , and left ventricular hypertrophy.1)2 ) with the development of percutaneous coronary intervention , the incidence of postinfarct vsr has been reduced ; however , mortality remains high.3 ) post - infarct vsr is associated with an 87% mortality within 2 months if managed medically.4 ) previously , many surgeons delay surgical intervention while waiting for myocardial fibrosis to occur because this facilitates surgical repair . more recently , the majority of surgeons have advocated more aggressive management , with immediate start of iabp and urgent repair of vsr . in general , most surgeons conduct the operation via left ventriculotomy or via the infarcted free wall . however , in the case of posterior vsr , access is difficult through left ventriculotomy or infarcted free wall.5 ) ventricular incision also has several disadvantages : increased postoperative bleeding , ventricular malfunction , and ventricular arrhythmia . in 1986 , filgueira et al.6 ) described the transatrial approach to repair the vsr in order to avoid some of the problems of ventricular incision . it is difficult to expose the vsr and to accurately identify the vsd due to the trabeculation of the right ventricle . in addition , the placement of the stitch through the tricuspid valve may interfere with the subvalvular apparatus , and tricuspid regurgitation can occur . however , the tricuspid valvectomy method requires tricuspid valve repair after vsr repair . in the present case , we performed a tricuspid valve replacement instead of a repair to avoid the risk of tricuspid regurgitation that might occur if the valvular mechanism is compromised during exposure and patch closure of the vsr . moreover , inferior myocardial infarctions may result in right ventricular dysfunction and low cardiac output syndrome postoperatively , which can worsen due to residual tricuspid regurgitation . accurate identification of vsr is crucial to avoid residual shunt flow and can be achieved by injecting blood - tinged saline solution through a left ventricular vent catheter placed via the right superior pulmonary vein . massetti et al.7 ) found that posterior vsr could be successfully repaired via the transatrial approach . by avoiding additional damage to the ventricle lee et al.8 ) reported a successful right atrial approach operation for post - infarction rupture of the posterior ventricular septum . however , unlike in our case , the operation was conducted 12 days after infarction . in conclusion , many surgeons advocate the use of ventriculostomy or infarcted tissue for the treatment of postinfarct vsr ; however , posterior septal rupture can be repaired successfully through an alternative transatrial approach , thus avoiding the complications that can occur due to ventriculotomy .

ventricular septal rupture ( vsr ) is a disastrous mechanical complication of myocardial infarction . although several surgical interventions have been developed , mortality due to surgical management remains high , especially in the case of posterior vsr . we report a successful case of repair of posterior vsr using an alternative transatrial approach to avoid the complications related to ventricular incision .

a 24-year - old male was admitted to our hospital with a 1 week history of progressive headache and fever . his previous medical history was clear . on the neurologic examination , his mental status was normal without focal neurological deficits . lumbar cerebrospinal fluid ( csf ) analysis showed pleocytosis , increased total protein level ( 167 mg / dl ) and decreased glucose ( 32 mg / dl ) . tubercle bacillus was detected in the csf and the adenosine deaminase ( ada ) level was elevated ( 12 brain mri revealed small , multiple high signal intensity lesions on the diffusion weighted image . his chest x - ray showed diffuse ill - defined patches and nodules with increased density in both apical lungs . tuberculosis meningitis and pulmonary tuberculosis were suspected and antituberculous treatment with rifampicin , isoniazid , ethambutol and pyrazinamide was started . nine days after the antituberculous treatment , his general condition and headache had improved significantly . however , four weeks after antituberculous treatment , he developed acute sensory disturbance below t10 dermatome as well as urination disturbances . spinal mri showed a long segmentally located soft tissue mass mimicking a meningioma in the intradural , extramedullary space of the posterior spinal canal from t2 to t6 with severe cord compression and displacement ( fig . 1 ) . surgical decompression and a biopsy were performed . during the operation , a t3 , the dura was thickened and the cord was infiltrated with an adhesive mass . the dura was opened and closed in the normal manner after removing the mass under microscope guidance . grossly , the mass was very soft with a dark green color . the cultures were negative for mycobacterium tuberculosis , and the stains for fungi and acid - fast micro organisms were negative . mri of the thoracic and lumbar areas showed lumbosacral tuberculosis spondylitis with multiple abscesses ( fig . anti - tuberculous medication was continued for 18 months . at the one year follow - up spinal involvement in tuberculosis is classified into four categories : potts spine , nonosseous spinal tuberculoma , tuberculous arachnoiditis and tuberculous meningitis . intradural spinal tuberculomas are estimated to be composed of only 2% to 5% of central nervous system tuberculomas.1,3 - 5 ) compton and dorsch8 ) reported 11 cases of intratudral extramedullary tuberculoma in 1984 . since then , there have been 19 more cases reported in the english literature.1,3,5 - 9 ) all cases , except for 4 initially presented with tubculous meningitis . most case reviews involved the thoracic spine.1,3,5 - 10 ) the diagnostic method is generally histopathology , even though afb cultures from the granulomas were positive in only one of the reported cases.7 ) a myelogram can be helpful but mri is the diagnostic procedure of choice . interestingly , most intradural extramedullary tuberculoma cases were detected after antibuberculous therapy had been initiated , which is known as paradoxical response.2,4,7 ) tuberculoma can occur at any time but most reported cases showed a paradoxical response during the early course of chemotherapy , which ranged from 3 weeks to 1 year.2,4,7 ) the mechanism for the paradoxical response is unclear . however , is is believed to be the result of an interaction between the host 's immune response and the direct effects of mycobacterial products.2,3,7,10 ) this condition has been recognized more frequently . narita et al.10 ) reported paradoxical worsening in up to 36% of patients with tuberculosis and aids after antiretroviral therapy . therefore , mental or focal neurological changes during the follow - up must be checked in patients with tubeculous meningitis.2,10 ) the prognosis for neurological improvement is good with a prompt surgical excison and appropriate antituberculous medication.3,4,6 ) although intramedullary tuberculoma can be treated with medication alone , an intradural extramedullary tuberculoma is essential for surgery when compression of spinal cord occurs . en plaque meningioma can present with thoracic spinal cord and nerve root compression.1 ) the case presented herein was initially diagnosed with en plaque meningioma based on the radiological findings . the differential diagnosis between tuberculous patchy meningitis and meningioma in the form of plaque is difficult without obtaining a biopsy specimen . in conclusion , intradural extramedullary tuberculoma can occur in tuberculous meningitis patients as a paradoxical response to antituberculous chemotherapy . although quite rare , intradural extramedullary tuberculomas should be considered in a differential diagnosis of an en plaque meningioma of the spinal cord .

a 24-year - old man with tuberculosis meningitis developed acute paraplegia and sensory disturbances 5 weeks after receiving conventional antituberculous therapy . magnetic resonance imaging revealed an intradural extramedullary long segmental mass mimicking en plaque meningioma at the t2-t6 vertebrae levels . prompt surgical decompression was performed . a histology examination of the mass revealed a tuberculoma . after surgery , the patient showed improved motor power and a normal bladder function . intradural extramedullary tuberculoma of the spinal cord is rare complication of tuberculosis meningitis , which can occur as a response to conventional antituberculous therapy .

ticks were sampled in the villages of diiso and el - humow and at the livestock market and abattoirs in garissa district , north eastern province of kenya , during april may 2008 ( figure ) . garissa district is in a semi - arid to arid ecologic zone that receives sporadic rainfall from march to may ; vegetation consists primarily of acacia - commiphora bushes . its population is largely composed of nomadic herders who travel between districts in northern kenya in search of water and pasture ( 8) . location of garissa district ( a , box ) in north eastern province , kenya , and tick collection sites ( b ) . ticks were picked by hand from infested livestock , stored in labeled sterile vials , and transported in liquid nitrogen to the kenya medical research institute laboratory . in the laboratory , ticks were washed in sterile water , rinsed first with 70% ethanol , and then rinsed with minimum essential medium containing antimicrobial agents ( 100 u / ml penicillin , 100 g / ml streptomycin , and 1 l / ml amphotericin b ) . they were identified to species by using taxonomic keys ( 9,10 ) and pooled in groups of 2 to 10 by species , sex , collection date and site , and host . the tick pools were homogenized by using 90-mesh alundum sand in a prechilled , sterile mortar and pestle with 1.6 ml2 ml ice - cold bovine albumin 1 medium ( 1 medium 199 with earle salts , 1% bovine albumin , 100 u / ml penicillin , 100 g / ml streptomycin , and 1 l / ml amphotericin b ) under high containment . the homogenates were clarified by centrifugation at 1,500 rpm for 15 min at 4c , and supernatants were stored at 80c . viral rna was extracted from tick homogenates by using trizol - ls ( invitrogen , carlsbad , ca , usa ) reagent , according to the manufacturer s instructions . rna was screened by reverse transcription pcr ( 11 ) to amplify a 536-bp fragment of the gene encoding for the nucleocapsid protein in the small ( s ) segment of the cchfv genome by using the following primers ( 12 ) : cchf f2 ( 5-tggacaccttcacaaactc-3 ) and r3 ( 5-gacaaattccctgcacca-3 ) , positions 135153 and 653- 670 , respectively , on the reference strain cchfv 10200 . electrophoresis of the pcr products was performed by using 1% agarose gels in tris - acetate - edta buffer containing ethidium bromide ; product bands were visualized and documented with the canon uvp photodoc - it gel imaging system ( uvp , llc , upland , ca , usa ) mounted with a digital camera . the pcr products of a subset of 4 of the cchfv - positive homogenates were purified by using the qiaquick pcr purification kit ( qiagen sciences , germantown , md , usa ) , according to the manufacturer s instructions , and sequenced by using the bigdye terminator version 3.1 cycle sequencing kit ( applied biosystems , foster city , ca , usa ) and the abi 3730 and automated 3130xl genetic analyzer ( applied biosystems ) . the sequences were analyzed by using the basic local alignment search tool ( blast ; http://blast.ncbi.nlm.nih.gov/blast.cgi ) and the genbank database to confirm the identity of the virus . data ( including tick species , collection site , animal host , and virologic test results ) were entered into an excel database ( microsoft corp . , redmond , wa , usa ) and analyzed by using pivot tables . a total of 8,600 ticks , of 3 genera and 8 species , were sampled primarily from camels , cattle , goats , and sheep , principally hyalomma rufipes and hy . ticks of the genus hyalomma were sampled 3 more frequently in diiso than in el - humow ( table ) . rufipes ( 3 from cattle and 1 from a camel ) , 18 pools of hy . truncatum ( 14 from cattle and 4 from camels ) , and 1 unidentified hyalomma species ( table ) in which single dna bands corresponding to the predicted 536-bp pcr product were detected . * cchfv , congo - crimean hemorrhagic fever virus . the detection of cchfv in pools of hyalomma spp . ticks from diiso village and the garissa district slaughterhouse provides strong evidence of cchfv presence in northeastern kenya and indicates that cchfv circulation in kenya is underestimated . livestock play a role in the amplification of the virus because the animals become viremic for 7 days ( 2,3 ) , during which time they can infect more ticks . our findings indicate that cchfv circulates in northeastern kenya with substantial involvement of camels and cattle . the detection of cchfv in ticks from camels at the slaughterhouse also suggests the potential of exposure for abattoir workers . the presence of cchfv among hyalommid ticks in northern kenya highlights the risk to the resident population and requires the assessment of human exposure . health care workers must therefore help create awareness among the population and take steps to prepare for and prevent outbreaks .

as part of ongoing arbovirus surveillance , we screened ticks obtained from livestock in northeastern kenya in 2008 to assess the risk for human exposure to tick - borne viruses . of 1,144 pools of 8,600 hyalomma spp . ticks screened for congo - crimean hemorrhagic fever virus by reverse transcription pcr , 23 pools were infected , demonstrating a potential for human exposure .

malignant peripheral nerve sheath tumor ( mpnst ) accounts for about 5 - 10% of all soft tissue sarcomas . the fact that the presence of this tumor in the buttock region is extremely rare has prompted the authors to report this case . this is an interesting tumor not encountered very much in the neuro - oncology literature . it can occur in sporadic form or over a setting of neurofibromatosis-1 ( nf-1 ) . when mtt develops over nf-1 , the diagnosis can be confirmed based on morphologic grounds supported by an immunostain such as s-100 protein . a 40-year - old male presented with a large mass in the gluteal region gradually increasing in size over 6 months . ten years back , operation of a nodule in the same area had revealed histopathological diagnosis of neurofibroma . on further questioning , the patient revealed having two small painful nodules over his back since the presence of the operated nodule . his family history suggested that his father was a diagnosed case of von recklinghausen disease ( nf-1 ) . the resected mass consisted of a large globular mass measuring 10 7 6 cm . the cut section showed solid grey - white and hemorrhagic areas ( fig . 1 ) . multiple sections studied revealed alternate hypocellular and hypercellular areas composed of spindle cells with wavy nucleus and nuclear palisading . thick - walled blood vessels were seen with perivascular accentuation of tumor cells in the hypocellular areas ( fig . mitotic figures were brisk in the cellular areas , 11/10 high power fields with few abnormal mitoses . the tumor also showed a good number of scattered large cells both rounded and elongated with abundant deep eosinophilic cytoplasm and clearly visible cross striations ( fig . the patient was subjected to radiotherapy on being referred to the oncology department . during a follow - up period of 8 months , slightly more than half of the cases of mtt have been reported in conjunction with nf-1 . in our case , when it occurs in sporadic form , other spindle cell sarcomas like fibrosarcoma , malignant fibrous histiocytoma and rhabdomyosarcoma can come as differential diagnosis . the tumor develops after a long latent period of 10 - 20 years . in our case there is an agreement that the diagnosis of mpnst can be on morphologic grounds supported by positivity for s-100 protein . the morphologic features are ( a ) alternating hypocellular and hypercellular regions , ( b ) the appearance of thin wavy comma - shaped / bullet - shaped nucleus in the hypocellular areas , ( c ) presence of nuclear palisading , ( d ) presence of nerve whorls or tactoid bodies resembling wagner - meissner corpuscles , ( e ) prominent thick - walled vasculature , and ( f ) presence of heterologous elements like rhabdomyoblasts , cartilage and bone . such tumors show focal positivity for s-100 protein in 50 - 90% of cases , suggesting a nerve sheath origin . . the 5-year survival rate of mtts is only 5 - 15% in contrast to mpnsts where it is 50 - 60% . integrated positron emission tomography and computed tomography have been used to assess remission and response to therapy . the prognosis of mtt depends on the location , grade and completeness of surgical margins . it is good for the head , neck , extremities and worse for other sites including the buttock . in a study it was observed that mtt in association with nf-1 has a poor prognosis compared to sporadic forms .

malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation , malignant triton tumor , has a rare incidence . we report such a case in a 40-year - old male who presented with a mass over the buttock . he was a previously diagnosed case of neurofibroma in the same area . histomorphology supported by immunostaining with s-100 protein confirmed the diagnosis . malignant triton tumor has a poor prognosis owing to its aggressive biological behavior . the fact that the presence of this tumor in the buttock region is extremely rare has prompted the authors to report this case .

multiple myeloma is a monoclonal malignant neoplasm of plasma cell origin which occurs in the bone marrow and may result in extensive destruction of skeletal structures . it most commonly affects the skull , vertebrae , and pelvis , with pain , fatigue , swelling , and anemia being some of the most common clinical presentations . an 83-year - old african american female presented to the emergency department with mild pain and swelling over the posterior left mandibular alveolar ridge [ figure 1 ] . the lesion was approximately 2 cm 2 cm 1.5 cm in the area of the missing first molar . on palpation , it was firm with no fluctuance . radiographically , an ill - defined radiolucency of the alveolar bone was identified [ figure 2 ] . initial clinical presentation of the patient with gingival swelling between teeth 18 and 20 radiograph showing an ill - defined radiolucent area in the bone between teeth 18 and 20 an incisional biopsy revealed a malignant neoplasm of hematopoietic origin with sheets of malignant plasma cells ranging from mature to immature forms as well as pleomorphic - cells and occasional binucleated cells [ figure 3 ] . the neoplastic plasma cells were strongly positive with kappa light chain antibody and with cd138 [ figure 4 ] , both supporting the histological diagnosis of plasmacytoma . ( a ) low magnification shows stratified squamous mucosa on right upper end with cellular infiltrate in left lower portion of the image ; ( b ) magnification of h and e histology shows sheets of plasma cells , most are large and atypical with large nuclei and prominent nucleoli immunohistochemistry stain for cd138 antibody . note uniform positive staining with cd138 , a marker for plasma cells a bone marrow aspirate biopsy was performed on the left posterior iliac crest where sheets of neoplastic plasma cells were identified . a random urine collection showed elevated bence - jones protein ( 0.09 g ) and a subsequent 24 h urine collection demonstrated a level of 0.13 g ( normal 0.0500.080 ) . the disease is usually found in the sixth and seventh decades of life with a median age of 62 , 66 , 71 . it is a multicentric and generalized bone marrow disease that affects multiple bones and can include the jaws . in a review of 193 patients with diagnosed multiple myeloma , epstein et al . reported that out of 783 multiple myeloma patients , 14.1% had oral manifestations in the form of jaw pain , severe periodontitis , tooth mobility , bone destruction , pathologic fracture , paresthesia , and soft tissue swelling . usually , if the jaws are involved , it is an indication of an advanced stage of the disease . in the case we report , no other lesions were identified on the osseous skeletal survey performed the same month as the biopsy . this patient had a systemic manifestation of multiple myeloma despite the jaw being the only bone affected ; she had mild anemia , elevated serum calcium , and bence - jones protein in her urine . she also had sheets of neoplastic plasma cells in her bone marrow biopsy performed on the left posterior iliac crest . the neoplastic plasma cells were positive with cd138 antibody confirming the presence of the disease in an area away from the jaw . we present a case of multiple myeloma first manifesting in the mouth as a mildly painful gingival swelling with underlying irregular bone destruction . we recommended that all patients receive a routine oral examination by their dentist and primary care physician to insure early recognition of malignant neoplasms of the mouth .

it is rare that multiple myeloma ( mm ) occurs as a primary lesion in the jaws ; we report such a case in an elderly patient involving the gingiva of the left posterior mandible . multiple myeloma is a monoclonal malignant neoplasm of plasma cell origin which occurs in the bone marrow and may result in extensive destruction of skeletal structures . if the jaws are involved , it usually indicates an advanced stage of the disease.thi s makes our case very unique due to the fact no other osteolytic lesions were identified at the time of the diagnosis of multiple myeloma . we report a rare case of multiple myeloma which was diagnosed from an intraoral gingival lesion on the lower left mandible .

natural orifice transluminal endoscopic surgery ( notes ) has recently generated significant interest amongst surgeons and gastroenterologists . pushing minimally invasive surgery a step forward , the concept of incisionless surgery is appealing to patients and physicians alike . accessing the abdominal cavity and its organs via natural orifices , such as the mouth , anus , vagina , and urethra , may enable surgeons in the future to approach operations they traditionally performed in open and laparoscopic fashions . the potential benefits of such techniques include less physiologic stress and trauma ; faster recovery ; less pain ; fewer complications , such as intestinal adhesions and hernia ; better cosmesis ; and decreased healthcare cost by decreasing the rate of hospitalization . several operations including cholecystectomy , splenectomy , appendectomy , gastrojejunostomy , and oophorectomy have been performed . however , little data are available in humans . a 61-year - old woman with ulcerative colitis refractory to medical therapy presented to our department following numerous admissions for anemia requiring blood transfusions . she had a history of deep venous thrombosis and pulmonary embolism status post placement of an inferior vena cava filter , cerebrovascular disease with left hemiplegia , and coronary artery disease . she underwent an uneventful proctocolectomy with end ileostomy . on postoperative day 1 , the patient developed phlegmasia cerulea dolens , and an mri revealed thrombosis of her inferior vena cava and iliac veins . transanal drainage through the anal cuff was performed at the bedside with irrigation of the lower pelvis with a mushroom catheter , and antibiotic therapy was instituted . the patient remained septic and 2 days later underwent placement of a percutaneous drain by interventional radiology . the drain was ineffective due to the organized nature of the infected hematoma with multiple septations and phlegmonous reaction . re - exploration and drainage of the abdomen was entertained , but due to the frailty of the patient , a transanal endoscopic drainage was performed on postoperative day 17 in the endoscopy suite . room air under pressure was used to insufflate the abdominopelvic cavity for visualization . a large , multiseptated and organized intraperitoneal hematoma was encountered with the pigtail drain embedded in a fibrinous collection ( figure 2 ) . following mechanical and hydrogen peroxide fragmentation , the hematoma was retrieved with suction , forceps , and baskets , and the pelvis and lower abdomen were cleared ( figure 3 ) . ct scan images were used to guide the depth of intervention to minimize any injury to small bowel . two 19 french round blake drains were introduced via the anal opening and guided into the abdomen with the use of an endoscopic snare . the patient 's clinical status rapidly improved , and her white count normalized within 2 days . the evolution of minimally invasive surgery over the last 2 decades has redefined the practice of surgery . driven by technological advances and supporting clinical data , the implementation of these new techniques has been remarkable and has stimulated inquiry into future directions . undoubtedly , most of the initial human data will come from case reports and short series attesting to the feasibility , safety , and limitations of notes . rao and colleagues from india have already demonstrated that appendectomy via a transgastric route is feasible in humans . although transanal drainage of pelvic sepsis ( the bedside drainage we performed in our patient as initial intervention ) is a known procedure in the surgical armamentarium , this report illustrates the utilization of flexible endoscopy to reach a higher location in the abdominopelvic cavity and to perform more extensive drainage of a loculated infected hematoma . none of these interventions were successful due to the location , extent , and nature of the organized hematoma , and the overall debilitated state of the patient . although commonly used , percutaneous drainage is not always successful at controlling abdominal sepsis . in our case , re - exploratory laparotomy was the only remaining option to evacuate the patient 's infected hematoma . considering her clinical status , such an intervention would have carried significant morbidity . with the patient 's full consent , obviously in this case , the rectum was surgically missing so access to the peritoneal cavity was unhindered , and it was not necessary to close the anorectal stump . had the rectum been present , a transanal proctotomy would have been necessary to gain access to the abdomen . but this case illustrates that it is technically feasible to endoscopically tackle the postoperative abdomen , fragment , retrieve , and drain infected hematoma with current equipment . the equipment and endoscopic expertise to perform such a task in a routine , reliable , and safe fashion are currently limited but growing . in addition , several endoluminal methods and devices are being developed to gain entry access into the peritoneal cavity through the digestive tract . notes is a field in its infancy , and whether it will gain widespread acceptance and application is yet to be determined . although most advances in this field will be driven by animal experimentation , some may result from challenging situations such as our case that may provide opportunities to push the boundaries of our current surgical practices .

natural orifice transluminal endoscopic surgery ( notes ) is an evolving experimental field exploring the technical feasibility and outcome of therapeutic interventions performed through the natural orifices of the body . the knowledge accumulating in notes is the result of animal experimentation and ongoing early clinical experience in humans . in this report we describe a patient treated with transanal endoscopic drainage of postoperative abdominopelvic sepsis .

a 28-year - old unmarried male from low socioeconomic status came to the hospital for consultation of psychiatric problems along with his caregivers . the presenting complaints were unprovoked aggressive outbursts , poor self - care , disorganized behavior and self - mutilating behaviors for last 4 years . physical examination revealed old burnt scar over the forehead , fused ear lobules bilaterally and polydactyly in right upper limb and crush injury of left thumb and index finger . on mental status examination , he was diagnosed as undifferentiated schizophrenia according to international classification of disease-10 version . patient was advised to take in - patient psychiatric care on the same day of consultation . he remained aggressive , disorganized and on two occasions , he tried to crush his left index finger and thumb by hammering it with a stone . he was started on electro convulsive therapy ( ect ) sessions considering aggressive outburst and self - mutilating behavior . during pre - ect evaluation computed tomography scan of the head revealed grade ii csp [ refer figure 1 ] . along with ect , patient also received oral chlorpromazine 600 mg / day , which was hiked gradually over 2 weeks . plain computed tomography image of the patient and arrow head pointing towards cavum septum pellucidum patient started showing improvement in aggressive and self - mutilating behavior . on mental status examination , he reported that he became angry for no apparent reason , following which he would start hammering his fingers with stones . he received nine sessions of ect with minimal cognitive impairment and was continued on oral chlorpromazine 600 mg / day without any side effects . there was improvement in self - mutilating behavior , disorganized behavior and poor self - care , but occasional aggressive outbursts persisted . anatomically , the septum pellucidum consists of two laminae of tissue composed of white matter surrounded by gray matter . the septum is known to be a part of the limbic system and is connected via the medial forebrain bundle to the hypothalamus and via the fornix to the hippocampus and amygdala . csp is present in 100% of fetuses and premature infants , but the posterior half of the leaves normally fuse by 36 months of age . the presence of a csp later in life reflects neurodevelopmental abnormalities of structures bordering the septum pellucidum , such as the corpus callosum and hippocampus and may be considered as a marker of limbic system dysgenesis . despite being a normal variant , csp has been seen in various psychiatric disorders , most commonly in schizophrenia . in a recent meta - analysis , trzesniak et al . have mentioned that incidence of csp ranges between 1.1% and 89.7% in healthy volunteers , and from 10.0% to 89.5% in patients with schizophrenia spectrum disorder . they have also suggested that the clinical significance of csp may depend more on its size rather than presence or absence in schizophrenia . nevertheless , one recent longitudinal study has also found that csp may not be linked to the neurobiology of emerging psychotic disorders and may be related to the progression of the disorder per se . the exact function of the septum is not yet completely understood ; however it may appear to moderate behaviors such as rage and arousal . interestingly , csp is associated with aggressive features of antisocial personality disorder compared to nonaggressive ones , indicating particular relevance of septal disruption to aggression in human . researchers have also found that csp influences neighboring dense synaptic networks and cause irregularities in the distribution of serotonin ( 5-ht2a ) receptors that may affect the septal regulatory role in the limbic system . in addition , improvement of severe self - mutilation has been reported after limbic leucotomy . based on these studies , we hypothesize that limbic dysgenesis may be the reason for aggressive and self - mutilating behavior in our patient . self - mutilation has an aggressive element , wherein the person , through self - mutilation directs anger inward which enables him or her to feel in control . though several line of management have been proposed including clozapine , lithium , and naltrexone and so on , we preferred ect and chlorpromazine as there have been previous successful treatment of self - mutilating behaviors with ect and we chose chlorpromazine considering its sedating property which also reduce psychotic aggression . in our patient , self - mutilating behavior was not secondary to any psychotic process or any affective disturbance ; neither it was explainable from mental status examination . it may be argued that csp , which has been found to be associated with aggression in a different patient population , also might play a role in self - mutilating behavior in our patient with schizophrenia . we conclude that the self - mutilating behavior in our patient may be an aggressive behavior directed towards self and might be neurobiologically linked to csp and its relationship with limbic system dysgenesis .

cavum septum pellucidum ( csp ) is a neurodevelopmental anomaly , which is commonly reported in schizophrenia patients . various symptoms of schizophrenia , including thought disturbances have been associated with csp . we present a rare case of undifferentiated schizophrenia with csp who presented with self - mutilating behaviors .

he was admitted for an elective left typanomastoidectomy and removal of the incus and malleus with tympanoplasty . his medical history included hypertension , dyslipidemia , gout , polycystic kidney disease and gastroesophageal reflux disease . his medications at the time of admission were rosuvastatin , domperidone , esomeprazole , allopurinol , irbesartan / hydrochlorothiazide and labetalol . on the first day postoperatively the patient experienced a sudden drop in his level of consciousness accompanied by marked agitation , and required intubation . his glasgow coma scale score was 9 ( eyes 3 , verbal 3 , motor 3 ) . there were no focal neurological signs , his pupils were symmetrical but sluggish to react and the fundi appeared normal . a computed tomography scan of the patient s head showed no structural abnormalities , no masses and no hematoma . lumbar puncture was performed and revealed cloudy cerebrospinal fluid ( csf ) with an elevated protein level ( 5.78 g / l ) , low glucose level ( < 1.0 mmol / l ) and a leukocyte count of 11,97410/l , with 95% neutrophils . based on these findings , the patient was treated empirically for bacterial meningitis with intravenous ( iv ) vancomycin , ceftriaxone and dexamethasone pending culture results and sensitivities . further blood work revealed a blood leukocyte count of 13.810/l , hemoglobin level of 123 g / l and platelet count of 15410/l . mmol / l , potassium level 3.6 mmol / l , chloride level 106 mmol / l , urea level 12.6 mmol / l and creatinine level 190 mol / l . initial gram stain of the csf using the cytospin technique revealed abundant polymorphonuclear leukocytes and no organisms . preliminary reports revealed growth of gram - negative coccobacilli , and at this point metronidazole was also added to the treatment regime until an anaerobic cause was ruled out . ultimately , the organism was identified from aerobic cultures as p multocida using the vitek 2 identification system ( biomrieux , usa ) . interestingly , a swab of the left ear performed on postoperative day 2 grew the same organism as that cultured from the csf ( growth on chocolate and blood agar ; no growth on macconkey s or inhibitory mold agar ) . the empirical antibiotics and dexamethasone were discontinued , and the patient was started on a 14-day course of iv penicillin g at a dose of 2,000,000 units every 4 h. the patient made a rapid recovery from his meningitis , and he was discharged on postoperative day 10 to continue treatment as an outpatient . on further questioning , it was revealed that the patient was the primary caregiver of several pet cats and a dog , although he reported no history of bites . the pets were allowed on the furniture , including his bed , and would occasionally lick his face . meningitis is an uncommon outcome of p multocida infection ( 3 ) , making p multocida a rare cause of adult bacterial meningitis . two reviews spanning 1950 to 1999 report only 29 cases published in the english literature during that time period ( 4,5 ) . animal contact was a major risk factor , present in 89% of cases , and a history of a bite was much less common , occurring only 15% of the time ( 4 ) . previous cranial / facial surgery or skull fracture has been reported as a cause of p multocida meningitis ( 513 ) . table 1 summarizes adult cases of p multocida meningitis published in the english literature after 1999 ( 1322 ) . animal contact was present in all cases , while only two ( 20% ) reported a history of a bite . the current report presents one of only a handful of cases of p multocida meningitis ever documented in the literature from a canadian site ( 5,6,9,21,23 ) . the patient had the typical csf findings of bacterial meningitis ( low glucose , high protein , high leukocytes ) . penicillin is the most commonly used antibiotic to treat p multocida meningitis ( 4,15 ) , and our patient recovered fully with a course of iv penicillin g. many of the more recent cases describe treating with third generation cephalosporins ( table 1 ) . p multocida meningitis has been reported following mastoidectomy ( 11,12 ) , and the pathogenesis of infection is hypothesized to involve contiguous spread of the organism from a colonized ear canal . supporting this theory , local spread from an adjacent infected site has been proposed as an etiology ( 4 ) because chronic otitis media and otorrhea have been found in association with p multocida meningitis ( 4,19,2427 ) . our patient showed no signs of clinical meningitis preoperatively ; therefore , extension to the surgical site is the likely mechanism in this case . a preoperative ear swab has been proposed for patients having a mastoidectomy that have a history of exposure to animals ( 12 ) , and may be supported by the present case .

pasteurella multocida is a gram - negative anaerobe that is known to colonize household pets ; in fact , it has been reported to be present in a majority of cats and dogs . p multocida can cause a variety of infections in humans , of which skin infections are the most common . this article describes a case involving a 56-year - old man who developed meningitis caused by p multocida following tympanomastoidectomy . the authors discuss the treatment course and most likely route of infection in this case , and summarize the cases of p multocida - associated meningitis presented in the literature .

epithelioid hemangioendothelioma ( ehe ) is a rare tumor of the vascular endothelium that is considered to be intermediate between hemangioma and angiosarcoma . typically , the soft tissue is involved and , less frequently , the skin , bone , liver and lung are involved . in this report , we describe a case of cutaneous ehe that was present since birth involving the whole of the right lower limb . a 16-year - old female presented with painful progressive swelling and erythematous skin lesions involving the whole of her right lower limb . she had lesions since birth and also had restricted movements but with no other systemic complaints . an examination revealed diffuse swelling of the right lower limb , including the vulva and the lower abdomen . there were multiple erythematous papules , nodules and plaques of varying size that were firm in consistency [ figure 1 ] . diffuse swelling of the whole of the right limb with multiple erythematous papules , nodules and plaques on subjecting the patient for investigations , her bleeding time , clotting time , prothrombin time and complete blood count were normal . x - ray [ figure 2 ] and computed tomography scan revealed soft tissue swelling with diffuse osteolysis of the femur , tibia and fibula . the other differential diagnosis was klippel trenaunay syndrome , but due to the presence of osteoporotic changes in the bone , this was excluded . x - ray showing diffuse osteolysis of the right lower limb bones compared with the normal left limb biopsy of the erythematous nodule showed nodular tumor in the dermis , which was composed of oval- to spindle - shaped epithelioid cells . the nuclei showed a coarse chromatin pattern , and occasional mitotic figures were also noted . the cells were strongly immunoreactive for cd34 [ figure 4 ] and factor viii - related antigen . finally , a diagnosis of ehe was made and the patient was referred to the vascular surgery department for further management . hematoxylin and eosin showing multiple vascular spaces with red blood cells ( 40 ) cd 34 antigen positivity ( 40 ) ehe has also been reported in the liver , bone , gingiva , mediastinum and lung . ehe is most commonly located on the extremities , and tumor is evenly distributed through all the adult age groups . the neoplasm usually presents as solitary , rarely multiple , slightly painful erythematous papules , nodules , plaque and nonhealing ulcer . microscopic examination reveals aggregates of oval to polygonal cells with abundant eosinophilic cytoplasm and round nucleus . the nuclear chromatin pattern is vesicular and frequent prominent nucleoli are present . the more commonly used antisera are factor viii - related antigen , cd 31 , cd34 and ulex europeus lectin . our case is unusual because of its presence since birth and because of its location in the dermis and involving the underlying bone with diffuse osteolysis . a case of cutaneous ehe with underlying bone involvement was described by malane , sau , benson in 1992 . the prognosis of ehe is uncertain as the mortality rate for ehe of the liver is 35% and lung is 65% . treatment should be limited to simple surgical excision , provided the possibility of an underlying bone lesion has been excluded .

epithelioid hemangioendothelioma is an intermediate - grade vascular tumor arising from the vascular endothelium , which usually arises in soft tissue , and skin involvement is extremely rare . we report a case that presented with primary cutaneous tumor involving the whole limb and was present since birth .

partial cholecystectomy , whether open ( 1,2 ) or laparoscopic ( 3 ) , has been described as a safe and effective operation . it is indicated in cases of severe acute cholecystitis , gangrenous cholecystitis or necrotizing cholecystitis , where the degree of inflammation precludes safe visualization of biliary structures . partial cholecystectomy removes the majority of the gallbladder , leaving a portion of the neck , and occasionally the posterior wall of the gallbladder , in place . ( 4 ) it is reportedly tolerated well , with few minor postoperative complications , however the issues that do occur include recurrent symptoms , choledocholithiasis and persistent biliary fistula . ( 5 ) historical accounts have documented partial cholecystectomy performed in the 1950s with washout and removal of gallbladder contents with placement of a drain in the orifice of the cystic duct when dissection of the hilar structures would be too dangerous , or the gallbladder was densely adherent to the liver bed . ( 6 ) it should be noted that even though technically possible , partial cholecystectomy is less than the ideal procedure , which is a complete cholecystectomy , with total removal of the gallbladder and closure of the cystic duct remnant . partial cholecystectomy may not provide a cure in all patients because leaving a portion of the gallbladder in situ in connection to the common bile duct may contribute not only to the formation of new stones , but also leaves a path intact for continued biliary colic and obstruction . we review a case in which partial cholecystectomy was performed due to severe inflammation in a young otherwise healthy patient . a 22 year - old male presented with a history of several months of nausea , vomiting , right upper quadrant abdominal pain and in the last two weeks , jaundice . seven months prior , he had undergone a partial cholecystectomy at an outside institution for severe acute cholecystitis . the case was attempted laparoscopically , however due to lack of visualization , it was converted to an open approach . the gallbladder was dissected from the fundus in a retrograde fashion , but extended far posteriorly , and was noted to be beyond surgical reach , secondary to the acute inflammation . a partial cholecystectomy was done with a surgical ta stapler across the infundibulum leaving approximately 25% of the gallbladder , including the entire cystic duct . mrcp shows the residual gallbladder ( x ) , and the dilated common bile duct with stones ( white arrows ) over seven months , he had continued frequent recurrent attacks of acute cholecystitis , and choledocholithiasis . his symptoms included frequent nausea , vomiting , persistent sharp right upper quadrant pain , fever , and jaundice , which precipitated multiple emergency department visits to multiple institutions . the patient presented to our institution with an outside ultrasound that demonstrated multiple residual stones , a sonographic murphy s sign , and dilated common bile duct of 9 mm . . 1 ) and gallstones were visualized in the remaining portion of the gallbladder by ct , ( fig . preoperatively , an ercp with sphincterotomy was performed to extract the remaining common bile duct stones ( fig . ct of the abdomen demonstrates the residual portion of the gallbladder , marked by an the staple line , indicated by a black arrow , is seen best on the coronal imaging at operation , the previous transverse right upper quadrant incision was reentered , and dissection continued down to the liver . the previously stapled remnant of gallbladder was identified as partially intrahepatic , and located at the most inferior portion of the liver . the cystic duct and artery were then each ligated and a completion cholecystectomy was performed . due to the still severe inflammation , the estimated blood loss was 1800 cc . on pathology , the patient tolerated the surgery well and was discharged home the following day , and made a full recovery with no further symptoms . ercp shows a residual cystic duct ( black arrow ) , and choledocholithiasis ( white arrows ) in patients with acute cholecystitis , partial cholecystectomy can be performed , when the severity of inflammation precludes safe dissection of the hilar structures . depending on the extent of the resection and the structures left in situ , there is a definite risk of recurrence of symptoms , including recurrent acute cholecystitis ( 7 ) , gallstone pancreatitis ( 8) and choledocholithiasis . the morbidity of recurrent gallbladder issues will require further endoscopic and/or surgical interventions in at least some patients , as the underlying pathophysiologic process responsible for the acute cholecystitis has not been definitively managed . all efforts should be made to remove remaining gallstones from the remaining portion of the gallbladder ; as this was not done in our patient it explains the lack of recovery from the partial cholecystectomy . analogous to a cholecystostomy tube placement , partial cholecystectomy should therefore be considered another option for temporizing the patient experiencing severe acute cholecystitis . however , there needs to be an awareness that the patient will need to be considered for definitive surgical management with a completion cholecystectomy . in our case , this was done via an open approach due to the adhesions and the posterior position of the remnant gallbladder , but in more favorable cases it has been performed laparoscopically .

partial cholecystectomy has been documented in the literature as a safe alternative in the management of patients with acute cholecystitis when the degree of inflammation prevents a safe dissection to identify the biliary structures for complete removal of the gallbladder . partial cholecystectomy however is not without risk of recurrence , and the need for further surgical or endoscopic intervention in management of complications . we review a case in which partial cholecystectomy was performed without any relief of symptoms , to review the possible postoperative complications , and caution that these patients will need to be considered for a completion cholecystectomy .

a 34-year - old female presented with history of injury to the left eye while viewing bursting firecrackers during a local festival . on initial evaluation , the patient was noted to be alert and oriented , and to have an isolated injury to the left orbit with normal neurologic examination . local examination showed a metallic bar embedded in her left orbit with a part of it protruding out through her upper eyelid [ fig . anterior segment examination was unremarkable except a relative afferent pupillary defect in the left eye . ( a ) clinical photograph showing metallic foreign body protruding through left upper eyelid , ( b ) computed tomography scan of the orbits showing the l- shaped metallic foreign body , ( c ) computed tomography scan three - dimensional reconstruction showing spike - like protrusions along the foreign body ( streak artifacts ) , ( d ) foreign body measurement after removal fundus examination of the left eye revealed blurred optic disc margins , disc hemorrhage , multiple choroidal folds , and retinal hemorrhages at the posterior pole [ fig . foreign body indentation was present nasal to the optic disc which became more prominent with adduction . a localized retinal detachment of 34 disc diameters size with subretinal hemorrhage was present adjacent to it [ fig . 2b ] , but no retinal break was noted , which indicated the possibility of posterior scleral injury by the sharp end of the foreign body and self - sealing of the wound subsequently . ( a ) fundus photograph showing indistinct optic disc margins , retinal hemorrhages , and choroidal folds at posterior pole , ( b ) foreign body indentation with subretinal hemorrhage and localized retinal detachment in the nasal retina , ( c ) postoperative resolution of disc edema , hemorrhages , and choroidal folds , ( d ) postoperative disappearance of foreign body indentation and resolution of retinal detachment , with persistent subretinal hemorrhage computed tomography ( ct ) scan of the orbits [ fig . l- shaped metallic foreign body passing through left upper eyelid , coursing through medial rectus , bending laterally , indenting the sclera , passing superior to the optic nerve compressing it downward . extraorbital part measured 8 mm , vertical limb of intraorbital part was 27 mm , and posterior limb was 20 mm . three - dimensional reconstructed ct image showed spike - like protrusions along the foreign body [ fig . there was decreased amplitude and increased latency on visually evoked response in the left eye . under general anesthesia , surgical removal of the foreign body laser delimitation around the retinal detachment was done . on examination of the removed foreign body [ fig . 1d ] , no sharp protrusions were seen and the imaging findings were considered as streak artifacts . she was advised oral antibiotics and oral steroids , with topical antibiotic - steroid drops for 2 weeks . the patient recovered well with restoration of full ocular movements and improvement in optic nerve function . at 2-week follow - up , retinal hemorrhages cleared [ fig . 2c ] and retinal detachment was well delimited and settled with absorption of subretinal fluid though some subretinal hemorrhage persisted [ fig . metallic intraorbital foreign bodies are known to be well tolerated and associated with minimal adverse visual prognosis when their path does not traverse through the globe and globe integrity is maintained . however , there have been several reports of orbital foreign bodies with potential serious eye injuries [ table 1 ] . clinical characteristics and outcome of various intraorbital foreign bodies in this case , on initial evaluation , the foreign body did not appear to cause any harm to the eyeball as it was embedded in upper lid with its sharp protruding end away from the globe and there were no signs of trauma in anterior segment . the relative afferent pupillary defect alerted that the optic nerve was compromised , either due to direct injury or through orbital compartment syndrome secondary to hemorrhage and/or edema . posterior segment examination provided an important clue that the foreign body passed through the medial orbit to cause injury from behind the globe . we emphasize the need for evaluation of the posterior segment and optic nerve function in every case of intraorbital foreign body . ct scanning assists in the proper localization of the foreign body , estimation of its consistency , size , path , and the relation to the adjacent orbital tissue . in this case , the unusual path of the foreign body was well delineated by ct scan , which supported the clinical findings of optic nerve compression and globe indentation by the posterior part of foreign body , and helped in planning surgical removal . however , streak artifacts should be differentiated from any protrusion of the linear metallic foreign body . surgical intervention in a case with intraorbital foreign body is usually indicated in the presence of a sharp foreign body , signs of infection , proptosis , restricted motility , palpable orbital mass , optic nerve compression , abscess , suspicion of organic material , fistula formation , or when adjacent structures are compromised . in our case , indications for surgical intervention were direct optic nerve compression by the foreign body , risk of infection along the foreign body tract , restricted ocular motility , and foreign body compressing the globe on posterior surface . our experience with this case shows that curved metallic orbital foreign bodies with benign appearance may be associated with sight - threatening retinal injuries . although foreign bodies with protruding end can simply be removed along their tract , one should be aware of an unusual intraorbital course . in our case , thus , we advocate early surgical exploration and guarded extraction of the foreign body to improve the visual prognosis and outcome in such cases .

a 34-year - old female presented with firecracker injury with curved metallic foreign body embedded in the left orbit and protruding out through the upper eyelid . the report highlights notable aspects in diagnosis , decision - making , and successful removal of this unusual case of retro - orbital foreign body .

nivolumab is an anti - programmed death 1 ( pd-1 ) monoclonal antibody and has been reported to be useful for treatment of cancers including malignant melanoma . a recent case report documenting fatal nivolumab - related myasthenia gravis ( mg ) has caused concern about the risk of increased toxicity in patients with pre - existing mg who receive nivolumab for the management of metastatic melanoma . it is unclear what will occur if a patient with pre - existing mg receives nivolumab . a 79-year - old man began to receive immune checkpoint therapy with nivolumab at a dose of 2 mg / kg every 3 weeks for the management of submandibular lymph node metastasis from malignant melanoma , which arose in his forehead 3 years ago and was previously treated with cytotoxic chemotherapy . the patient had a > 20-year history of ocular mg according to osserman s classification . durable remission was maintained by treatment with oral corticosteroids ( 3 mg every other day ) . a routine serum chemical test on day 43 , after the patient had received 2 doses of nivolumab , revealed a grade 3 elevation of the creatine phosphokinase ( cpk ) level to 1,627 iu / l ( institutional normal range , 62 to 287 iu / l ) ( fig . the patient did not experience muscle pain , muscle weakness , or any other symptoms . the cpk level decreased to 469 iu / l on day 100 and was at worst grade 1 subsequently . however , after the third dose of nivolumab , the patient had diplopia , facial muscle weakness , and difficulty in drinking water on day 106 . positive results of an acetylcholine receptor antibody test ( 20.0 nmol / l , normal range , 0.2 nmol / l ) supported the diagnosis . . apparent shrinkage of the metastatic lymph nodes was observed on computed tomographic imaging after the patient had received 10 doses of nivolumab ( fig . fdg - pet / ct before and after administration of nivolumab uptake of lymph node metastases decreased after ten doses of nivolumab . anti - pd-1 antibodies enhance immune response to tumor cells and to normal host tissues as well , and cause adverse effects related to the autoimmune diseases . other immune checkpoint inhibitors , anti - programmed death - ligand 1 ( pd - l1 ) antibodies and anti - cytotoxic t - lymphocyte - associated protein 4 ( ctla4 ) antibodies , also have been reported to cause immune - related diseases , such as myositis and myasthenia gravis . the present case with pre - existing mg showed cpk elevation , and symptoms suggesting deterioration of mg such as diplopia and facial muscle weakness after nivolumab administration . after suspending nivolumab , cpk decreased and symptoms improved , and nivolumab was able to be administered safely . since cpk elevation is not usually observed in mg patients , we could not exclude the possibility the symptoms were caused by myositis but not by mg . although it is not easy to distinguish symptoms of mg from those of myositis , the difference in the time of onset of cpk elevation and that of symptom exacerbation tends to indicate that the symptoms derived from mg rather than myositis . in a case previously reported , an acetylcholine receptor antibody test was low - titer positive ( 2.9 nmol / l ) before the onset of mg induced by nivolumab . nmol / l one year and seven months prior to the beginning of nivolumab treatment , and it increased to 20 nmol / l when mg was exacerbated . the antibody titer is considered to be useful to predict the onset of mg and to observe mg symptoms . there are no clear criteria to decide whether nivolumab is allowed to be restarted after discontinuation of the administration due to adverse events such as cpk elevation or mg exacerbation . we consider that the restart with careful observation might be allowed when the adverse events improved to mild symptoms , for instance , nci - ctcae grade 1 . despite the transient exacerbation of mg - associated symptoms , this patient - tolerated nivolumab therapy went well and provided substantial benefit , suggesting that the drug is not necessarily contraindicated in patients with preexisting mg . nivolumab - related mg might have a distinct etiological background from that of primary mg . yuichi ando received honoraria from ono pharmaceutical co. , ltd . , and has held a consultant / advisory role with ono pharmaceutical co. , ltd ..

abstracta 79-year - old man with lymph node recurrence of malignant melanoma received nivolumab , an anti - programmed death 1 ( pd-1 ) monoclonal antibody . he had pre - existing ocular myasthenia gravis ( mg ) and a continued small amount of corticosteroid . grade 3 creatine phosphokinase elevation appeared after two doses of nivolumab , and the treatment was postponed until it improved to grade 1 . after three doses of nivolumab , he experienced diplopia and facial muscle weakness which were consistent with an acute exacerbation of mg , and the symptoms relieved without additional treatment for mg . he achieved shrinkage of metastasis after ten doses of nivolumab . although a case who died due to mg after administration of nivolumab was reported recently , pre - existing mg is considered not to be always a contraindication of nivolumab .

. however , focal abnormalities on electroencephalography ( eeg ) have also been observed in patients with generalized epilepsy . in such cases , it is supposed that the cortical focus drives widespread epileptic networks that results in generalized seizures . , we describe a patient who had eyelid myoclonia with absences , along with focal motor seizures . ictal video - eeg recording showed generalized spike waves preceded by focal frontal discharges , suggesting that focal cortical abnormalities may induce eyelid myoclonia with absences . the patient is a 7-year - old boy who exhibited eyelid myoclonia with absence seizures . since the age of 5 years , he complained of involuntary movements of the eyelids , which occurred a few times per week . a physician diagnosed this as a tic disorder . however , his parents and brother noticed brief episodes of unresponsiveness , often associated with jerks of the eyelids , lasting 35 s. these episodes gradually increased in frequency , occurring many times a day . eyelid movement appeared like rapid blinking and was often accompanied with upward deviation of the eyeballs and retropulsion of the head . 1 ) . further , eeg recording during the interictal period showed normal background activity and focal epileptiform discharges over the frontal regions ( fig . however , he experienced a focal motor seizure with impairment of consciousness and a generalized tonic seizure during sleep in the next 2 years . the focal seizures consisted of clonic movements of the right upper limb and upward deviation of the eyeballs , lasting less than a minute . the onset of the generalized seizure was not detected . when the patient started to have focal seizures , he underwent mri scanning of the brain , the result of which showed no abnormality . we describe the case of a patient with eyelid myoclonia with absences who had been misdiagnosed with facial tic disorder . our patient had eyelid myoclonia that appeared like rapid blinking and was often associated with jerky upward deviation of the eyeballs and retropulsion of the head . the absences were brief , lasting 35 s. ictal eeg revealed 4- to 5-hz generalized polyspike waves . these electroclinical features were in contrast to those of a typical absence seizure in childhood absence epilepsy wherein the eyes look straight ahead , and the absence seizures are associated with 3-hz spike - and - wave discharges over a longer duration . eyelid myoclonia has been well recognized as the clinical feature of seizures in individuals with generalized epilepsy . jeavons syndrome , a form of generalized epilepsy , is clinically characterized by the triad of eyelid myoclonia with or without absences , eye closure - induced seizures or eeg paroxysms , and photosensitivity . electroencephalographic manifestations have been described as focal frontal or occipital predominant epileptiform discharges followed by generalized epileptiform discharges in both interictal and ictal recordings . thus , it has been suggested that either the frontal or the occipital lobe initiates eyelid myoclonia . a recent cortical stimulation study has demonstrated that vertical upward eye movements can be induced by electrical stimulation of the frontal eye field ( fef ) , which is located in the frontal cortex . upward deviation of the eyeballs occured with frontal epileptiform activity in a patient with epilepsy . a subgroup of children with absence epilepsy showed an eeg epileptic abnormality with frontal onset of the typical generalized 3-hz spike - and - wave discharges . dense - array eeg , a technique that improves the spatial resolution of scalp eeg , has demonstrated that selective cortical networks in the frontal lobe are involved in the propagation of ictal discharges in absence seizures . further , microarchitectural abnormalities in the frontal lobe have also been reported as potential facilitators of seizures in juvenile myoclonic epilepsy , another generalized epilepsy . in generalized epilepsy , many brain regions have a tendency to reach an epileptogenicity level that exceeds the seizure threshold . thus , generalized seizures may start from focal brain regions and tend to generalize very fast . eyelid myoclonia with absences may represent a type of secondarily generalized seizure originating in the frontal lobe . in conclusion , our case suggests an important role of the frontal lobe in the generation of eyelid myoclonia with absences . eyelid myoclonia with absences is a unique type of absence seizure associated with a variety of epilepsy syndromes . our case supports the concept that there is no absolute distinction between generalized and focal epilepsy but rather a continuum .

eyelid myoclonia with absences is classified as a unique type of generalized seizure . its pathogenesis is proposed to involve the functional abnormalities in cortical subcortical networks . here , we describe the case of a 7-year - old boy who had eyelid myoclonia with absences , along with focal motor seizures . video - eeg monitoring demonstrated eyelid myoclonia associated with 4- to 5-hz generalized polyspike waves preceded by focal frontal discharges . interictal eeg showed focal epileptiform discharges over the frontal regions . our case suggests an important role of the frontal lobe in the generation of eyelid myoclonia with absences .

the double duct sign is a simultaneous dilatation of both the pancreatic duct ( pd ) and the common bile duct ( cbd ) and is usually a result of narrowing of terminal parts of both the ducts due to various causes including encasement of the cbd and main pd by a pancreatic head tumour , ampullary carcinoma , pancreatitis and periampullary stenosis . we report a 54-year - old male who came with abdominal pain and was found to have an unusual etiology of double duct sign . a 54-year - old man presented to us with right upper abdominal pain of 4 months duration . the pain was episodic , severe and used to last around h and occurred in relation to meals . there was no associated vomiting , jaundice , fever , loss of weight or appetite . he had a history of chronic alcohol abuse as well as oral opium ingestion for last 25 years . he used to daily consume oral powdered form of opium husk ( vernacular bhukki ) . blood tests revealed a serum bilirubin of 1.0 mg / dl ( normal , 0.2 - 1.4 mg / dl ) , aspartate aminotransferase of 47 u / l ( normal 1 - 35 u / l ) , alanine aminotransferase of 41 u / l ( normal , 1 - 35 u / l ) and alkaline phosphatase 156 u / l ( normal , 20 - 128 u / l ) . abdominal ultrasound revealed a dilated anechoic cbd with mild central intra - hepatic biliary radical dilatation and dilated pd . contrast enhanced computed tomography of the abdomen showed a double duct sign with cbd dilated until lower end and a dilated pd [ figure 1 ] . endoscopic ultrasound ( eus ) using a linear echoendoscope ( gf - uct 180 linear echoendoscope , olympus corp , tokyo ) revealed simultaneous dilatation of the cbd and pd ( double duct sign ) with anechoic lumens of both the ducts . the ampullary region revealed a prominent hypoechoic layer suggestive of muscular hypertrophy [ figure 3 ] . a diagnosis of opium - induced sphincter of oddi dysfunction ( sod ) was made , and patient underwent biliary sphincterotomy with improvement in his symptoms and normalization of his liver enzymes . post sphincterotomy spy glass cholangioscopy ( boston scientific corp , natick , mass ) was also done for evaluation of the bile duct mucosa at the terminal end and it revealed normal mucosa [ figure 4 ] . he was referred for de - addiction and was asymptomatic after 3 months follow - up . abdominal computed tomography showing dilated common bile duct ( arrow ) side viewing endoscopy revealing a bulky papilla endoscopic ultrasound showing dilated common bile duct and pancreatic duct with hypoechoic prominence at lower end ( arrow ) spy glass done for evaluation of common bile duct . sphincter of oddi dysfunction is a benign , non - calculous obstruction at the major duodenal papilla that can produce variable clinical symptoms including pancreaticobiliary pain , cholestasis , and/or pancreatitis . opiates produce multiple pharmacologic effects in the gastrointestinal tract with constipation being the most common . constant prolonged use of this drug might be responsible for the contraction and permanent dysfunction of the sphincter of oddi and sod due to chronic opium use is now recognized as a distinct clinical entity . clinical features of this entity are variable and may consist of anorexia , weight loss , constipation and abdominal tenderness . the endoscopic visualization of the second part of the duodenum may reveal a prominent papilla , but even ulcerative and tumourous changes are common in opium related sod and therefore it is important to carefully exclude the malignancy . eus is an important investigation for excluding the malignancy as even small lesions in the ampullary area can be detected by a carefully performed eus . eus can also help in the detection of hypertrophy of the muscular sphincter of oddi as was seen in the index case . the response to biliary sphincterotomy in such cases is excellent as was in the present case . the case is reported because opium abuse is an uncommon cause of sod and the history of opium abuse must be sought in patients with unexplained biliary dilatation or dual duct dilatation and residing in areas where opium abuse is common . furthermore , eus provides an excellent modality to rule out small malignant lesions resulting in double duct sign .

double duct sign where there is a simultaneous dilatation of both the common bile duct ( cbd ) and pancreatic duct is usually associated with sinister causes like malignancies of pancreatic head or ampulla . occasionally , benign causes like chronic pancreatitis or sphincter of oddi dysfunction ( sod ) may cause double duct sign . chronic opium abuse is a rare cause of the double duct sign , and endoscopic ultrasound ( eus ) findings of this rare entity have been occasionally reported . we report about a 54-year - old male with a history of chronic alcohol and opioid abuse evaluated for episodes of abdominal pain and found to have opioid - related sod and improved with biliary sphincterotomy . eus was done to rule out malignancy and revealed hypoechoic prominence around terminal cbd suggestive of hypertrophied muscle .

pilonidal sinus is a well recognized chronic inflammatory condition which affects the natal cleft , finger web space , and it is rare in the penis . we report on a case of penile pilonidal sinus in a 24-year - old man who has severe mental illness , autism , and developmental delay . the literature was reviewed and there were a few cases reported worldwide ( val - bernal et al 1999 ; chikkamuniyappa et al 2004 ; kalsi et al 2004 ; okane et al 2004 ) . a 24-year - old man who is known to have severe mental illness , autism , and a learning disability , presented with more than a one year history of recurrent ulcerative lesion on the middle of the dorsum of his penile shaft , intermittent erythema , and swelling . despite several courses of antibiotics prescribed by his local general practitioner , the lesion did not resolve and the swelling was observed to grow bigger between these episodes . clinical examination revealed an indurated , erythematous 89 mm ulcerative lesion in the middle of the dorsal aspect of the penile shaft . the patient had not been circumcised and had never reported a similar complaint from anywhere else in his body . excisional biopsy was performed , and histology showed the classical findings of pilonidal sinus with hair shaft within . it is essential to highlight the fact that diagnosis was made post - operatively based on histopathological findings rather than on preoperative clinical assessment . in some cases diagnosis pilonidal sinus was first described by anderson ( 1847 ) . it is a condition that affects hairy areas such as the natal cleft , axillae , perineum , finger web space in the hands of barbers , and amputation stumps ( saharay et al 1997 ) . the pathogenesis of pilonidal sinus is controversial although it is agreed that it is acquired . the present view is that the large majority of pilonidal sinuses have an acquired pathogenesis . the initiating event appears to be follicular hyperkeratosis with plugging , leading to retention of follicular products ( lucas 1997 ) . the rupture of a follicular unit or a cyst with secondary infection results in abscess formation . in some instances , sinuses formed to drain the abundant suppuration . the sinuses are lined by stratified squamous epithelium and inflammatory granulation tissue . very often , numerous hair shafts penetrate into dermis and elicit foreign body giant cell reaction ( figure 13 ) . the support for the acquired theory of the pilonidal sinus comes from their occurrence in situations such as the finger web space in the hands of barbers , and in amputation stumps . therefore many cases are acquired as a result of trauma from jeep seat , rough riding syndrome , and occupational physical injury ( val - bernal et al 1999 ) . the literature was reviewed and a few cases were reported worldwide ( val - bernal et al 1999 ; chikkamuniyappa et al 2004 ; kalsi et al 2004 ; okane et al 2004 ) . the literature referred to penile pilonidal sinus with some other pathologies such as actinomycosis infection ( val - bernal et al 1999 ; chikkamuniyappa et al 2004 ) or erectile dysfunction ( kalsi et al 2004 ) . therefore , a good clinical assessment and bacteriological examination is essential to obtain better results . in our case , we reported on a pilonidal sinus where diagnosis was made on histopathological findings with no evidence of associated bacterial infection or malignancy ( rao et al 2006 ) . our patient had full recovery and no symptoms since , his post - operative follow - up involved two out - patient visits at up to 12 weeks post - operation . the patient had no symptoms or recurrence up to that time , interval circumcision was deferred due to the patient s general health status and his mental disability .

pilonidal sinus is a well recognised chronic inflammatory condition which commonly affects the natal cleft and finger web space . pilonidal sinus of the penis is a rare clinical entity ; few cases have been reported previously . the treatment strategy is almost the same as treating any pilonidal sinus and involves total excision of the sinus . our diagnosis was established on the histopathological findings rather than a clinical - based diagnosis , follow - up is advisable since the recurrence rate is high . our case report is one of few cases reported on pilonidal sinus all over the world . our patient had total excision of the sinus with complete recovery .

dacron arterial grafts are widely used in thoracic aortic replacement , but few cases of non - anastomotic aneurysmal dilatation or rupture have been reported . in most cases that have been reported , knitted , rather than woven , we report the clinically successful result of thoracic endovascular repair ( tevar ) of the late rupture of a woven dacron graft . the dacron graft had been implanted in the patient s descending thoracic and upper abdominal aorta 20 years previously . a 56-year - old man with a descending thoracoabdominal aortic aneurysm underwent descending thoracic and upper abdominal aorta replacement with a hemashield 20-mm woven dacron graft ( meadox medicals inc . , he was followed up as an outpatient , and no adverse events were noted until 2009 , when he presented with sudden - onset chest pain and severe dyspnea . at that time , chest computed tomography ( ct ) revealed a massive right hemothorax , a large pseudoaneurysm around the implanted graft just above the diaphragm level , and marked contrast medium extravasation from the graft ( fig . 1 ) . the patient underwent an emergency open thoracotomy , in which a disruption was found at the site of the anastomosis between the dacron graft and lumbar intercostal arteries . the disrupted site was repaired with 2 layers of running sutures , and the graft was wrapped with the calcified aneurysmal sac . in 2012 , the patient presented with back pain , abdominal pain , and indigestion . a follow - up chest ct revealed that the size of the aneurysm in the descending aorta had increased in comparison with the findings of the previous ct examination . dye collection in the space between the graft and native aneurysmal sac was also observed , and was suspected to be indicative of a late rupture of the dacron graft itself ( fig . the rupture was unrelated to the previous anastomosis or disrupted site . due to the high morbidity and mortality associated with reoperation , we decided to perform thoracic endovascular stent therapy as an alternative to open thoracotomy for the late graft failure . the primary goal of using tevar was to cover the entire length of the dacron graft in the descending aorta with a stent graft . the right common femoral artery was exposed under general anesthesia , and a guidewire and angiographic catheter were introduced through it . aortic angiography revealed multiple dye leakages from the graft that were caused by late failure of the dacron graft ( fig . 3 ) . after obtaining an aortogram , two stent grafts ( 3030150 mm and 3636100 mm ; s&g biotech inc . , seongnam , korea ) were also introduced through the right femoral artery and placed in the dacron graft . the proximal end of the stent graft was deployed distally to the left subclavian artery , and the distal end was placed just above the celiac trunk . the total length of the thoracoabdominal aorta covered by the stent graft was approximately 220 mm . a postoperative ct scan showed that the disruption of the dacron graft had been successfully treated ( fig . the patient was discharged with no procedure - related complications and , during the 1-year follow - up period , was free of any symptoms . dacron vascular grafts are known to be durable and effective as aortic vascular prostheses . however , a few cases of non - anastomotic aneurysmal dilatation or rupture of these grafts after implantation in the descending thoracic aorta have been reported . the probable cause was degeneration over time of the graft fibers or the round black yarn used as the guideline . intrinsic graft failure of dacron grafts is a rare complication ( 0.5%3% ) ; and in most reported cases , knitted , not woven , dacron grafts had been used . in our case , woven dacron graft rupture occurred 20 years after the initial operation , probably caused by the degeneration of graft fibers over a long time . although woven dacron graft rupture is extremely rare , reoperation is inevitable when it does occur . however , the hospital mortality after aortic reoperation remains high ( 8%15% in many reports ) , and is even higher ( 25%50% ) among patients receiving urgent or emergency care . tevar is increasingly being used for descending aortic diseases as a safe and feasible alternative to open graft repair . in light of the high morbidity and mortality associated with reoperation , we decided to perform endovascular therapy as an alternative management strategy for late graft failure in our patient . to the best of our knowledge , the endovascular repair of primary graft rupture in a woven dacron graft has not been reported . the case presented here demonstrates the effectiveness and feasibility of tevar implantation to treat the non - anastomotic rupture of a dacron graft after descending aortic replacement .

the intrinsic structural failure of a dacron graft resulting from the loss of structural integrity of the graft fabric can cause late graft complications . late non - anastomotic rupture has traditionally been treated surgically via open thoracotomy . we report a case of the successful use of thoracic endovascular repair to treat a dacron graft rupture in the descending aorta . the rupture occurred 20 years after the graft had been placed . two stent grafts were placed at the proximal portion of the surgical graft , covering almost its entire length .

nppe is an uncommon complication of extubation in the early post - operative period and most commonly caused by laryngospasm . acute laryngeal spasm results in airway obstruction and can cause life threatening pulmonary edema due to negative intra thoracic pressure . the incidence of nppe has been reported to be 0.05 to 0.1% of all anesthesiology practices however it may be underreported due to failure of recognizing or misdiagnosing . a 37-year - old male patient was referred to the emergency department due to developing of severe chest pain , hypotension , and dyspnea following septoplasty operation . on admission , physical examination revealed blood pressure of 70/40 mmhg , heart rate of 75/min , bilateral diffuse crackles at the lung and wheezy breathing . oxygen saturation was 60 - 70% while breathing high - flow oxygen through a face mask . twelve - lead resting electrocardiography ( ecg ) revealed deep and down - sloping st - segment depressions of 3 mm in the precordial leads [ figure 1 ] . due to elevated cardiac biomarkers on admission ( troponin i level : 2,1 ng / dl and creatine kinase myocardial band ( ck - mb ) level : 7,4 mg / dl ) he immediately underwent cardiac catheterization with the diagnosis of non - st elevation myocardial infarction , acute pulmonary edema and cardiogenic shock . twelve - lead resting electrocardiography showing deep and down - sloping st segment depressions in the precordial leads bedside transthoracic echocardiography showed . ejection fraction was normal , estimated pulmonary arterial pressure was not elevated and there was no evidence of thrombus in the main pulmonary artery . there was only increase in diffuse density of the lungs supporting congestion : [ figure 2 ] . thoracic computerized tomography angiography demonstrating increase in diffuse density of the lungs supporting acute diagnosis acute pulmonary edema after coronary angiography , the patient was followed in the intensive care unit . during the follow - up , his oxygen saturation and blood pressure levels were increased , chest pain , and dyspnea were improved with high - flow oxygen , inotropic support , and diuretics . based on these laboratory parameters , imaging results and clinical course , the patient was diagnosed as hypoxia - induced acute subendocardial myocardial infarction , which is an unexpected result of negative pressure pulmonary edema . nppe is one of the causes of non - cardiogenic pulmonary edema which occurs with increased inspiration effort and upper respiratory tract obstruction following upper respiratory , oropharyngeal , and nasal surgeries . nppe , usually occurs during emergence from general anesthesia in the early post - operative period especially after upper respiratory tract surgery and may less frequently occur in various clinical situations ( electroconvulsive therapy , etc . ) . upper respiratory tract obstruction caused by laryngospasm which occurs especially after extubation has been suggested as the most common cause of the nppe . increased inspiration effort caused by upper respiratory tract obstruction elevates the negative intrathoracic pressure and this condition progresses to the nppe . there are some investigations which reports healthy , young , and well - built adults are more prone to nppe because of their ability to further increase the negative intrathoracic pressure . an entirely different population carries high nppe risk which consist of the patients who are obese and patients who have obstructive sleep apnea syndrome , mediastinal mass , and upper respiratory tract obstruction . depending on the clinical circumstance , if diagnosed early , many patients do not need aggressive therapies but some patients will need invasive or non - invasive mechanical ventilation . patients usually respond to well fluid restriction , diuretics and corticosteroids . to the best of our knowledge , the diagnosis of nppe was missed in the early post - operative period , and therefore , the clinical status of the patient deteriorated with time . the complains of chest pain and dyspnea were misdiagnosed as acute coronary syndrome , acute pulmonary edema and hypotensive shock in the emergency service . in conclusion , other than anesthesiologists , the cardiologists , pulmonologists , and emergency physicians must be aware of the nppe , a diagnosis of exclusion , in the differential diagnosis of acute pulmonary edema .

negative pressure pulmonary edema ( nppe ) is defined as fluid transudation into the pulmonary interstitium which occurs as a result of elevated negative intrathoracic pressure caused by the upper respiratory tract obstruction and strong inspiratory effort . nppe is usually seen during emergence from general anesthesia in the early post - operative period especially after upper respiratory tract surgery . we present a case of a 37-year - old male patient who underwent septoplasty operation and developed nppe which could not diagnosed and progressed to acute subendocardial myocardial infarction .

tufted angioma is a rare vascular neoplasm clinically characterized by slowly progressive pink to red macules or plaques on the neck , trunk , or shoulders of children or young adults . these tumors follow a benign course of angiomatous proliferation , without any evidence of malignancy . we report herein a case of multifocal tufted angiomas with annular morphology in a child for the rarity of the condition . an 8-year - old boy was referred to us for the evaluation of multiple reddish elevations on the back , which were first noticed 4 years before presentation . starting as two small reddish asymptomatic patches , the lesions gradually became elevated and fresh lesions appeared in the vicinity of the older ones . the lesions have remained stable for the preceding several months . increased sweating over the patches was noted by the parents . family history and past medical history was unremarkable . on examination , two brightly erythematous annular plaques of about 6 cm and 3 cm diameter in addition , several erythematous papules and plaques of varying sizes were seen around the lesions as well as on the right side of the back . routine laboratory tests , blood coagulation profile , and serum biochemistry panel were within normal limits . two annular erythematous plaques and several papules and plaques of varying sizes on the back we considered the differential diagnoses of tufted angioma , sudoriferous angioma ( eccrine angiomatous hamartoma ) , and capillary hemangioma . crescent - shaped cleft like spaces were seen around the lobules [ figures 2 and 3 ] . based on the clinical findings and characteristic histology , a diagnosis of tufted angioma was made . after discussion about the possible modalities of treatment and the chance of spontaneous regression , the parents preferred regular follow - up without any active intervention and the child was kept under observation . histopathology showing well - circumscribed poorly - canalized capillary lobules in the dermis with crescent - shaped spaces around them . h and e , 100 showing well - defined capillary tufts surrounded by crescent - shaped clefts . ta was described for the first time by wilson jones in 1976 as an acquired vascular proliferation of peculiar histological characteristics and reports of additional cases had helped better characterization of this clinicopathologic entity . it commonly presents in infancy or early childhood , though adult - onset cases have been reported also . ta usually presents as a macule , papule or plaque over the upper trunk , neck and proximal part of the limbs ; involvement of other locations like face , oral mucosa and lip is also known . ta is a sporadic disease with more than half of the cases occurring during the first 5 years of life . hyperhidrosis , as in our case , and hypertrichosis can also occur over the lesion . eccrine sweat gland elements however were not seen to be increased in the histopathology of our patient . ta should be differentiated from kaposiform hemangioendothelioma , juvenile capillary , or strawberry angioma , haemangiopericytoma , glomeruloid haemangioma , or pyogenic granuloma . ta can however be diagnosed by the distinctive histopathological pattern characterized by the vascular tufts of densely packed capillaries , randomly scattered throughout the dermis in a so called cannonball configuration . there are crescentic spaces surrounding the vascular tufts and lymphatic - like spaces within the tumor stroma . immunohistochemical stains show strong positivity for ulex uropaeus i lectin and en4 and unlike infantile hemangioma , negative staining for glut 1 . ta , like kaposiform hemangioendothelioma , may be associated with the kasabach - merritt syndrome ( kms ) . physicians should look for the appearance of petechiae or ecchymotic patches and if they occur , a complete hemogram ( including a platelet count ) , prothrombin time and/or activated partial thromboplastin time and full screening for disseminated intravascular coagulation should be ordered . common association with kms , morphologic similarity , association in the same patient , and common expression of lymphatic markers had led to the suggestion that ta may be a minor form of kaposiform hemangioendothelioma or part of a spectrum of the same condition . ta usually pursue a persistent course , but spontaneous regression may occur in a proportion of cases . different modalities of treatment including complete surgical excision , cryosurgery , and radiotherapy had been tried for ta . pulsed dye laser has also been reported as an effective option for treatment of ta . embolization and vincristine are found to be effective in patients having kms . in conclusion , we have reported a rare case of multiple tufted angiomas with annular morphology in a child . the possibility of this tumor should be kept in mind while dealing with patients having lesions of similar morphology . we described a boy who had multifocal lesions with large annular plaques as well as papules and nodules .

tufted angioma ( ta ) is a localized benign hamartomatous vascular proliferation usually presenting in the childhood as an erythematous plaque . we report here a rare case of multifocal ta in an 8-year - old boy who presented which two large annular lesions as well as multiple papules and nodules on the back for the duration of 4 years . histology showed typical well circumscribed poorly canalized vascular lobules with cannon ball configuration .

diabetic muscle infarction ( dmi ) , which was described for the first time by angervall and stener in 1965 , is a rare complication of dm . it is usually seen in long - standing dm along with complications , such as , nephropathy , retinopathy and neuropathy . until now it commonly occurs in the thigh muscles and we have been able to find only five patients with upper extremity involvement.[46 ] in this article , we describe a patient with poorly controlled dm and muscle infarction of her forearm . a 58 year - old - woman with a three - week history of left upper forearm pain was admitted to our hospital . she noted a history of type ii dm , which was diagnosed five months ago . her current condition began with a sudden onset of swelling and pain in the left forearm , extending to her fingers . before this recent admission , several antibiotics and non - steroidal anti - inflammatory drugs were prescribed for her , with no improvement . on physical examination the white blood cell count was 17500 ( normal , 480010800 ) with 80% neutrophils ( normal , 3375% ) . fasting blood glucose was 235 mg / dl ( normal , 70105 ) and hemoglobin a1c was 6.5% ( normal , < 6% ) . other laboratory results were as follows : erythrocyte sedimentation rate ( esr ) 17 mm / hour ( normal , 025 ) , creatine kinase ( ck ) 5930 u / l ( normal , 25175 ) , lactate dehydrogenase ( ldh ) 780 u / l ( normal , 0480 ) , aspartate transaminase 89 u / l ( normal , < 31 ) , and alanine transaminase 53 u / l ( normal , < 31 ) . magnetic resonance imaging ( mri ) showed diffuse edema and abnormal low and high signal intensities in the soft tissues of the left forearm [ figure 1 ] . her muscle enzymes were gradually decreased to 143 and 504 for creatine kinase ( ck ) and lactate dehydrogenase ( ldh ) , respectively . she was discharged without any swelling or pain eight days later . on a follow - up visit after two months , she did not have any complaint in her forearm . after three months , post - treatment mri was performed that showed no abnormal signal intensity in the soft tissues , or bony structures [ figure 2 ] . it shows diffuse edema and low signal intensities in the left forearm muscles , with subcutaneous soft tissue swelling ( arrowheads ) . ( b ) a sagital t2-weighted magnetic resonance image shows diffuse edema and high signal intensities in the forearm muscles , with subcutaneous soft tissue swelling ( arrow ) ( a ) axial t1-weighted and ( b ) coronal t2-weighted magnetic resonance images after three months show no abnormalities its exact cause is unknown , but several hypotheses have been described , including , arteriosclerosis and microangiopathy , hypoperfusion - induced anoxia , abnormalities of the coagulation system , association with antiphospholipid antibodies , and hypoxia - reperfusion injury . most patients are young and females , with a mean age of 42 years . unlike our patient , they usually have long - standing type i dm . the presentation of dmi ispain and swelling that mostly involves the thigh muscles , and rarely , like in our patient , involves the upper extremity . the best diagnostic tool is the mri , which shows high and low ( or normal ) signal intensity on t2- and t1-weighted images , respectively . although the diagnosis of dmi can be confirmed by muscle biopsy , and it was done in a majority of the cases in the past , due to nonspecific findings and increased risk of recurrence , it is better for it to be reserved for atypical cases . the differential diagnosis of dmi is broad and the most common ones are thrombophlebitis , soft tissue infection , and diabetic amyotrophy . our patient was afebrile , her blood cultures were negative , and we did not find any evidence of muscle or soft tissue infection . the best method for dmi management is the conservative method , with blood glucose control . she responded well to the rest , with blood glucose control , and without any recurrence in the follow - up visits . diabetic muscle infarction should be noted in the differential diagnosis of any diabetic patient presenting with a swollen and painful limb .

diabetic muscle infarction ( dmi ) is an unusual complication of diabetes mellitus . it is usually seen in long - standing diabetes mellitus . this article presents a case of dmi in the left forearm of a 58-year - old woman . she had a swollen forearm . the level of creatine kinase was 5930 u / l . her condition was initially suspected for either cellulitis or venous thrombosis . a magnetic resonance imaging ( mri ) study of the forearm showed diffuse edema and abnormal signals of the left forearm . the diagnosis of dmi was made . she was treated conservatively and her symptoms resolved within a short period of time . dmi should be considered as a differential diagnosis of any painful and swollen limb in diabetic patients .

a 43-year - old man had sudden blurring of vision , metamorphopsia , and paracentral scotoma in his left eye on the second postoperative day of dacryocystorhinostomy operation on the same side . ocular examination showed best - corrected visual acuity of 20/20 in the right eye and 20/80 in the left eye . fundus fluorescein angiogram detected multiple leakage sites with smokestack pattern because of pooling of the dye under the neurosensory retinal detachment in the macula [ fig . 1 ] . after one week of omission of the nasal drops , there was a gradual resolution of symptoms . fundus photograph and fluorescein angiogram of the left eye showing few hyperfluorescent lesions within the foveal avascular zone and along the superior arcade , from the early venous phase , with evidence of profuse leakage along with pooling of dye during the late phase , indicative of cscr central serous chorioretinopathy is a multifactorial disease , which took many years to reach the precise clinical definition because of controversial etiopathology . it is usually associated with corticosteroid use , type a personality , organ transplantation , pregnancy , hypertension , and psychopharmacologic medication . recently , we have also reported a case of bilateral cscr following one intraarticular injection of corticosteroid . in this patient or case , only a topical instillation of xylometazoline in nasal mucosa produced cscr . it is documented that post - traumatic stress is associated with excessive release of catecholamines . topical application of xylometazoline , an alpha adrenergic receptor agonist , through the nasal route can cause severe systemic side effects . even a single intranasal dose of xylometazoline has been shown to be associated with coma in a neonate and sudden death in a 13-year - old boy due to the rupture of an intracranial aneurysm . in an observational case series of cscr associated with the use of sympathomimetic agents , occurrence of one case of cscr followed the use of oxymetazoline nasal spray three to four times daily for one month . in our case , excessive systemic absorption of xylometazoline from the inflamed nasal mucosa may have enhanced the post - traumatic stress - induced adrenergic stimulation leading to an increase in the choroidal circulation and alteration of the pump action of the retinal pigment epithelium , events which are related to the development of cscr . on the contrary , the intranasal instillation of a few drops of xylometazoline may have been a chance association with the occurrence of cscr , but one interesting point to refute this concept is the spontaneous quick resolution of visual symptoms after discontinuation of this topical medication . this case report widens the spectrum of drugs whose use should be enquired into while examining a patient with cscr .

a 43-year - old man developed central serous choroidoretinopathy in his left eye following dacryocystorhinostomy operation on the same side . he was using xylometazoline nasal drops in his left nostril . action of xylometazoline or the stress related to the operation or the effect of both factors played the role in the causation of this ocular condition . omission of nasal drops or relief from stress resulted in full recovery of vision and complete resolution of symptoms within one month .

to report a case of choroidal excavation accompanied by vogt - koyanagi - harada disease ( vkh ) . a 54-year - old japanese woman who was complaining of bilateral blurring of vision associated with headache underwent optical coherence tomography ( oct ) , fluorescein angiography , and indocyanine green angiography as well as a routine ophthalmological examination . oct of the left eye revealed the presence of a unilateral choroidal excavation under the fovea and subretinal fibrin over the site of the excavation . treatment successfully resolved vkh symptoms with gradual resolution of subretinal fibrin and fluid ; however , the choroidal excavation remained . our results suggest that choroidal excavation can be induced by choroidal inflammation caused by vkh . due to the increased availability of optical coherence tomography ( oct ) imaging , the entity of focal choroidal excavation has gained interest in recent years . it has been well - described in healthy individuals with otherwise normal eyes [ 1 , 2 , 3 , 4 ] . focal choroidal excavation has been defined as an area of choroidal excavation in the macular area detected with oct without the existence of a posterior staphyloma or scleral ectasia . we report a case of unilateral choroidal excavation in a patient with vogt - koyanagi - harada disease ( vkh ) . a 54-year - old japanese woman presented with bilateral blurring of vision associated with headache that had started 2 weeks earlier . her general family medical history and her personal medical history , including her history of ocular disease , were unremarkable . the best - corrected visual acuity on presentation was 0.6 in the right eye and 0.1 in the left eye . slit - lamp biomicroscopy showed bilateral anterior chamber cells and posterior synechiae , and fundus examination showed bilateral disc swelling with exudative retinal detachment ( fig . fluorescein angiography detected bilateral multifocal leakage with pooling of dye in the subretinal space in the late phase ( fig . 1d ) . a spectral - domain oct ( cirrus hd - oct 4000 ; carl zeiss meditec inc . ) image of the eyes showed the bilateral presence of subretinal fluid and pigment epithelial detachment . moreover , only in the left eye was unilateral choroidal excavation detected under the fovea , and the contour of the retinal pigment epithelium appeared to be conforming to the shape of the fibrin ( fig . thus , our patient was treated with intravenous pulse methylprednisolone followed by oral prednisolone ( initial dose of 40 mg / day ) with tapering over a course of 6 months . treatment successfully resolved the vkh symptoms , and serial oct showed a gradual reduction in size and a subsequent disappearance of the subretinal fibrin and fluid ( fig . d ) . however , 1 month after having started treatment , the eye was left with an area of choroidal excavation at the fovea despite a normal contour in the right eye ( fig . 2d ) . there was a good recovery of visual acuity , with 1.2 and 0.9 in the right and the left eye , respectively . since then , many reports have been published , discussing the association between choroidal excavation and central serous chorioretinopathy [ 2 , 3 , 4 ] . however , the current case of choroidal excavation associated with vkh is the first report in which a preexisting choroidal inflammation caused these conditions . concerning the pathogenesis of choroidal excavation in our case , it has been speculated that subretinal fibrin might exert a direct pressure effect on the choroidal layer by disrupting its integrity . our case suggests one of the possible mechanisms of developing choroidal excavation as a result of ocular inflammation . it is likely that the inflammation induced focal choroidal atrophy , and thinning could also contribute to the development of the choroidal excavation . a further study should be warranted to elucidate the clinical course and possible mechanism of choroidal excavation associated with vkh . in conclusion the pathogenesis of choroidal excavation still remains to be addressed ; however , the current case highlights the fact that choroidal excavation can be induced by choroidal inflammation caused by vkh . the authors have no proprietary or commercial interest in any of the material discussed in this article .

purposeto report a case of choroidal excavation accompanied by vogt - koyanagi - harada disease ( vkh).methodsa 54-year - old japanese woman who was complaining of bilateral blurring of vision associated with headache underwent optical coherence tomography ( oct ) , fluorescein angiography , and indocyanine green angiography as well as a routine ophthalmological examination.resultsfundoscopy showed papilloedema and serous retinal detachment in both eyes . fluorescein angiography detected bilateral multifocal leakage with pooling of dye in the subretinal space . indocyanine green angiography showed patches of hyperfluorescence and hypofluorescent spots bilaterally . a diagnosis of vkh was reached soon afterwards . oct of the left eye revealed the presence of a unilateral choroidal excavation under the fovea and subretinal fibrin over the site of the excavation . treatment successfully resolved vkh symptoms with gradual resolution of subretinal fibrin and fluid ; however , the choroidal excavation remained.conclusionsthis case is the first report of choroidal excavation associated with vkh . our results suggest that choroidal excavation can be induced by choroidal inflammation caused by vkh .

internal limiting membrane ( ilm ) free autografts have been used to repair refractory macular holes instead of using conventional surgical techniques like vitrectomy and peeling off the ilm , with encouraging results according to several authors . furthermore , to treat retinal tears in retinal detachment surgery , laser therapy or cryotherapy are used with excellent results , achieving appropriate sealing of the tears and thus contributing to retinal reattachment . laser therapy is also applied for the transoperative treatment of retinotomies that are occasionally performed to drain the subretinal fluid . not having those means to achieve tear sealing poses a complication that in some hospital sites , mainly in third world countries like mexico , is not entirely uncommon . in this case report we describe the use of an ilm autograft to seal a transoperative retinotomy in a patient who underwent primary vitrectomy for rhegmatogenous retinal detachment and proliferative vitreoretinopathy . a 55-year - old female patient came for consultation with sudden loss of vision and also reporting a rapidly progressive superior altitudinal scotoma of her right eye . on examination , the patient had a visual acuity of hand motion on her right eye , and fundus examination with a goldman 3-mirror lens revealed total retinal detachment with peripheral horseshoe tears in the 1 , 2 , 7 , and 9 o'clock positions , with presence of an inferior and temporal fixed fold . vitrectomy surgery was then performed with membrane peeling under perfluorocarbon fluids , placing a # 287 exoplant with a # 240 band and silicone oil as intraocular tamponade . the tears were all treated with laser therapy . while the air - fluid exchange was being completed , a retinotomy in the raphe was inadvertently made . laser treatment of the retinotomy site was no longer possible at that moment due to malfunction of the laser device . 0.02 ml of brilliant blue g ( fluoron gmbh , ulm , germany ) were injected into the vitreous cavity to stain the ilm . afterwards , a tano scratcher and an ilm forceps were used to peel off a small graft of the ilm adjacent to the retinotomy site . once obtained , it was displaced under a perfluorocarbon fluid bubble and placed inside the retinotomy ( fig 1 , fig 2 ) . finally , a perfluorocarbon fluid - air exchange was performed , and 5 ml of silicone oil were injected into the vitreous cavity . three weeks later the retina was completely reattached and the retinotomy sealed with the ilm graft ( fig 3 ) . it was not possible to take an optical coherence tomography ( oct ) image due to the presence of a lens opacity . peeling of the ilm is nowadays considered one of the main steps that contribute to a higher success rate in macular hole surgery . this has been achieved more efficiently with the use of dyes such as brilliant blue g that provide better visualization of such membrane [ 3 , 4 ] . furthermore , some techniques have been reported using an ilm flap as well as free ilm autografts over and inside the macular hole , with good anatomical and functional results according to several authors [ 1 , 5 , 6 ] . conventionally , to treat rhegmatogenous retinal tears , methods such as diathermy , cryotherapy or laser therapy have been used . in the latter , for example , an inflammation area this effect induces the formation of a chorioretinal scar around the retinal tear that seals it and prevents vitreous fluid from entering into the subretinal space and detaching the retina again . in this case report we describe the use of an ilm autograft to block the retinotomy , since we did not have these conventional methods available anymore at that moment of the surgery . the apparent sealing of the tear at the 3-week postoperative follow - up could be observed clinically , although it was not possible to confirm this finding by an oct image . in different histological preparations , mller cell remains were found in the ilm once it had been removed from the rest of the retina . these cell remains might be involved in the migration and repositioning of photoreceptors as well as in the formation of fibroglial tissue . hence , the ilm autograft might work as a potential source of mller cell remains and as a scaffold where fibroglial tissue can probably grow , contributing to the sealing of the retinal defect [ 1 , 5 , 8 ] . in their study of a modified technique of autologous transplantation of ilm for macular holes , hernndez - da mota and bjar - cornejo reported the presence of oct hyperreflective images where the ilm autograft was placed , which might suggest the presence of this type of tissue filling the macular hole defect . the same phenomenon might occur in rhegmatogenous retinal tears and retinotomies such as the one presented in this case report . animal studies as well as larger case series of patients and eventually comparative studies with conventional techniques are required to determine the real impact of the use of ilm autografts for the treatment of rhegmatogenous retinal detachment and retinal tears . other possible applications of this surgical technique combined with a retinal pigment epithelium autograft might be in diseases such as albinism and degenerative myopia that have little pigmentation of the retinal pigment epithelium .

during a pars plana vitrectomy , an unplanned retinotomy in the raphe was performed in a 55-year - old female patient with rhegmatogenous retinal detachment and proliferative vitreoretinopathy . since diathermy and laser therapy were not available at that moment , it was decided to peel off a small graft of the internal limiting membrane adjacent to the retinotomy site which had been previously stained with brilliant blue g. the graft was displaced under perfluorocarbon fluids and placed inside the retinotomy . three weeks after surgery , the apparent closure of the retinotomy was observed clinically .

mucosal cysts of the minor salivary glands are oral soft - tissue lesions that arise as dome - shaped swellings seen commonly on mucosal surface of lower lips and buccal mucosa . many modalities of treatment for mucosal cysts exist , including excision and suturing , deroofing , marsupialisation , radiosurgery and cryotherapy . four patients of age between 9 and 25 years were treated between june and december 2011 . a male patient aged 9 years had a dome - shaped smooth , tense , bluish cyst of size 5 mm on the lower lip . the second patient was a female aged 24 years , with a mucosal cyst of 4 mm on the buccal mucosa . the third patient , a female aged 21 years , had a lesion of size 6 mm in the buccal mucosa . the fourth patient was a male patient aged 25 years , who had a recurrent mucosal cyst [ figure 1 ] in the buccal mucosa which was treated 10 months back by electrocautery . no local anaesthesia was used . under aseptic precautions , a very small quantity of 88% phenol was injected into the cyst cavity using insulin syringes [ figure 2 ] . patients were given analgesics post procedure for 1 day . injection of 0.1cc of 88% phenol into cyst the procedure was uneventful in all the cases . two of the patients complained of slight burning sensation during the procedure , which subsided within a few minutes . this was followed by complete healing with slight scarring within 2 weeks [ figure 3 ] . although many other modalities of treatment of mucosal cysts exist , intralesional 88% phenol presents a simple and effective way of treating them . the advantage is that there is no intraoperative or postoperative bleeding , there are minimal surgical defects and there is minimal scarring . hence , intralesional injection of 88% phenol presents an easy and effective way for treatment of mucosal cysts .

mucous cysts are one of the common cystic lesions of minor salivary glands seen mostly on mucous surface of lower lip and buccal mucosa . many modalities of treatment for mucosal cysts exist including excision and suturing , deroofing , marsupialization , radiosurgery and cryotherapy . we have used 88% phenol as a mode of treatment of mucosal cysts .

spinal muscular atrophy ( sma ) is a neuromuscular disorder characterized by degeneration of the -motor neurons , leading to symmetrical muscle weakness and atrophy mainly of the lower limbs 1 . here , we report on an infant with sma type 1 with a rare compound heterozygosity of the survival of motor neuron 1 gene ( smn1 ) and characteristic fasciculations of the tongue . a 4-month old , caucasian boy with an unremarkable antenatal history ( first child of nonconsanguineous , healthy parents , no polyhydramnios ) was admitted to our hospital due to dyscataposia and mucus congestion with suspicion of a pulmonary infection . a mild muscular hypotonia was observed by the family 's pediatrician during standard examinations by age 1 month . our examination revealed a poor sucking reflex , failure to thrive , proximal and peripheral muscular weakness with a bell - shaped chest and arreflexia . lower limbs were more strongly affected than upper limbs . on inspection , fasciculations of the tongue were observed most prominently at the periphery of the tongue ( video s1 ) . magnetic resonance imaging ( mri ) of the brain showed a mega - cisterna magna ( mcm , fig.1 ) . during hospitalization , molecular genetic testing showed a rare compound heterozygosity for a point mutation c.815a > g ( p.y272c ) in exon 6 of the smn1 gene ( omim 600354 ) on the paternal allele and a deletion of smn1 exon 7 and 8 on the maternal allele , in the presence of two copies of the smn2 gene ( omim 601627 ) confirming the diagnosis of sma . at parental request spinal muscular atrophy is a heterogeneous neuromuscular disorder and the second most common lethal , autosomal recessive disease in caucasians after cystic fibrosis 2 . three clinical subtypes depending on age of clinical onset and maximum motor function have been identified 1 . type 1 ( werdnig - hoffmann , omim 253300 ) the most severe and common type of sma has an early onset and progressive unrelenting course resulting in death due to respiratory insufficiency within the first 2 years 2 . characteristic clinical features include profound hypotonia , symmetrical paralysis , little or no head control and areflexia 1,3 . fasciculations and atrophy of the tongue affect roughly one - third to one - half of patients with sma , and may be noted during the first months of life , as in our patient 3 . fasciculations of the tongue may also be seen in neonates with other medical conditions such as hypoxic - ischemic injury ( hie ) , mobius syndrome , and storage disorders ( pompe disease ) 3 . usually , these distinct clinical entities can be differentiated from sma by taking a detailed birth history ( e.g. , for hie ) or taking into account other characteristic clinical findings ( e.g. , macroglossia in pompe disease ) . in addition to tongue involvement , fasciculation of the eyelids may also be seen in children with sma . several studies in infants have found an association between cerebrospinal fluid space abnormalities such as ventricular dilatation and congenital myotonic dystrophies 4 ; such abnormalities , like dilatation of the cisterna observed in our patient , may not result in serious complications and remain in most of the cases uninvestigated . mcm is a controversial entity which is generally thought to be an anatomic variant with no clinical significance but may constitute part of several malformation syndromes such as dandy walker complex ( dwc ) . to the best of our knowledge , there is only one reported association between sma type i and blake 's pouch cyst , which along with mcm is regarded as a less severe malformation included in the dwc 5 . the vast majority of patients display a homozygous absence of smn1 exons 7 or 8 or exon 7 only , whereas only a few ( approximately 4% ) show compound heterozygosity for a point mutation on one and a deletion on the other chromosome 2 . unfortunately , in patients who do not present a homozygous disruption of the smn1 gene , adversities in terms of diagnosis , prognosis and genetic counseling still occur . c.815a > g ( p.y272c ) , a missense mutation in a highly conserved region in exon 6 of smn1 68 , accounts for approximately 20% of sma patients with compound heterozygosity for a point mutation 2,6 . p.y272c is found to result in almost complete reduction in the self - oligomerization capacity 9 , which suggests it to be a severe mutation8 and is in agreement with our case . in conclusion , muscular hypotonia is a common clinical sign in infants and may be associated with several conditions including neuromuscular disorders , connective tissue disorders , metabolic diseases , or even prematurity . the presence of tongue fasciculations , although not pathognomonic , and in combination with a thorough history , may be suggestive of sma diagnosis . additional supporting information may be found in the online version of this article : video s1 .

key clinical messagemuscular hypotonia in infants may be associated with several conditions , such as spinal muscular atrophy ( sma ) . we report on an infant with tongue fasciculations and a rare mutation of the smn1 gene . the presence of tongue fasciculations in combination with a thorough history may be suggestive of sma .

zolpidem is a short - acting nonbenzodiazepine hypnotic of the imidazopyridine class used for short - term treatment of insomnia . zolpidem selectively activates only one of the benzodiazepine binding sites of the gabaa receptor ( alpha-1 ) which may account for its selective sedative effects and relative lack of muscle relaxant and anticonvulsant effects compared to benzodiazepines . previously , some rare cases of zolpidem - induced hallucinations have been reported from developed countries . a 20-year - old female patient visited our psychiatric clinic with sadness of mood and irritable behavior for 4 months . now she would remain worried and preoccupied with thoughts of her marital life most of the time in a day with gradual loss of interest in pleasurable as well as routine activities . she had to push herself to go to office and would not take much interest in grooming herself now . her biological functions were disturbed and her risk assessment for suicide revealed a high score . she was diagnosed as a case of major depressive disorder and was put on paroxetine 12.5 mg twice a day and zolpidem 10 mg at bed time for sleep . next day , she reported with complaints of experiencing dream - like pleasurable state in which there was lilliputian like visual and elementary auditory hallucinations after half an hour of ingestion of zolpidem . the mood disturbances observed in the patient slowly over the next 3-month treatment were replaced by euthymia most of the time . the patient started doing her routine activities and no any hallucinations were reported by the patient in her follow - up . in the index case report , the patient developed hallucinations after intake of first dose of zolpidem . patient experienced lilliputian visual and elementary auditory hallucinations within half -an hour of intake of zolpidem . its onset of action is usually within 15 minutes and its half - life is 2.6 hours . , the australian therapeutic goods administration attached a black box warning to zolpidem , stating , that zolpidem may be associated with potentially dangerous complex sleep - related behaviours that may include sleep walking , sleep driving , and other psychotic behaviours . keto and koga reported visual hallucinations in an 82-year - old woman with diagnosis of major depressive disorder after 1 and half month of administration of zolpidem . in contrast , our index patient reported the visual hallucination on the first night . there are reports of occupational hazards with zolpidem - induced hallucinations in a 54-year old driver and the continued use of zolpidem with fluoxetine resulted in nystagmus and gait disturbances . it was recommended that zolpidem be used for short periods of time using the lowest effective dose . zolpidem 10 mg is effective in treating insomnia when used intermittently no fewer than three and no more than five pills per week for a period of 12 weeks . tsai et al . postulated that visual hallucinations associated with zolpidem may be related to sudden withdrawal and restarting of zolpidem . further the author hypothesized the mechanism of this phenomena was associated with alteration in the gaba - a receptor . the author also suggested that zolpidem should never be used as needed basis and the dose should be lowest effective dose . zolpidem has not proven effective in maintaining sleep and is more used for sleep initiation problems . our case highlights that clinicians must be aware of the various pharmacological properties of zolpidem and this molecule can induce hallucinations as was observed in the indexed case . further , such cases suggest more research in this clinical area for the better wellbeing of the patients .

we are reporting a case of zolpidem - induced hallucinations in a 20-year - old patient . the duration of this phenomenon was brief , 15 - 20 minutes . our case suggests that clinicians must be aware of this phenomenon while prescribing zolpidem .

disease research in pathology informatics requires archiving , retrieving , organizing , sharing , and analyzing diverse pathology - related data sources . the most important data source for pathologists is the data collected by anatomic and clinical pathologists ( e.g. , blood tests , surgical pathology reports , autopsy reports , annotated images , and specialized studies ) . it is a sad irony that the data collected by pathologists is seldom available for serious scientific inquiry . many pathologists can not freely access the full set of anatomic and clinical data collected within their own departments , though de - identified pathology data is exempted from regulation by hipaa and the common rule . any pathology department could , without violating federal laws , de - identify and distribute their archived anatomic and clinical pathology datasets to the scientific community . for reasons economic , legalistic , and psychologic , no pathology departments have , as yet , distributed de - identified collections of their collected datasets to the public . as a result , there is virtually no scientific research currently being conducted on large , multi - institutional collections of pathology data . moreover , the primary data for research done on collections of single - institution data is seldom released to the public . when research is conducted on pathology data , and the data is withheld from the public , there is no way to validate the conclusions . hence , the u.s . national academy of sciences , along with the editors of many scientific journals , have established a policy requiring authors to release the primary data supporting their conclusions . because pathology departments have not released their de - identified research records to the public , record - based pathology informatics research can not be published in journals that conform to the national academy of sciences recommendations . in the rare instance where an institution has published scientific results based on global analyses of their own datasets , the raw data , upon which those results are based , scientific results have no value unless the results are backed by data that can be openly examined . where pathology departments have failed , the u.s . enormous sources of individualized but de - identified death records and cancer records are available at no cost to medical researchers . eighty - five million de - identified death records are available from the cdc ( center for disease control and prevention ) . each record contains basic demographics on the decedent ( age , race , gender , place of death ) , the cause of death , and ( if provided in the death record ) the underlying causes of death and significant additional medical conditions . each annual data file provides about 2 million byte - indexed sequential - line records . this means that there is one death record per line , and each line contains coded data indexed for ranges of bytes that are designated by a data dictionary . for example , the icd-10 code of the underlying cause of death , for deaths occurring in 1999 , are found in bytes 142 - 145 of each record . this may seem like an awkward way of organizing data , when you consider the ease with which modern specifications ( such as rdf ) can encapsulate data with metadata . nonetheless , the byte - indexed sequential files can be parsed very efficiently with just a few lines of code . though pathologists prefer autopsy data over death certificate data , autopsies are performed on a very small fraction of decedents . despite early efforts to standardize and collect autopsy data , pathologists have not succeeded in sharing their autopsy data in a national database . consequently , death certificates are the most important source of mortality data available to medical researchers . seer offers a public use dataset , containing de - identified records on about 4 million cancers that have occurred since 1973 . each seer record is a single cancer case . with about 4 million carefully curated cases , scientists can draw certain types of inferences that could not possibly be made with the data accumulated at a single medical institution . to get the seer public use data files , you must first complete a data access request available at : seer.cancer.gov/data/request.html seer sends you a username and password that you will need to access the data files . each seer record is a line on a data file , and consists of 264 alphanumeric characters . byte data includes the patient 's race , gender , age at diagnosis , primary tumor site , diagnosis , and information related to tumor size and occurrence of metastases . a data dictionary provides the byte location of the various field values contained in each record . in the past several years , i have written hundreds of blog entries explaining how open source materials ( i.e. , data , algorithms , nomenclature , metadata specifications , and software ) can be used to collect , organize , integrate , and analyze pathology - related information . the blogs are available at : julesberman.blogspot.com a blog tutorial for seer data files appeared on november 14 , 2008 . a blog tutorial on the cdc mortality files appeared on december 2 , 2008 . all of the 300 + blogs entries for the blog site can be accessed through a linked archive web page at : http://www.julesberman.info/blog_in.htm blog entries related to the cdc and seer data include topics such as data mashups ( e.g. , mapping the geographic locations of disease occurrences ) , age distributions ( e.g. , following the average age of occurrence of diseases that progress through diagnostic categories over time ) , and trends in disease incidence ( e.g. , measuring the annual incidence of genetically screened diseases ) . there are blog entries of general interest to those working with pathology datasets , including entries on de - identification methods , image manipulation and annotation , data specification methods , and various computational algorithms .

the day has not arrived when pathology departments freely distribute their collected anatomic and clinical data for research purposes . nonetheless , several valuable public domain data sets are currently available , from the u.s . government . two public data sets of special interest to pathologists are the seer ( the u.s . national cancer institute 's surveillance , epidemiology and end results program ) public use data files , and the cdc ( center for disease control and prevention ) mortality files . the seer files contain about 4 million de - identified cancer records , dating from 1973 . the cdc mortality files contain approximately 85 million de - identified death records , dating from 1968 . this editorial briefly describes both data sources , how they can be obtained , and how they may be used for pathology research .