Acute gallbladder volvulus continues to remain a relatively uncommon process, manifesting itself usually during exploration for an acute surgical abdomen with a presumptive diagnosis of acute cholecystitis. The pathophysiology is that of mechanical organo-axial torsion along the gallbladder's longitudinal axis involving the cystic duct and cystic artery, and with a pre-requisite of local mesenteric redundancy. The demographic tendency is septua- and octo-genarians of the female sex, and its overall incidence is increasing, this being attributed to increasing life expectancy. We discuss two cases of elderly, fragile women presenting to the emergency department complaining of sudden onset right upper quadrant abdominal pain. Their subsequent evaluation suggested acute cholecystitis. Ultimately both were taken to the operating room where the correct diagnosis of gallbladder torsion was made. Pre-operative diagnosis continues to be a major challenge with only 4 cases reported in the literature diagnosed with pre-operative imaging; the remainder were found intra-operatively. Consequently, a delay in diagnosis can have devastating patient outcomes. Herein we propose a necessary high index of suspicion for gallbladder volvulus in the outlined patient demographic with symptoms and signs mimicking acute cholecystitis. Acute gallbladder volvulus continues to remain a relatively uncommon process, manifesting itself usually during exploration for an acute surgical abdomen with a presumptive diagnosis of acute cholecystitis. The pathophysiology is that of mechanical organo-axial torsion along the gallbladder's longitudinal axis involving the cystic duct and cystic artery, and with a pre-requisite of local mesenteric redundancy. The demographic tendency is septua- and octo-genarians of the female sex, and its overall incidence is increasing, this being primarily attributed to increasing life expectancy. Despite significant challenges in pre-operative diagnosis, a high index of suspicion and prompt surgical intervention results in an overall mortality of approximately 5 percent.A 99-year-old Caucasian female presented with a 3 day history of acute onset right upper quadrant abdominal pain with intermittent radiation to the right flank and back. It was described as colicky in nature on a baseline dull character, and with no obvious precipitating, aggravating or relieving factors. Associated phenomena included anorexia and nausea, but no constitutional upset, vomiting, or change in bowel habit. Her medical history included peptic ulcer disease, uncontrolled hypertension, tobacco abuse, diverticulosis, a hiatal hernia, and dementia. Her surgical history was significant for an appendectomy.Clinical physical examination revealed an apyrexic frail patient in no acute distress with stable vital signs. Focused abdominal examination demonstrated a soft, mildly distended abdomen with tenderness to palpation in the right upper quadrant, and a positive Murphy's sign. There was no overt peritonism. A reducible left inguinal hernia was also appreciated. Laboratory parameters yielded a mild leukocytosis with neutrophilia, and hypokalemia. Her liver function enzymes were elevated in a cholestatic distribution with a total bilirubin of 3.9 mg/dL, direct bilirubin of 0.9 mg/dL, and an alkaline phosphatase of 150 IU/L.A computed tomography (CT) scan was initially obtained prior to surgical consultation; it demonstrated a largely distended, hydropic gallbladder, pericholecystic fluid with wall thickening, a dilated common bile duct and prominent intra-hepatic bile ducts Figure . A hydroThe patient and her durable power of attorney (DPOA) refused the recommended surgical intervention of cholecystectomy. Non-operative measures with bowel rest, intravenous fluids and intravenous antibiotics were instituted. On hospital day 2, progressive elevation in her bilirubin and alkaline phosphatase prompted a gastro-enterology consultation and an endoscopic retrograde cholangiogram (ERC). This demonstrated dilatation of intra- and extra-hepatic bile ducts, a patent cystic duct, but non-filling of the gallbladder Figure . A sphinA cholecystostomy tube was planned, but due to unfavorable anatomy through the liver, it could not be performed. On hospital day 6, despite a normal white blood cell count and apyrexia, she complained of worsening abdominal pain. Following an appropriate pre-operative cardiac workup, the patient and DPOA then consented to an open cholecystectomy with a presumptive diagnosis of acute cholecystitis.On entering the abdominal cavity, a gangrenous distended gallbladder with omentum adhesed to it circumferentially was immediately noted Figure . On furtShe succumbed from cardio-respiratory failure on post-operative day 4, and was made comfort care respecting her do not resuscitate wishes.An 89-year-old Caucasian female with no significant past medical history presented with acute right upper quadrant abdominal pain of approximately 5 hours duration. The pain radiated to the right flank, was crampy with intensities of sharpness, and was precipitated by a large meal. There were no aggravating or relieving factors. Associated phenomena included anorexia and nausea, but no fevers, chills or change in bowel habit. Her past surgical history was significant for an appendectomy.Focused clinical abdominal examination revealed a soft, mildly distended abdomen tender to palpation in the right hypochondrium; a positive Murphy's sign was present. She was afebrile with stable vital signs; laboratory parameters were within normal limits. An abdominal ultrasound revealed a distended gallbladder with mild wall thickening Figure . There wAfter appropriate consent, the patient was taken to the operating room for a laparoscopic cholecystectomy with a pre-operative diagnosis of acute cholecystitis. After entering the peritoneal cavity and appropriate establishment of pneumoperitoneum, exploration quickly revealed an obvious necrotic gallbladder in the right upper quadrant. Further investigation noted that the gallbladder was twisted 180 degrees on its small pedicle with a thrombosed cystic artery. Following reduction of the torsion, the gallbladder was resected in the standard laparoscopic fashion. Histology demonstrated congested and ischemic serosa with necrotic mucosa consistent with torsion. Her post-operative course was unremarkable and she was discharged on post-operative day 1.First reported by Wendel in 1898, and dubbed the "floating gallbladder", gallbladder volvulus is a recognized surgical entity .Multiple hypotheses have been proposed as to the mechanism of gallbladder torsion, but the exact etiology continues to be unidentified. The pre-requisite of local mesenteric redundancy however is necessary for organo-axial torsion around its pedicle. Two anatomic variants have been described: 1) a torsion-prone mesentery, and 2) a mesentery supporting only the cystic duct allowing a completely peritonealized gallbladder to hang free. The susceptibility for rotational instability may be compounded by the elderly's fat loss and tissue atrophy suspending the gallbladder freely . This waet al; this relationship may also be attributable to our first case when reviewing her history and to our knowledge, is the only other report of this association in the literature [et al investigated 245 cases in the Japanese literature noting that cholelithiasis is an infrequent cause of gallbladder volvulus; gallstones were demonstrated in only a quarter of patients afflicted [Further mechanisms may include violent peristaltic movements of neighboring organs, visceroptosis, and a tortuous atherosclerotic cystic artery . Kyphoscterature . Nakao ePatients presenting to the emergency department with an acute surgical abdomen complaining of right upper quadrant abdominal pain invite a myriad of differentials including acute cholecystitis, choledochal cysts, choledocholithiasis, gastritis and peptic ulcer disease, intussusception, acute appendicitis, and nephrolithiasis. Laboratory parameters are equally unrewarding and non-specific noting general inflammatory changes.The correct pre-operative diagnosis of gallbladder volvulus is very challenging, with less than a dozen cases having been diagnosed accurately with pre-operative imaging . DespiteOperative surgical intervention involving reducing the torsion followed by removal of the gallbladder is the treatment of gallbladder volvulus. With further surgical advances, this has been reported safely with laparoscopic approaches in both the adult and pediatric population regardless of obtaining the correct diagnosis of torsion before surgery -12.Gallbladder volvulus continues to remain an uncommon surgical condition despite an increase in incidence. Although multiple imaging modalities are involved in attempting to obtain an accurate pre-operative diagnosis, no one has proven to be adequately sufficiently sensitive. The prompt diagnosis is critical to ensure that the patient undergoes an emergent index cholecystectomy rather than temporizing measures with antibiotics for a subsequent interval intervention. Herein we revisit and remind that the onus is on the surgeon to practice with a necessary high index of suspicion for gallbladder volvulus in the outlined patient demographic in order to circumvent treatment delays that may be fatal.Written informed consent was obtained from the patients for publication of these case reports and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.NJM designed and drafted the manuscript, performed the literature search and was involved in the critical review. BC, RS, RD and BK were all involved in the peri-operative and surgical care of the patients. RD and BK provided supervision in drafting the manuscript and its critical review. All authors read and approved the final manuscript.


Development of a sarcomatous component in a germ cell tumor is an uncommon phenomenon. Most cases reported have a grim prognosis. Growing teratoma syndrome is also an uncommon phenomenon and occurs in approximately 2% to 7% of non seminomatous germ cell tumors and should be treated surgically.We report the case of a 12-year-old Asian girl with an ovarian mixed germ cell tumor containing a rhabdomyosarcomatous component. She was treated with a germ cell tumor chemotherapy regimen and rhabdomyosarcoma-specific chemotherapy. Towards the end of her treatment, she developed a retroperitoneal mass that was increasing in size. It was completely resected, revealing a mature teratoma, consistent with growing teratoma syndrome. She is still in complete remission approximately three years after presentation.The presence of rhabdomyosarcoma in a germ cell tumor should be treated by a combined chemotherapy regimen (for germ cell tumor and rhabdomyosarcoma). In addition, development of a mass during or after therapy with normal serum markers should raise the possibility of growing teratoma syndrome that should be treated surgically. Development of a sarcomatous component (SC) in a germ cell tumor (GCT) is a rare phenomenon. Many histologic types of SC are described which can be present in the primary tumor or in the metastatic sites. The presence of sarcomas within GCTs is mostly encountered in testicular and mediastinal GCT while their presence in an ovarian GCT has rarely been reported. Meticulous histological examination is very important to determine the type and percentage of SC within a GCT as this affects prognosis. Growing teratoma syndrome (GTS) is also a rarely reported phenomenon. It was first described in 1982 . It requThe patient's primary tumor histopathology, her response to chemotherapy, and development of rare GTS make this patient's case worth reporting.A 12-year- old Asian girl presented with lower abdominal pain and distension of two months duration. A non-tender abdominal mass was found by palpation. Past medical history was unremarkable. She had not had her menarche yet. Computerized tomography (CT) scan showed a huge, heterogeneously enhancing pelviabdominal mass with multiple cystic and necrotic areas, originating from the right ovary Figure . The resShe was treated with eight courses of chemotherapy Table . She wasSix months after presentation and while she was on chemotherapy a right hypochondrial mass was detected by physical examination. Imaging studies revealed a new complex multiloculated mass in the right suprarenal area Figure . Serum mShe is still in complete remission 32 months after presentation.We describe an ovarian mixed GCT with rhabdomyosarcomatous components and elevated serum AFP and B-HCG in an adolescent girl. The development of SC in GCT is an uncommon phenomenon. Histologic types of SC reported in the literature are the following: rhabdomyosarcoma, high grade unclassified sarcoma, rhabdomyosarcoma admixed with high-grade unclassified sarcoma, angiosarcoma, and low-grade myxoid sarcoma -7. The set al. They identified 46 cases of GCT with SC. Rhabdomyosarcomatous (RMS) components were found in 28 cases. Only two patients were in the pediatric age group. Details of treatment were not reviewed, however most patients were treated recently, suggesting the use of multimodality treatment. Nevertheless, the outcome of these patients appeared to be less than favorable [The largest study was published by Malagon Although the pathogenesis of the development of sarcomatous components in GCT has not been fully elucidated, origins proposed have included dedifferentiation or malignant transformation of certain mesenchymal components within teratomas, origin from primitive germ cells or transformation of the blastematous stroma in yolk sac tumor ,8,12. UpGTS is a very rare phenomenon. Prognosis is favorable when surgery is radical ,3. OtherThe pathogenesis of the GTS is still unclear but two mechanisms have been considered: malignant cell differentiation into mature teratoma (MT) or a chemotherapy-induced destruction of the component other than mature teratoma  . As our Successful pregnancy after development of GTS has been reported indicating the necessity of a fertility sparing surgical approach in the treatment of young female patients .Many factors have been proposed that might predict the subsequent development of a GTS including the following: presence of MT in the primary NSGCT; no reduction in the size of metastases during chemotherapy; and the presence of MT in post chemotherapy residual masses .Clinical complications associated with GTS were estimated as approximately 12% and generally related to organ compression . MalignaAlpha-interferon therapy has been reported as an option for treatment of recurrent mature teratoma although it was not confirmed in a cohort of patients . New invWe report a rare case of an ovarian mixed GCT associated with a rhabdomyosarcomatous component which was treated successfully using a combined regimen (for GCT and RMS). The case was also complicated by the occurrence of the GTS which was treated surgically.Written informed consent was obtained from the patient's next-of-kin for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.UA interpreted the patient data and was a major contributor to the writing of the manuscript. AA collected the clinical data. MAlh obtained and interpreted pathological studies. FAj obtained and interpreted radiological studies. UA and IS reviewed the literature. All authors read and approved the final manuscript.


Introduction. Intussusception is a rare cause of bowel obstruction in adults and is usually associated with an underlying pathology, benign, or malignant. This is a report of a case of jejunojejunal intussusception secondary to non-Hodgkin's B-cell lymphoma in an adult patient. Case Presentation. A 74-year-old male with no previous significant medical history presented with symptoms of acute intestinal obstruction. A CT scan of the abdomen and pelvis revealed 2 areas of jejunojejunal intussusception, which were surgically managed successfully. Histopathological examination of the specimen revealed the presence of high grade diffuse large B-cell-type non-Hodgkin's lymphoma, and the patient was referred to the oncology team for further management. Discussion. B-cell lymphoma is a rare but well-documented cause of intussusception in adults, with most cases being at the ileocolic region. We present a rare case of jejunojejunal intussusception as the initial presentation of non-Hodgkin's B-cell lymphoma in an adult patient. Intussusception of the bowel, first reported in 1674, can be described as the telescoping of a proximal segment of the intestine within the lumen of the adjacent segment [Lymphoma is a very rare cause of adult intussusception, with only 36 cases reported in the literature between 2000 and 2011 [We report a rare case of non-Hodgkin's B-cell lymphoma presenting with 2 areas of jejunojejunal intussusception. A similar presentation, with one area of jejunojejunal intussusception, has been reported only once previously .The patient was a 74-year-old male who presented to the emergency department of our hospital complaining of severe abdominal pain associated with vomiting and failure to defecate for three days. A detailed history revealed that his symptomatology included a 2 month history of significant weight loss (12 Kg over 2-months) and poor appetite. He also complained of a recent change in bowel habit, alternating between constipation and loose motions, and an intermittent colicky abdominal pain, which was relieved by passing flatus and defecation.9/L) and anaemia . An abdominal X-ray showed distended loops of small bowel. Clinical examination revealed a distended, soft abdomen, with generalised tenderness, but no peritonism, organomegaly, or masses. Bowel sounds were reduced and rectal examination was unremarkable. All other aspects of the examination were normal. Initial blood investigations showed raised inflammatory markers  scan of the abdomen and pelvis with contrast showed a 9 cm circumferential wall thickening of the small bowel inferior to the transverse colon, associated with two areas of intussusception and further abnormal loops of bowel . A decisPostoperatively the patient was managed on the Intensive Care Unit and was discharged to the ward on day 6. His course was complicated with an upper gastrointestinal bleed on day 7 due to a Mallory-Weis tear, which was managed with an emergency esophagogastroscopy. The remaining of the recovery period was uncomplicated and the patient was discharged after a total of 26 days of hospital stay. The histopathological examination of the specimens revealed features of high grade non-Hodgkin's B-cell lymphoma of the diffuse large B-cell type . TherefoPrimary lymphoma of the gastrointestinal tract accounts for 30–40% of lymphomas arising extranodally and comprises 10–15% of all non-Hodgkin lymphomas . In the Intussusception is rarely considered clinically in the differential diagnosis of adult patients with vague abdominal complaints. Therefore diagnosis is usually made on CT or during exploratory laparoscopy/laparotomy . The cliIn conclusion, intussusception in the adult population is usually associated with an underlying cause, including lymphoma. This report illustrates a rare case of a jejunojejunal intussusception secondary to a high grade diffuse large B-cell-type non-Hodgkin's lymphoma.


Homonymous quadrantanopsia results from retrochiasmal lesions in the visual pathway. Invasive mole is a benign tumor that arises from myometrial invasion of a hydatidiform mole via direct extension through tissue or venous channels. Cerebral metastasis of invasive mole is rare and there has been no report demonstrating homonymous quadrantanopsia as the first manifestation of metastasis in any trophoblastic neoplasms.We report the case of a 31-year-old Asian woman who presented with right homonymous inferior quadrantanopsia from the mass effect of a solitary cerebral metastasis from an invasive mole. A magnetic resonance image (MRI) of the brain showed a metastatic tumor in the left occipital lobe. The visual field improved slightly after chemotherapy. There was a reduction in the tumor size and the surrounding edema. This is the first case report demonstrating that homonymous quadrantanopsia should be included in the manifestations of the metastasis of an invasive mole.The presentation of homonymous quadrantanopsia must alert ophthalmologists to conduct a complete medical history and arrange specialist consultation. Homonymous hemianopia is usually secondary to stroke, head trauma, or tumors . Homonym2 Goldmann size III stimulus (0.43°diameter) on a dim background (31.5apostilb) revealed a right homonymous inferior quadrantanopsia (Figure A 31-year-old Asian woman who complained of 'loss of right side vision', headache, dizziness, nausea, paroxysmal eye pain, and blurred vision was seen in our clinic. Humphrey visual field testing with a 4-mma Figure . Her meda Figure . On eye 2 echogenic dots in the anterior wall of the lower uterine segment. An MRI of her brain revealed a low signal lesion at the left occipital lobe on a T1-weighted image (Figure On medical examination, an ultrasonic test of her pelvis showed 1.0 × 2.0 cme Figure  and highe Figure . An MRI e Figure , but shoe Figure .et al. [et al. [et al. [Hydatidiform mole refers to an abnormal pregnancy characterized by varying degrees of trophoblastic proliferation and vesicular swelling of placental villi associated with an absent or an abnormal fetus and or embryo . It has et al.  presenteet al.  and striet al.  leading et al.  reviewed [et al.  presente [et al.  reportedThis is the first reported case of cerebral metastasis by invasive mole presenting with a homonymous inferior quadrantanopia. The presentation of homonymous quadrantanopsia must alert ophthalmologists to search for a complete medical history and specialist consultation. The key value of urgent neuroimaging in all cases of acute onset homonymous hemianopsia should be emphasized. Additionally, physicians looking after patients with hydatidiform moles should consider metastasis in patients complaining of visual symptoms. The timely diagnosis and treatment could result in a favorable outcome.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.The authors declare that they have no competing interests.DLS was the major contributor in studying the case and writing the manuscript and was involved in the medical care of the patient. YZ was the physician who admitted the patient and performed the visual field test. FF is the head of the department of radiology and responsible for the MRI reading. YL was involved in the pathological diagnosis and MHL was involved in the chemotherapy of the patient. All authors read and approved the final manuscript.


Tuberculosis (TB) is still endemic in many developed countries. Involvement of the hand and wrist at presentation is extremely rare, and the diagnosis is often missed. A 57 years old male presented with swelling over the left wrist since 3 years Three swellings over dorsal aspect of the left wrist Soft in consistency Non tender Non compressible Mobile at right angles to the plane of the wrist joint. ESR: 45 mm in 1 hr and rest blood investigations were normal. Ultrsonography showed giant cell tumor of Extensor Digitorum sheath. X-ray: soft tissue swelling and MRI was suggestive of extensor tendon sheath extraskeletal synovial Koch's, or giant cell tumor of tendon sheath. Excision of swelling was planned and intraoperatively, rice bodies were seen inside it. Histopathological examination showed caseous necrosis with granuloma formation. Patient was put on DOT1 therapy. Tuberculous tenosynovitis was first described by Acrel in 1777. Rice bodies occurring in joints affected by tuberculosis were first described in 1895 by Reise. Rice bodies will be diagnosed on plain radiographs when mineralization occurs. More than 50% of cases recur within 1 year of treatment. The currently recommended 6-month course is often adequate with extensive curettage lavage and synovectomy should be performed. Surgery is essential, but the extent of surgical debridement is still debatable. The surgeon has to be aware of the significance of loose bodies when performing routine excision of innocuous looking wrist ganglia. Tuberculosis (TB) is still endemic in many developed countries. Involvement of the hand and wrist at presentation is extremely rare, and the diagnosis is often missed. Extrapulmonary tuberculosis involvement of the musculoskeletal system is uncommon, accounting for only 10% of tuberculosis (TB) cases.57-years-old male presented with swelling over the left wrist since 3 years. No history of trauma, fever. Past history: no history of tuberculosis, diabetes, and hypertension. Pulse-78 beat/min BP-128/70 mmhg.No pallor, cyanosis, clubbing, edema, and lymphadenopathyThree swellings over dorsal aspect of the left wrist: soft in consistency, nontender, noncompressible, and mobile at right angles to the plane of the wrist joint. Respiratory system: AEBE. Cardiovascular system: S1 S2 present. Abdominal examination: NAD. Central nervous system: NAD.  Hb: 12 gm%, TLC: 8600/cmm, P: 65%, L: 20%, M: 03%, and E: 01% ESR: 45 mm in 1 hr, Bsl (R): 95 mg%, BT: 01 min 5 sec, and CT: 04 min 10 sec, BUN: 25 mg%, S.creat: 0.8 mg%, and USG: Giant-cell tumor of Extensor Digitorum sheath.  X-ray: soft tissue swelling, MRI: extensor tendon sheath, extraskeletal synovial Koch's, or giant cell tumor of tendon sheath.Histopathological examination showed caseous necrosis with granuloma formation. Patient was started on DOTS Category I and stitches were removed on postoperative day 8.Tuberculous tenosynovitis was first described by Acrel in 1777 . Rice boTuberculous tenosynovitis of wrist is rare and treatment comprises of excision of lesion and antituberculous chemotherapy will minimize recurrence of disease. The surgeon has to be aware of the significance of loose bodies when performing routine excision of innocuous looking wrist ganglia.


The standard operation for carcinoma of the pancreatic head is a partial pancreaticoduodenectomy. Unusual histological findings may occasionally occur in the surgical specimen. We present three unusual histologic diagnoses after pancreaticoduodenectomy.In the first case, an 86-year-old Caucasian woman was admitted with abdominal pain and nausea. Preoperative evaluation showed a 3 cm cystic lesion in the head of the pancreas. Pathology revealed a benign multicystic mesothelioma. In the second case, a 45-year-old Caucasian man complained of nausea, vomiting and general malaise for several months. Endoscopic retrograde cholangiopancreatographic examination and a computed tomography scan showed a stenosis of the distal bile duct secondary to a mass in the head of the pancreas and duodenum. Histology showed an adenomyoma of the ampulla. In the third case, a 59-year-old Caucasian man presented with chronic alcoholic pancreatitis. A computed tomography scan revealed a 3.5 cm lesion in the head of the pancreas with cystic and solid components. Pathology showed an undifferentiated carcinoma, sarcomatoid variant.Partial pancreaticoduodenectomy is usually performed for ductal adenocarcinomas, neuroendocrine tumors or chronic pancreatitis. Compared to the majority of the above diagnoses, the three cases in our study are very rare. Benign multicystic mesothelioma is a very rare tumor that originates from the peritoneum. Although it demonstrates a benign clinical behaviour, it frequently recurs after resection. Adenomyoma of the bile duct or ampullary region is a very unusual, benign, localized lesion characterized by adenomyomatous hyperplasia. Undifferentiated carcinoma, sarcomatoid variant, is an aggressive tumor and is characterized by spindle cells. As the lesions were suspicious for carcinoma, partial pancreaticoduodenectomy was justified in all three patients. The histologic diagnosis after partial pancreaticoduodenectomy may differ from the preoperative and intraoperative findings. These cases demonstrate that a definitive diagnosis may only be obtained by a pathologic examination of the surgical specimen. The standard operation for pancreatic cancer within the head of the pancreas or uncinate process is a partial pancreaticoduodenectomy ('Whipple procedure'). The standard Whipple procedure involves the removal of the head of the pancreas, the distal part of the stomach, the duodenum, the first part of the jejunum, the common bile duct and the gallbladder. Pancreaticoduodenectomy was popularized by Dr Allen Whipple after his success in his initial three cases in 1935 . In 1978Occasionally, unusual pathology was present in the surgical specimens which were not initially suspected. We present three unusual histological findings.An 86-year-old Caucasian woman was admitted with nausea and abdominal pain. Abdominal and endoscopic ultrasound, as well as a computed tomography (CT) scan, showed a 3 cm cystic lesion in the head of the pancreas with a dilated pancreatic duct and regional lymphadenopathy Figure . UltrasoA gross 2.5 cm tumor of the head of the pancreas was described. Histopathology showed multiple small cysts covered by cubic mesothelial cells with uniform, small nuclei Figure  and 2B. A 45-year-old Caucasian man complained of nausea, vomiting and general malaise for several months. CA19-9 was within normal range (20.5 U/mL). A CT scan of the abdomen and endoscopic retrograde cholangiopancreatographic (ERCP) examination showed a mass in the head of the pancreas and duodenum with a stenosis of the duodenum and distal bile duct Figure . Due to A macroscopic examination revealed a 5.5 cm lesion in the ampulla of Vater. Microscopically, multiple hyperplastic glands and cysts were found. They were covered by a single-layer epithelium, consisting of cuboidal, columnar and, in part, mucinous cells. Hyperplastic glandular lobes were surrounded by hyperplastic mesenchymal tissue which consisted of muscle fibers, fibroblasts and myofibroblasts Figure  and 4B. A 59-year-old Caucasian man presented with chronic alcoholic pancreatitis. An ultrasound and a CT scan of the abdomen showed a 3.5 cm lesion of the head of the pancreas with cystic and solid parts . The prognosis is often limited to several months after resection [Undifferentiated carcinoma, sarcomatoid variant, is a rare variant of pancreatic tumors. The malignant tumor arises esection . Our patesection . Histoloesection . The spiIn light of these factors, complete resection offers the only potentially curative treatment option in malignant tumors of the head of the pancreas. A preoperative diagnosis could not be obtained in any of our cases without a biopsy. Although adenocarcinoma of the pancreas was suspected preoperatively in each case, the final pathology demonstrated unexpected and unusual diagnoses. Due to the suspicion of a carcinoma, partial pancreaticoduodenectomy was justified in all three patients. Although the Whipple procedure is still considered to be a major surgical intervention with high morbidity and low long-term survival, partial pancreaticoduodenectomy is indicated for all suspicious tumor-like lesions of the head of the pancreas. These unexpected pathologic diagnoses underscore the fact that confirmatory evidence of suspicious diagnoses by surgery is still the gold standard.CT: Computed Tomography; ERCP: Endoscopic Retrograde Cholangiopancreatography; CK: cytokeratin; CD: Cluster of differentiation; CA19-9: carbohydrate antigen 19-9; POD: postoperative day; PPPD: pylorus-preserving pancreaticoduodenectomy.The authors declare that they have no competing interests.Written informed consent was obtained from all patients for publication of this case report and accompanying images. A copy of the written consents is available for review by the Editor-in-Chief of this journal.NL was involved in the conception and design and wrote the first draft of the manuscript. KC was involved in the preparation and review of the manuscript, the literature review and made a substantial intellectual contribution. SEB performed the histopathological work-up and contributed to the pathology part of the manuscript. PK performed the radiological work-up and contributed to the radiology part of the manuscript. JSaE and CFE performed surgery and critically revised the manuscript. WTK was involved in the initiation of the report and made a substantial intellectual contribution to the conception, acquisition and interpretation of the data. All authors read and approved the final manuscript.


Ovarian sex cord-stromal tumors, including granulose cell tumors (GCTs), are a group of neoplasms that occur rarely, especially in children. Only 0.1 percent of all ovarian tumors and 4-5 percent of GCTs occur in children. The most common presentation of these tumors in children is precocious puberty. We report a 6 years old girl with isosexual precocity, presented as premature thelarche and vaginal bleeding. Ultrasonography of pelvis showed a hypoechoic solid mass of left ovary.Microscopic features of the resected mass were characteristic of juvenile GCT. Although in most of girls with precocious puberty, the etiology is idiopathic, important causes, such as ovarian tumors must be considered. Granulosa cell tumors of ovary first were described by Rokitansky in 1855.Pseudo precocious puberty is the most common presentation in young children. In older ages and adolescents, they cause other manifestations such as hirsutism, abnormal uterine bleeding and abdominal discomfort.The prognosis of the JGCT is excellent after salpingooophorectomy in patients who have only ovarian involvement.A girl, aged 6 years, with no significant past medical history presented with breast enlargement and intermittent periumblical abdominal pain of 4 months duration that was accompanied with nausea but without vomiting. She had no headache or visual complaint. At that time, medroxy progesterone, 5 mg PO daily was started with impression of pseudo-precocious puberty, because sonography of pelvis showed a cystic mass in left ovary. After several weeks, she developed intermittent vaginal bleeding and the drug was increased to 10 mg PO daily and LHRH agonist was added and she was referred to the Pediatric Endocrinology clinic in our medical center. When she was visited, her weight was 21 kg and her height was 120 cm. Breast enlargement and pubic hair were compatible with Tanner III. No abdominal mass was palpated in the abdomen. Her serum laboratory findings were consistent with peripheral precocious puberty: estradiol: 216 pmol/L; FSH: less than 0.5 IU/L; LH: less than 0.4 IU/L; DHEA-so4: less than 36 ng/ml; testosterone : 0.3 ng/ml; 17-OH-progesterone: 0.3 ng/ml; total T4: 9.1 μ g/dl; TSH: 2.4 mIU/L; alpha-fetoprotein: 2 ng/ml. LHRH test was done without increase in LH and FSH. Ultrasonography of pelvis showed a 6.2 × 5 × 4.1 cm hypoechoic solid mass in projection of left adnexa which had multiple cystic structures up to 1.2 cm, and left ovary could not be separated from it. Right ovary was normal. The size of uterus was appropriate for the patient’s age, with normal endometrial and myometrial echo pattern. dometrial thickness was 5 mm and no free pelvic fluid was detected by sonography. The spiral CT scan of pelvis showed a large non-uniform solid mass with multiple necrotic regions in the lower abdomen. So, we recommended diagnostic and therapeutic laparotomy for her. Under general anesthesia, laparotomy was done. An ovarian mass in the left side with diameters of 5 × 7 cm was detected and resected with left ovary. The right ovary was normal. No lymphadenopathy was detected in the pelvic area. The mass was sent for histopathologic study and ovarian JGCT was reported .Ovarian sex cord-stromal tumors are neoplasms containing granulosa cells, sertoli cells, theca cells, leydig cells, and fibroblasts of gonadal stromal origin.They should exclude primary gonadal disorders, adrenal gland, exogenous secretion of gonadotropin by a tumor, or autonomous ovarian cyst.13Advanced bone age and accelerated height velocity were seen due to tumor-derived estradiol.17


Introduction: Heterotopic mesenteric ossification is an extremely rare condition, which often follows trauma and is frequently symptomatic. To date, there are no reports in the literature of abdominal compartment syndrome occurring after surgical resection of mesenteric calcification. The present report documents an unusual case of compartment syndrome complicating resection of extensive mesenteric calcification despite abdominal closure with the components-separation technique. Method: A 48-year-old man undergoing components-separation technique for posttraumatic laparostomy hernia repair  was found to have extensive heterotopic mesenteric calcification, which needed resection. Results: Resection of the mesenteric calcification was complicated by intraoperative hemorrhage and unplanned small bowel resection. Later the patient developed secondary hemorrhage leading to an abdominal compartment syndrome, which was successfully treated by decompression, hemostasis, and Permacol-assisted laparotomy wound closure. The patient remains symptom-free more than 2 years after surgery. Discussion: The case herein reported gives an account of the rare occurrence of abdominal compartment syndrome following resection of posttraumatic ectopic mesenteric ossifications. It is highly unusual in that it occurred because of “secondary hemorrhage” and despite abdominal closure with the components-separation technique, which had been undertaken precisely to prevent compartment syndrome with direct closure. It therefore highlights the need for continued clinical vigilance in complex posttraumatic cases. Myositis Ossificans Progressiva.Paraosteoarthroplasty. Although Hansen was the first to describe peritoneal ossification in 1983,heterotopic mesenteric Ossification (HMO) was coined by Wilson et al in 1999 after concluding that 5 of their postabdominal surgical cases, with small bowel obstruction associated with heterotopic bone formation in the mesentery, clinically and pathologically resembled Myositis Ossificans as previously described by Ackerman.4,Heterotopic ossification was originally described in 1692 by Guy Patin, who noted the condition in children and termed it Intra-abdominal ossification is a rare entity with an otherwise poorly understood etiology and pathophysiology. Reported cases in literature often describe patients presenting with significant obstructive abdominal symptoms on a background of previous abdominal insult, with the process more likely to occur thereafter.7We herein report how one case of HMO, in an otherwise symptomless patient requiring complex surgical intervention, was complicated further by postoperative abdominal compartment syndrome (ACS) due to secondary hemorrhage.plastic surgery service. Prior to referral, he had undergone numerous exploratory laparotomies, washouts, and applications of vacuum-assisted closure (VAC) dressings. This period was further punctuated with an emergency decompression for ACS, pleural effusions, and a prolonged period of endotracheal intubation for treatment of respiratory failure.A 48-year-old man was admitted to hospital in mainland Europe with multiple traumatic injuries following a severe road traffic accident on the Christmas Day of 2009. He presented with a Glasgow Coma Scale of 4 to 6 with associated bilateral pneumothoraces; multiple rib fractures; left humeral, right femoral, and right patella fractures; and bilateral fractures of the L4 transverse processes and nasal bones. After initial hemodynamic stabilization, he underwent emergency cecostomy and loop ileostomy procedures for peritonitis secondary to “spontaneous” sigmoid colon perforation. The patient was subsequently transferred to the United Kingdom, where he spent 2 months on the intensive care unit (ICU) before being referred to the The patient was referred to the plastic surgeons for closure of his large “iatrogenic” abdominal hernia but the size of the defect and level of exposed herniating bowel precluded direct closure, necessitating the application of a split-thickness skin graft with VAC dressing.Six months later  or nonneoplastic origin.Interestingly, despite the increased relative risk posed and the mesenteric location of our patient's calcifications, he did not present with any obstructive signs or symptoms. The pathological findings from his excised specimens were however more in keeping with reported cases. Microscopy showed dense collections of lamellar bone, with some resembling the cortices of long bone and others resembling the medulla. These collections showed no active inflammation and were primarily within the vessels of excised fibroadipose tissue but also surrounded one normal lymph node. There were no features of malignancy in the report.Abdominal compartment syndrome is often a fatal, multiorgan, end-stage process, resulting from poorly compensated intra-abdominal hypertension. The latter occurs when increased capillary permeability in critically ill patients with systemic inflammatory response syndrome (SIRS) leads to the sequestration of fluid into the cavity. Our patient notably survived 2 episodes of hemorrhage-induced ACS, pre and post components-separation surgery, and has been symptom-free for more than 2 years.We concluded the innocuous appearance on radiological imaging, the significant challenge experienced during laparotomy, and the serious peri- and postoperative complications make HMO a rare but potentially fatal entity that warrants surgical intervention. Surgeons must therefore consider HMO and its complications as a differential when managing complex multitrauma patients with “suspicious” radiographic densities.


Collision tumors are exceptional, associating two independent tumoral contingents. We report a case of an association of two rare tumors: sinonasal adenocarcinoma and chondroid chordoma. Initially, only adenocarcinoma was diagnosed. The treatment consisted of endoscopic endonasal surgery followed by conventional radiotherapy. After 18 months, a local recurrence was diagnosed after a facial trauma, but the true histology was difficult to assess. The tumor was dual, associating adenocarcinoma and chondroid chordoma, with atypical localization in the ethmoid. Further evolution was particularly aggressive. We discuss the key points of this observation. Sinonasal adenocarcinoma and chondroid chordoma are both rare tumors.The incidence of sinonasal adenocarcinoma is less than 1 per 100,000 per year. The peak incidence is in the 5th to the 7th decades .Chordomas account for 2 to 4% of primary bone tumors, being the fourth most common pathology among primary bone cancers. The annual incidence is estimated to 1 per 1000,000, with a peak incidence in the 4th to 7th decade .We report and discuss here the first case of association of these two rare tumors.A 63-year-old man presented with a history of nasal obstruction and recurrent epistaxis for two months. He was woodworker for more than 40 years . He had no smell anymore for 20 years, which was thought to be due to the intensive use of varnish and glue. He also described a discrete right ptosis for several months. There were no additional symptoms. The patient had a history of hypertension, hypercholesterolemia, and septoplasty 30 years prior, for nasal obstruction.An initial examination found a polyp masking the olfactory cleft with many mucopurulent secretions. An antibiotherapy treatment (amoxicillin + clavulanic acid) and steroids were prescribed and a CT scan was performed. The CT scan highlighted a tissular process invading the posterior part of the ethmoid, the olfactory cleft, and the nasal septum, with swelling in the maxillary sinus. There was no evidence for skull base invasion or intracranial extension. The tumor was partially ossified . There wThe patient underwent endoscopic surgery . After aPatient followup was marked by the persistence of nasal obstruction because of crusts, requiring repeated and frequent local care, but without sign of recurrence.Eighteen months afterward, the patient presented with an increasing trismus, four weeks after a local trauma. It was associated with hypoesthesia and pain in the infra-orbital area. The CT scan highlighted a zygoma fracture. There was also a partial lysis from the posterolateral and the lateral wall of the maxillary sinus. This was considered to be highly suspicious, but as the endoscopic control found no evidence for recurrence, the first diagnosis retained was osteoradionecrosis. The patient was treated with pristinamycin, metronidazole, and pentoxifylline, with a control by CT scan and MRI at one month.The pejorative clinical and radiological progression—with palsy of the right oculomotor nerve—led to the realization of biopsies.Histopathological examination revealed a cartilaginous tumor with immature chondrocytes. Their nuclei were irregular, with densified chromatin and frequent nucleoli. The stroma was myxoid without lobular organization, with inflammatory infiltration and foci of necrosis. The diagnosis retained was grade II chondrosarcoma. A second biopsy led to the same diagnosis.After complete evaluation , the tumor was found to be centered on the maxillary sinus, invading its posterolateral and anterolateral walls, the infra-temporal fossa, and the fat space of the cheek, with bilateral node invasion, but without distant metastasis. Multidisciplinary discussions led to the proposition of a right maxillectomy with bilateral lymph node dissection.The tumor was approached through a Weber-Ferguson incision. The exeresis was extended laterally to the zygoma, upper to the superior part of the malar and periorbital soft tissues, medially to the right nasal bone, inferiorly to the homolateral hard palate, and posteriorly to the infratemporal fossa.The final histologic examination excluded the diagnosis of chondrosarcoma and revealed a dual tumor. There were foci of the initial adenocarcinoma, but the main contingent was composed of a chondroid chordoma. The chondroid component, mimicking a grade II chondrosarcoma was intricate with an epithelial component. Cells were organized in a trabecular, cribriform, or acinous mode, with a high nucleocytoplasmic ratio, hyperchromic nuclei, with thin chromatin and large nucleoli. Atypical mitoses were frequent. These areas of epithelial differentiation were sometimes included in a hyaline stroma. Some physaliphorous cells could be observed. Diagnosis was confirmed by immunohistochemical staining. Cells strongly expressed S-100 protein and were positive for cytokeratin AE1-AE3, EMA , and at a lower level for cytokeratin 19; margins were not clear. Lymph nodes were massively invaded by adenocarcinoma with capsular effraction. One subcutaneous lymph node was invaded by chondroid chordoma. As the presentation of this case was unusual, the diagnosis of chordoma was confirmed by a second laboratory and the histological slides of the first tumor were reviewed. This lead to the discovery of a small contingent of chondroid chordoma, lost in the adenocarcinoma, in ethmoidal samples. The collusion of the two tumors was present at the outset . HoweverAfter multidisciplinary discussion, as no additional radiotherapy could be performed, targeted therapy was proposed with Imatinib.However, the situation worsened with a rapid course progression, no objective response to targeted therapy, local evolution, and pulmonary metastasis.The patient died shortly thereafter, two years after initial diagnosis.The association of chondroid chordoma and nasal adenocarcinoma has never been described before. This case raises the question of a possible link between both tumors.The physiopathology of chordoma remains unclear; chordoma derive from the primitive notochord. The initial lesions might be benign chordal ectopias called ecchordosis physaliphora, which are encountered in asymptomatic adults with an incidence of 1% .However, no precise responsible locus could be found in the different genomic studies, despite several different chromosomic abnormalities identified . LikewisNevertheless, the collision of the two tumors seems to have influenced their evolution. In a general manner, adenocarcinomas do not have a very aggressive behavior, which is reflected in their low metastatic potential 1-2%) [-2% [1], Another interesting aspect of this case is the initial localization of the chordoma in the ethmoid. Chondroid chordoma are thought to derive from the notochord. The notochord is a transient structure of embryogenesis, which arises as a pouch from the mesoderm during gastrulation. It defines the primitive axis of the embryo, conditions its elongation, and the formation of the neural tube. In higher vertebrates, it regresses completely throughout the column except in the nucleus pulposus. This process explains why chordomas are located along the axial skeleton  . Extra aThe localization of the adenocarcinoma was more typical. In a recent report, we pointed out that adenocarcinomas seem to arise from the olfactory cleft , so thatThis case illustrates the difficulty in diagnosing chondroid chordoma. The similarities with chondrosarcoma are so important that the existence of chondroid chordoma has long been questioned , 12. TheTherapeutic strategy is based on aggressive but safe surgery for both tumours , 3.Since our understanding that adenocarcinomas arise from the olfactory cleft, we have developed an endoscopic approach instead of lateral rhinotomy , 16. Thiα/β ratio for linear quadratic modeling, conventional radiotherapy has often been associated with particle beam radiotherapies or radiosurgery, which enables higher doses.Adenocarcinomas and chordomas have a poor radiosensitivity , 3; thisFor both tumors, conventional chemotherapy is usually only used in palliative intention , 3. For This is the first case of the association of chondroid chordoma and adenocarcinoma of the olfactory cleft. Our report features a particularly aggressive example of these two rare tumors, with an exceptional localization of the chordoma. It illustrates the difficulty in diagnosing chondroid chordoma, questions the endonasal endoscopic approach, and confirms the poor radiosensitivity of both tumors.


Gingival adenoid cystic carcinoma (ACC) is a rare malignancy. We describe the diagnosis and treatment of a 43 year-old woman who presented with a persistent oral ulcer for approximately 1 year, and subsequent pain in the left posterior maxillary region. Clinical examination revealed an ulcer in the left upper molar gingiva, with swelling in the region from the second premolar to the third molar. X-ray images demonstrated the involvement of the maxillary alveolar bone. The histopathological and immunohistochemical features were diagnostic of ACC. ACC is often presented as a gingival lesion; thus, it may easily be neglected by patients. The identification of this tumor using specific pathological analyses prevents misdiagnosis and enables clinicians to determine the appropriate treatment. In this case, no recurrence or distant metastasis was observed after 2 years of follow-up. Adenoid cystic carcinoma (ACC) is an uncommon malignant neoplasm that develops within secretory glands. ACC commonly occurs in the major and minor salivary glands of the head and neck. Other sites include the lungs, breasts, lacrimal glands, and skin,ACC is known to have long benign periods followed by instances of malignancy, but it may behave aggressively in some patientsA 43 year-old woman complained of an oral ulcer that persisted for almost 1 year as well as pain in the left posterior maxillary region for 1 month. Clinical examination revealed an ulcer on the left upper molar gingiva, with swelling from the second premolar to the third molar in the same region. X-ray images showed the involvement of the maxillary alveolar bone (Upon admission, malignant mesenchymal or odontogenic neoplasia was suspected. An incisional biopsy was then performed under local anesthesia. The histopathological and immunohistochemical features were diagnostic of ACC. Microscopic examination showed prominent solid, microcystic, and focally tubular glandular patterns of growth, as well as numerous cells with myoepithelial characteristics and a few luminal cells. The histopathological features were similar to those of the solid subtype of salivary gland ACC (The patient underwent left total maxillectomy, followed by buccal fat pad flap reconstruction. Histopathological analysis of the resected specimen showed similar features with those of the incisional biopsy. The surgical margins revealed no residual carcinomas.Extensive clinico-radiological investigations were performed to identify potential metastasis. Chest radiography, as well as computerized tomography of the head and neck regions, was conducted. No metastatic lesions or other primary neoplasms were detected. The patient remained in good health after 25 months of follow-up, with no evidence of local recurrence.Carcinomas of the maxillary gingiva are relatively common but easily neglected by patients. Such tumors differ from those that originate from the maxillary sinus and have secondary extensions to the gingiva. Radiography of the paranasal sinuses aids differential diagnosis and allows for the careful evaluation of the extent of bone involvement. Gingival carcinomas in the maxilla usually invade the maxillary sinus. These carcinomas may extend onto the palate or the tonsillar pillar.,ACC is one of the most common tumors in the salivary glands; it mainly involves the palate but rarely affects the gingivaMost, if not all, patients with long-term ACC eventually develop distant metastases, mainly in the lungs and bone, despite the local control of the tumor. The occurrence of bone metastasis usually corresponds to rapid tumor dissemination and death of the patient, whereas lung metastases demonstrate a less aggressive clinical course-In this case, the central salivary gland tumors should be included in the differential diagnosis. These tumors develop within the mandible or maxilla and can extend to the more usual locations. Most central salivary gland tumors of the jaw involve the mandible, which account for approximately 65% of intraosseous ACC


Here is a rare case of lingual leiomyomatous hamartoma (LLH) with bifid tongue tip and tongue-tie in a patient with non-oral-facial-digital syndrome (OFDS). A 29-year-old male consulted for a painless tumor over the midline of the tongue dorsum measuring 2 × 1.5 cm. The tumor was excised and the tongue-tie was corrected. Diagnosis of LLH was based on histo-pathologic and immuno-histochemical studies. The epidemiologic data and differential diagnosis of LLH, as well as related literature, are discussed. To date, only 14 cases of LLH have been reported in English literature. This may be the first reported case of LLH with bifid tip and ankyloglossia in a non-OFDS patient. Hamartoma was first defined by Alberecht  in 1904 Leiomyomatous hamartoma (LH) is composed mainly of smooth muscle tissue intermixed with other adjacent tissue and often occurs in the lungs and kidneys. Cases involving the oral cavity are extremely rare and most have occurred in the Japanese, with few cases among Caucasian and Latin Americans of young age ,3. Only The OFDS is a complex syndrome with 10 different subtypes that include oral, facial, and digital abnormalities. LH with a lobulated tongue tip or short frenulum has been reported in some types of OFDS . This reA 29 year-old man was referred in June 2008 for a painless, slow-growing mass over the tongue dorsum that had been noted since childhood. Upon examination, there was evidence of an exophytic, reddish, non-tender mass, measuring 2 × 1.5 cm over the midline of the tongue dorsum. There was also a bifid tongue tip and tongue-tie  in our report remains obscure. The sporadic mutation in the transcription factor may occur during the tongue development at the fourth embryonic week, causing the abnormality of morphogenesis at the end stage of the lingual swelling mergence. As poor fusion between the lateral and the medial lingual swelling occurs, the tongue tumor, combined with the bifid tongue and ankyloglossia may occur together on the tip of the tongue midline. Environmental factors, for example, poor nutrition; overuse of vitamins; use of drugs, alcohol or heroin; smoking; and exposure to thalidomide or pathogens may cause sporadic mutation with abnormality of morphogenesis during embryologic development.In patients with a congenital midline tongue mass, the differential diagnosis includes choristoma, leiomyoma, and benign mesenchymoma. Choristoma ,9 is a wHowever, there are other rarer tumors that should be put into the differential diagnosis of LLH. First, congenital epulis  which usImmunohistochemical characteristics are essential in differentiating soft-tissue neoplasms. The SMA and S-100 protein are the most commonly used markers in confirming the histomorphologic findings in LLH -4,7,8. HMost case reports advocate surgical excision with a safe margin as the treatment of choice for LLH ,5,9-14. Here is a special case of LLH combined with bifid tongue tip and tongue-tie in a patient without OFDS. To date, this may be the first such case reported in English literature. It is a reminder that when confronted with a smooth-mass lesion over the midline of the tongue dorsum, leiomyomatous hamartoma should be considered in the differential diagnosis, especially when the patient is a young adult and without OFDS.Written informed consent was obtained from the patient for publication of this case report and any accompanying images (IRB number: KMUH-IRB-20120262). A copy of the written consent is available for review by the Editor-in-Chief of this journal.LLH: Lingual leiomyomatous hamartoma; OFDS: Oral-facial-digital syndrome; SMA: Smooth muscle actin.The authors declare that they have no competing interests.HLW and LFW performed data collection, manuscript composition and submission. CFT and FYC took part in the care of the patient, and performed the literature search. KBT performed pathology analysis and photography. All authors read and approved the final manuscript.


Ischemic bowel disease comprises both mesenteric ischemia and colonic ischemia. Mesenteric ischemia can be divided into acute and chronic ischemia. These are two separate entities, each with their specific clinical presentation and diagnostic and therapeutic modalities. However, diagnosis may be difficult due to the vague symptomatology and subtle signs.We report the case of a 68-year-old Caucasian woman who presented with abdominal discomfort, anorexia, melena and fever. A physical examination revealed left lower quadrant tenderness and an irregular pulse. Computed tomography of her abdomen as well as computed tomography enterography, enteroscopy, angiography and small bowel enteroclysis demonstrated an ischemic jejunal segment caused by occlusion of a branch of the superior mesenteric artery. The ischemic segment was resected and an end-to-end anastomosis was performed. The diagnosis of segmental small bowel ischemia was confirmed by histopathological study.Mesenteric ischemia is a pathology well-known by surgeons, gastroenterologists and radiologists. Acute and chronic mesenteric ischemia are two separate entities with their own specific clinical presentation, radiological signs and therapeutic modalities. We present the case of a patient with symptoms and signs of chronic mesenteric ischemia despite an acute etiology. To the best of our knowledge, this is the first report presenting a case of acute mesenteric ischemia with segmental superior mesenteric artery occlusion. Ischemic bowel disease comprises mesenteric ischemia and colonic ischemia. Mesenteric ischemia can be divided into acute and chronic ischemia ,2. TheseA 68-year-old Caucasian woman was admitted to our Department of Gastroenterology for persistent abdominal discomfort, melena, anorexia and fever for several months. She had a history of hypertension, cholecystectomy, hysterectomy and bilateral ovariectomy. Further questioning revealed diarrhea, melena and anxiety to eat (sitophobia) due to severe postprandial abdominal pain. She had lost 5 kg of bodyweight prior to presentation.On physical examination, tender palpation in the left lower quadrant of her abdomen with slight rebound pain was noted. An irregular pulse was also discovered. An electrocardiogram detected paroxysmal atrial fibrillation. Her white blood cell count and C-reactive protein level were elevated. Computed tomography (CT) of her abdomen and CT enterography revealed a stenotic jejunal segment with reduced enhancement of her bowel wall and the presence of inflammation in her left iliac fossa Figure . EnterosOur patient was transferred to the Department of Abdominal Surgery for exploratory surgery. The stenotic ischemic jejunal segment was resected and an end-to-end anastomosis was performed Figure . A histoOur patient recovered from surgery without complications and was discharged on the tenth postoperative day. She underwent radiofrequency ablation for the atrial fibrillation and is currently under anticoagulant therapy. Follow-up has been uneventful to this date.Acute mesenteric ischemia (AMI) accounts for 60% to 80% of all cases of mesenteric ischemia and has a mortality rate between 59% and 93% -7. AMI cChronic mesenteric ischemia (CMI) is a rare condition that accounts for only 5% of all intestinal ischemic events ,10,11. IRisk factors for CMI are the same as those for atherosclerosis, namely hyperlipidemia, hypertension, diabetes and smoking ,10,11,13As with AMI, clinical diagnosis is often difficult due to the vague symptomatology. Symptoms of CMI typically develop when there is a postprandial increase in blood flow demand. The most characteristic symptom is intestinal angina. This typical abdominal pain occurs within the first hour after ingestion of a meal. It is often located in the epigastric region or the mid-abdomen and is described by patients as dull and crampy. Diarrhea, nausea and vomiting are commonly present ,6,8-13. Duplex ultrasonography, IADSA, CT angiography and magnetic resonance angiography can be used to detect intra-abdominal vascular disease ,2,6-13. In the current case, confusion existed about the true etiology. There was a discrepancy between her symptomatology, namely intestinal angina, diarrhea and sitophobia suggesting CMI, and the diagnostic sign of an isolated occlusion of a branch of the superior mesenteric artery, which pointed towards the diagnosis of AMI. No reasonable explanation could be found for the symptoms of melena and fever. The AMI was caused by an arterial embolism, most likely due to atrial fibrillation. In this context we may conclude that this case is a late and unusual presentation of AMI. In our opinion, it is the size of the embolism that is responsible for the localization of the ischemic zone. The localization of this zone is a coincidence.An extensive work-up was done as a consequence of the confusion. An upper gastrointestinal endoscopy and an enteroclysis were performed to indicate the severity, extent and location of the ischemic segment. However they proved unnecessary, as angiography was the most accurate imaging technique -13.Revascularization by operative or endovascular procedures in this case was not possible because of the distal site of the occlusion. Resection of her small bowel segment was the only appropriate action. However, generally, revascularization procedures are mandatory ,3-13.As far as we know, this is the first report presenting a case with segmental occlusion of the superior mesenteric artery causing AMI.Mesenteric ischemia can be divided into acute and chronic ischemia, two separate entities, each with their own specific clinical presentation and diagnostic and therapeutic modalities. Diagnosis may be difficult due to the vague symptomatology and subtle physical signs. These diagnoses should therefore always be kept in mind in any patient with chronic postprandial abdominal pain in whom no other diagnosis can be made. The diagnosis should be confirmed by angiography. Management of intestinal ischemia consists of blood flow restoration through medical treatment or surgical management, depending on the etiology.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-in-Chief of this journal.The authors declare that they have no competing interests.FvT performed the IADSA and commented together with KN on the radiological images used in the manuscript. AC was a major contributor in writing the manuscript. All authors read and approved the final manuscript.


Context. Hepatic portal venous gas (HPVG) is a rare and sinister finding. Its mortality is associated with the underlying causative condition. When secondary to bowel ischaemia, mortality rates exceed 50%. Case Report. Two cases of HPVG are described. One case describes HPVG in association with gastric ischaemia, with complete resolution following conservative management. The second case describes HPVG in association with widespread intra-abdominal ischaemia, with resultant mortality. Conclusion. A “watch and wait” management of HPVG associated with gastric ischaemia is suggested in certain patients, with a low threshold for surgical intervention. HPVG associated with bowel ischaemia is an absolute indication for surgical intervention, where intervention may change the clinical course. Hepatic portal venous gas is a rare and sinister finding. It is associated with numerous underlying abdominal pathologies, ranging from benign conditions that require no invasive treatment to potentially lethal diseases that necessitate prompt surgical intervention. When secondary to bowel ischaemia, mortality rates exceed 50% . We descAn 81-year-old man of Indian descent presented to our emergency department with severe abdominal pain. The pain was localised to the right upper quadrant and epigastrium, radiated to his back, came on suddenly, and had worsened over the previous 12 hours. His previous medical history included angina, hypertension, type II diabetes mellitus, glaucoma (registered blind), and recurrent postprandial abdominal pain for which he received telmisartan, bendroflumethiazide and latanoprost eye drops. The patient denied any alcohol intake or smoking. He was febrile (38.3°C), tachycardic, and tachypnoeic with other observations within normal limits. On examination, he had generalised abdominal pain with severe tenderness in the epigastric and right upper quadrant regions. There were no signs of peritonism and bowel sounds were normal.Full serological testing was performed which revealed a deranged liver profile , deranged renal profile , a raised amylase of 2197 IU/L, and a metabolic acidosis; inflammatory markers were mildly raised and other parameters were unremarkable.Erect chest X-ray and abdominal X-ray showed no pathology. With signs of an acute abdomen and diagnostic doubt, a computerised tomography (CT) scan was carried out. Due to the patient's elevated creatinine, a noncontrast CT scan was performed. CT revealed extensive hepatic portal venous gas. Branching air filled structures within the liver extending to the periphery, representing portal venous radicles, were noted . FurtherFollowing discussion with the regional upper gastrointestinal tertiary centre, due to extensive comorbidities, the patient was deemed unsuitable for surgery. The patient was managed conservatively by commencement of antibiotic therapy (piperacillin with tazobactam) and a proton pump inhibitor (IV pantoprazole). Three days following the initiation of treatment, clinical improvement was noted and a repeat CT of abdomen was carried out. CT revealed significant interval improvement—no intraabdominal gas was seen .The patient completed a two-week course of piperacillin with tazobactam. He remained asymptomatic, biochemical markers improved and he was discharged with outpatient follow-up. Endoscopy was carried out five weeks later demonstrating no abnormality. The patient was followed up in clinic for over a year, remaining asymptomatic throughout.A 74-year-old Caucasian woman presented to our emergency department with abdominal pain, bleeding per rectum, diarrhea, and vomiting. She reported a three-day history of generalised abdominal pain, vomiting and diarrhoea. In the morning of presentation, she had noticed a bright red PR bleed mixed with stool. She reported no fevers or recent travel. Her previous medical history included pancreatic insufficiency, diverticular disease, recurrent ischiorectal abscess, previous pseudomonas colitis, previous peptic ulcer, previous myocardial infarction, hypertension, hypercholesterolaemia, peripheral vascular disease, right femoral pseudoaneurysm, aortobifemoral graft, and chronic obstructive pulmonary disease. Her medications included pancrelipase, clopidogrel, bisoprolol, isosorbide mononitrate, omeprazole, ramipril, and simvastatin. The patient admitted to smoking 15 cigarettes a day and denied any alcohol consumption. She was afebrile, tachycardic, and tachypnoeic with other observations within normal limits. On examination, she had generalised abdominal tenderness. A pulsatile mass was felt in the right groin. There was no signs of peritonism and bowel sounds were normal.9/L, C-reactive protein 131 mg/L), and metabolic acidosis; other parameters were unremarkable. Stool cultures returned with no growth and were negative for clostridium difficile toxin, ova, cysts, and parasites.Full serological testing was performed which revealed a deranged renal profile , deranged liver profile , raised inflammatory markers  with markedly deranged liver profile . Abdominal USS was carried out. The liver was visualised with normal size, echogenicity, and contour. Doppler imaging showed normal portal vein flow. Adenomyomatosis of the gallbladder was noted and the abdominal USS was otherwise unremarkable. Chest X-ray, blood culture, urine culture, and stool culture at this time showed no pathology. Whilst vomiting had now been resolved, the patient had experienced continuing intermittent diarrhoea during admission. The patient was started on the antimicrobial piperacillin with tazobactam for a sepsis of unknown origin. A repetition of chest X-ray several days later confirmed a pneumonia of the right lower lobe. Repeat blood cultures returned positive for Citrobacter koseri sensitive to current antimicrobials. Her abdominal pain had now settled. Inflammatory markers, however, showed minimal improvement to the current antimicrobial regime. Further to this event, the patient complained of right upper quadrant pain. Serological testing revealed a sharp rise in inflammatory markers  is a rare and sinister radiological sign. It was first described by Wolfe and Evans in 1955 in infants with necrotizing enterocolitis  SubsequeThe exact pathophysiology of HPVG is unclear and several theories have been hypothesised. The escape of microbe-derived gas production from the bowel lumen or in an abscess which then circulates into the liver and the presence of gas forming organisms within the portal system are proposed factors . No cleaThe diagnosis of HPVG can be made by plain abdominal X-ray, ultrasound with Doppler imaging or CT. Abdominal X-ray will only detect significant volumes of HPVG—if present, mortality is in excess of 50% . UltrasoHPVG is associated with numerous underlying abdominal pathologies, ranging from benign conditions that require no invasive treatment to potentially lethal diseases that necessitate prompt surgical intervention , 10–12. The first case we have presented is a patient with HPVG due to gastric ischaemia. It has previously been recommended that patients with acute gastric ischaemia undergo partial or total gastrectomy . In our In conclusion, we have reported two cases of HPVG. One case was associated with only gastric ischaemia and showed complete reversibility with conservative management. The second case was associated with widespread intra-abdominal ischaemia and resultant mortality occurred. The first case suggests the possibility of a “watch and wait” management of HPVG associated with gastric ischaemia in certain patients; the second case highlights the high mortality rate of HPVG associated with bowel ischaemia and the necessity for surgical intervention, where intervention may change the clinical course.


Acute ischemia of the rectum resulting in full thickness necrosis is extremely uncommon because of its excellent blood supply.We present 3 cases with spontaneous ischemic gangrene of the rectum. All three patients were elderly with atherosclerotic arterial disease and presented with hypotensive shock but in none of these patients we encountered a precipitating factor such as preceding vascular surgery or shock state.A high index of suspicion should be maintained in elderly patients with atherosclerotic disease who present with lower GI symptoms with hypotensive shock and an inflamed rectum on CT scan. Immediate beside proctoscopy should be offered to these patients and if the diagnosis is confirmed these patients should be taken to the operating room immediately. If the entire rectum is found to be gangrenous then an emergency APR should be performed and the perineal wound left open. If the rectum is partially gangrenous then a low anterior resection with Hartman's procedure for diversion is appropriate.Prompt diagnosis and resuscitation followed by immediate surgical intervention is necessary to save these elderly patients. Normally, the rectum is very well vascularized and acute spontaneous ischemia of the rectum is an unlikely occurrence.22.1A 77-year old male with history of hypertension, insulin dependent diabetes, hyperlipidemia, prior stroke and Parkinsonism was admitted from the emergency room with history of bloody diarrhea and lower abdominal pain. He had history of diarrhea off and on for the last 6 weeks and was recently treated for C diff colitis. He was hypotensive and tachycardic and abdominal exam showed marked tenderness in the left lower quadrant. Digital rectal exam revealed dark red blood and chunks of mucosal slough. Investigations revealed WBC of 22,000, acute renal failure and acidosis. CT showed peri rectal stranding and pneumatosis of the rectal wall . BedsidePatient was resuscitated with fluids, vasopressors and IV antibiotics were started. He was taken for an emergency exploratory laparotomy. During surgery, complete gangrene of the rectum extending from recto sigmoid junction to the dentate line was found. An abdominoperineal resection of the rectum was performed. The levators were divided close to the rectum since the surgery was done for a non-malignant condition. Once the specimen was exteriorized, a left lower quadrant colostomy was performed and the perineal wound was packed with Kerlex gauze. Five days later, when the patient was hemodynamically stable, he was brought back to the operating room and the perineal wound was closed. Postoperatively, the recovery was uneventful and the patient was discharged to a nursing home on the 17th postoperative day. Histopathological examination of the rectal specimen showed extensive ischemic ulceration and necrosis extending to the muscularis propria.2.2A 93-year old otherwise very active male was admitted with lethargy and lower abdominal pain. He was constipated recently and took some laxatives, as a result he had diarrhea just before admission. Past medical history was significant for hypertension, atrial fibrillation, stroke and a recent TIA. He also had a sigmoid colectomy in past for cancer. Upon examination he was hypotensive and tachycardic. Abdominal exam showed lower abdominal tenderness and distention. Fecal impaction was found on digital rectal exam.White blood count was elevated at 24,000. Lactic acid was elevated. CT scan showed some fluid in the pelvis and mild peri rectal stranding. Patient was taken for an exploratory laparotomy in view of abdominal tenderness, septic shock, leukocytosis and lactic acidosis. After induction of general anesthesia, manual disimpaction of rectum was performed. Very foul smelling pus, stools and blood was encountered. Proctoscopy at this stage revealed a completely black rectum all the way down to the dentate line. During laparotomy a swollen, discolored and inflamed rectum was identified. An abdominoperineal resection of the rectum was performed. The perineal wound was packed with Kerlex gauze. Patient was brought to the operating room on the 5th postoperative day for delayed closure of the perineal wound. After a prolonged and slow recovery he was discharged to a nursing home on the 18th postoperative day. Histopathological exam of the specimen showed severe acute ischemic proctitis .2.3An 80-year old female was admitted from nursing home with history of bloody diarrhea. Past medical history included hypertension, hyperlipidemia and history of stroke. She was hypotensive and septic. White cell count was 17,000. CT scan showed thickening of the rectal wall. She was started on IV fluids, vasopressors and intravenous antibiotics. A bedside flexible sigmoidoscopy was performed which revealed severe ischemia and necrosis of the rectal mucosa . Mild is3Acute rectal ischemia is rare because the rectum has abundant blood supply and rich collaterals. The superior rectal artery which is the terminal branch of inferior mesenteric artery supplies most of the blood to rectum. Some blood is derived from the middle rectal arteries which are branches of the internal iliac artery or the internal pudendal artery and also from the inferior rectal artery which is a terminal branch of the internal iliac artery. There is extensive intramural anastomosis between the branches of these arteries especially in the lower part of the rectum. Extramural collaterals also occur between the lowest sigmoid and superior rectal arteries. This watershed area  is not considered to be critical anymore because adequate collateral are almost always present.In addition to these named branches rectum also receives blood supply from muscular branches of the levator, and some twigs from the inferior vesical or vaginal arteries. This extensive blood supply ensures that rectum is perfused even in cases where the rest of the bowel is compromised.Pathophysiology: Unlike ischemic colitis or proctitis, frank gangrene of the rectum is very rare. It is usually described in elderly patients with significant atherosclerotic disease and cardiac risk factors in the presence of hemodynamic instability. It occurs as a result of sudden acute compromise in blood flow in patients with inadequate collateral circulation around the rectum. Most reported cases in literature involve an acute event such as interruption of inferior mesenteric artery during aortofemoral bypass or ligation of internal iliac vessels in patients with atherosclerotic narrowing of major arteries. Hypotensive shock or a low flow state will compound this problem and the rectum becomes ischemic despite the usually excellent collateral circulation. In none of our patients, a preceding surgical event, hemodynamic compromise or low flow state was encountered.Other rare causes include angiographic embolization of the blood supply of the rectum, infected strangulated hemorrhoids, vasculitis and or immune complex thrombi related to systemic lupus erythematosisClinical presentation and diagnosis: Most patients will present with diarrhea and rectal bleeding. Abdominal and pelvic pain and distention is also common. Fever, tachycardia along with hypotension is often present. Lower abdominal tenderness is often present but because of the extra peritoneal pelvic position of the rectum these signs may be delayed. Nelson et al. has reported loss of anal tone as an early sign of acute rectal ischemia.Leukocytosis and acidosis are reflective of sepsis and shock. CT scan shows rectal wall thickening and peri rectal stranding. Pneumatosis in the rectal wall may also be present in advanced cases as was seen in one of our patients. Later rectal perforation with extra luminal air may be seen. Angiography, though not advisable in acute gangrenous cases, shows diffuse atheromatous disease the aortoilliac vessels with abrupt cut off.Endoscopic examination of the rectum either using a rigid proctoscope or a bedside flexible scope remains the cornerstone for diagnosis of rectal ischemia. Early findings include erythema, mucosal and sub mucosal hemorrhages, and ulceration. In the setting of lower GI symptoms these changes can be confused with IBD or infectious colitis. With more serious compromise of the blood supply necrosis or frank gangrene of the rectum will be seen. In two of our three cases the rectal wall was black and completely gangrenous.Management: In early cases or in cases with superficial ischemia conservative management is appropriate but in cases of frank gangrene of the rectum, emergency surgery is the only option. While preparations are being made for surgery patient should be resuscitated and started on broad spectrum antibiotics. In the OR patient should be placed in modified lithotomy position with Lloyd Davis stirrups to allow access to the rectum during surgery. Operation should start with a proctoscopy to evaluate the extent of ischemia. Visual inspection of the rectum during surgery will also help determine the extent and level of ischemia. In most cases a complete proctectomy is required. In the emergency situation this is best accomplished by an APR. The perineal wound is packed in order to avoid infection in a closed space and to expedite the operation. The wound can be closed after a few days when patient is stable and the risk of infection has decreased. Proctectomy may also be achieved with intersphincteric dissection and excision of the rectum but is a longer more complex operation. During an emergency operation every effort must be made to abbreviate the operation and get the patient back to ICU for further resuscitation.In cases where the lower rectum is spared a low anterior resection of the rectum along with a Hartman's procedure may be performed. We do not recommend leaving behind a gangrenous or ischemic segment of the rectum as has been suggested by some.4Acute full thickness rectal ischemia with gangrene is a rare clinical entity. Immediate bedside proctoscopy should be considered in elderly patients with atherosclerotic disease who present with lower GI symptoms, hypotensive shock and the CT shows an inflamed and swollen rectum. Conservative management is not appropriate in these cases and preoperative resuscitation followed by emergency surgery to resect the frankly gangrenous bowel is necessary.None.None.I have obtained written consent from the patient. This can be provided if needed.Khawaja Azimuddin: Data and case selection and preparation.Tal Raphaeli: Writing.


Background. Uterine leiomyosarcoma is a rare and aggressive gynecologic malignancy with an overall poor prognosis. Lungs, bones, and brain are common sites of metastases of uterine leiomyosarcoma. Metastases of uterine leiomyosarcoma to the small bowel are extremely rare, and only four case reports have been published to date. Case presentation. A 55-year-old Saudi woman diagnosed with a case of uterine leiomyosarcoma treated with total abdominal hysterectomy (TAH) and bilateral salpingooophorectomy (BSO) presented in emergency room after sixteen months with acute abdomen. Subsequent work-up showed a jejunal mass for which resection and end-to-end anastomosis were performed. Biopsy confirmed the diagnosis of small bowel metastasis from uterine leiomyosarcoma. Further staging work-up showed wide spread metastasis in lungs and brain. After palliative cranial irradiation, systemic chemotherapy based on single agent doxorubicin was started. Conclusion. Metastatic leiomyosarcoma of small bowel from uterine leiomyosarcoma is a rare entity and is sign of advanced disease. It should be differentiated from primary leiomyosarcoma of small bowel as both are treated with different systemic chemotherapeutic agents. Uterine leiomyosarcoma accounts for 3% of all uterine malignancies and it metastasize through hematogenous dissemination to the lungs, liver, bones, and the brain . Small bHerein we report a case of a 55-year-old Saudi women who presented in emergency room with acute abdomen secondary to small bowel metastasis from uterine leiomyosarcoma.A 55-year-old Saudi woman was admitted to the Emergency Department with a chief complaint of episodes of paroxysmal abdominal pain. The pain started two weeks previously that was aggravated for the last two days with nausea, vomiting, and constipation. Past medical and surgical history revealed that she was diabetic since last 12 years and was on oral metformin 500 mg three times a day and she underwent total abdominal hysterectomy (TAH) and bilateral salpingooophorectomy (BSO) sixteen months back for uterine leiomyosarcoma outside of our hospital without any adjuvant treatment.9/L), high absolute neutrophil count (10.4 × 109/L), and normal hemoglobin and platelets counts. Serum chemistry showed lactate dehydrogenase (LDH) was 237 U/L ↑ , sodium 132 mmoL/L ↓ , serum creatinine was 226 μmoL/L ↑ , urea 15.1 mmoL/L ↑ , anion gap was 31.2 mmoL/L , and lactate was 2.3 mmoL/L . Computed tomography (CT) abdomen without contrast showed distended stomach, proximal small bowel loops extending down to midabdominal level where there was abrupt change in caliber of the bowel, and collapse of distal small bowel loops. At this transition point appeared a soft tissue density containing vessels, which raised the possibility of intussusception with a lesion; During physical examination, moderate tenderness over the right epigastric and hypochondrial areas and active bowel sounds were observed. Plain abdominal X-ray showed dilatation of small bowel without any gas-fluid levels; After her successful postoperative recovery, staging workup was done. Bone scintigraphy was found normal. CT chest showed multiple pulmonary metastatic nodules; largest one was of size 10 × 6 cm in right middle and lower lobes invading adjacent mediastinum, mediastinal pleura, pericardium, and right pulmonary vein and bronchus; 2).After discussing her case in multidisciplinary board meeting, she was given palliative cranial radiotherapy 20 Gy in 5 fractions over a week and then she was started on systemic chemotherapy based on single agent doxorubicin (60 mg/mAt time of publication patient was found alive and was receiving chemotherapy.Small bowel metastases are uncommon and account for 10% of all small bowel tumors . The mosThe mechanism of metastasis to the heart is not well known. Possible routes are direct extension, hematogenous route, implantation, or via lymphatic route. In our patient small bowel was affected through hematogenous pathway. Metastatic leiomyosarcoma shall be differentiated from rare primary intestinal leiomyosarcoma with help of IHC; both are treated with different chemotherapeutic agents . PrimaryMetastatic small bowel leiomyosarcoma secondary to uterine leiomyosarcoma is considered a sign of advanced disease with high tumor burden as seen in our patient. Such patients have shown response to doxorubicin alone, gemcitabine alone, or gemcitabine plus docetaxel with median survival rates of 14.7 to 24 months .In conclusion, small bowel metastasis following treatment for uterine leiomyosarcoma is rare. IHC helps to differentiate it from primary leiomyosarcoma of small bowel. Metastatic leiomyosarcoma is sign of advanced disease with grave prognosis and such patients may benefit from single agent doxorubicin-based systemic chemotherapy.


Abdominal compartment syndrome (ACS) is defined as an elevated intraabdominal pressure with evidence of organ dysfunction. The majority of published reports of ACS are in neonates with abdominal wall defects and in adults following trauma or burns, but it is poorly described in children. We describe the unusual presentation of an 11-year-old boy with a long history of chronic constipation who developed acute ACS requiring resuscitative measures and emergent disimpaction. He presented with a 2-week history of increasing abdominal pain, nausea, diminished appetite and longstanding encopresis. On exam, he was emaciated with a massively distended abdomen with a palpable fecaloma. Abdominal XR confirmed these findings. Within 24 hours of presentation, he became tachycardic and oliguric with orthostatic hypotension. Following two enemas, he acutely deteriorated with severe hypotension, marked tachycardia, acute respiratory distress, and a declining mental status. Endotracheal intubation, fluid boluses, and vasopressors were commenced, followed by emergent surgical fecal disimpaction. This resulted in rapid improvement in vital signs. He has been thoroughly investigated and no other condition apart from functional constipation has been identified. Although ACS secondary to constipation is extremely unusual, this case illustrates the need to actively treat constipation and what can happen if it is not. An 11-year-old boy presented to the emergency department with a long history of abdominal pain, profound chronic constipation, and encopresis. His new increasing abdominal pain started two weeks prior to presentation with nausea and diminished appetite, preventing him from attending school or tolerating solid foods. On exam, he was tachycardic but otherwise stable. He looked emaciated, with a hugely distended but nontender abdomen filled with palpable stool. He could not stand straight and rectal exam revealed a rectum filled with hard stools and blood at the anal verge. Abdominal XR confirmed these clinical findings . Within The day following his disimpaction (36 hours later) he suddenly developed free intraabdominal air and was taken to the operating room where a pinhole-sized perforation was identified at the apex of his enormously dilated rectosigmoid. A Hartmann procedure was performed. A descending colostomy was created and biopsies of his bowel were taken. All investigations for Hirschsprung's disease or other intestinal motility disorders were negative. Echocardiogram done following his recovery showed no structural abnormality to the heart. The patient was discharged after 19 days. His colostomy was reversed after seven months, and he continues to do well to date taking daily oral PEG 3350 to maintain regular bowel movements.Abdominal compartment syndrome (ACS) is defined as an elevated intraabdominal pressure (IAP) with cardiovascular, renal, and pulmonary dysfunction, where abdominal decompression has beneficial effects –4. AboveACS causes organ failure through direct mechanical effects. Respiratory derangements result when the elevated diaphragm decreases functional residual capacity and increases airway pressures, as depicted in our patient during his acute deterioration . CardiovACS is poorly described in children, and its development from constipation is very seldom reported in the pediatric literature, with only 2 cases published to date. Birkhahn and Gaeta, describe a boy with congenital megacolon who developed ACS from massive colonic dilatation, where surgical decompression resulted in reperfusion injury, coagulopathy, and the patient's fatal demise . GoreckiThorough investigations were undertaken in attempt to identify a primary etiology. Colonic and rectal biopsies were negative for Hirshsprung's disease. Repeat thyroid and celiac screens were negative, and an MRI of his spine ruled out the possibility of a tethered cord. A Shapes study was consistent with a diffuse colonic dysmotility, an intestinal motility study was unremarkable, and anorectal manometry revealed no evidence of outlet dysfunction . DespiteWe report a unique case of an otherwise healthy 11-year-old boy with severe longstanding functional constipation that evolved in abdominal compartment syndrome. His rapidly progressive presentation was identified early and managed with immediate resuscitation and prompt manual decompression. As depicted in this illustrative case, the importance of vigilant treatment of habitual constipation cannot be overemphasized. This rare case supports the need for aggressive management of constipation, as suboptimal management has the unfortunate but rare potential to progress to life-threatening conditions such as abdominal compartment syndrome.


Pachydermodactyly is a rare benign superficial fibromatosis, aetiology unknown, characterized by asymmetric painless swelling of the proximal interphalangeal (PIF) joints of the hands, most frequently in adolescent males. Radiographs and MRI show only soft tissue thickening. Histological exam reveals epidermic hiperplasia, collagen fibers and fibroblast proliferation and increased mucin deposition.Report a case of Pachydermodactyly.Analysis of the patient’s clinical record.rd PIF joint of the left hand. In 3 months all left hand PIF and MCF joints were affected. Three years later similar lesions appeared in the right hand (2nd and 5th PIF and MCF joints). There was no functional compromise, inflammatory signs or stiffness. The patient is otherwise healthy, without obsessive-compulsive behaviour or relevant family history. He does not practice activities requiring repetitive hand use.A 16 year-old caucasian male was sent to our department due to swelling of the lateral and dorsal regions of the metacarpophalangic (MCF) and interphalagic joints of the hands, with 3 years of progression. The swelling started on the 3On physical examination there was skin thickening in the above mentioned joints; remaining physical exam was normal.Blood analyses  and hand radiographs showed no anomalies. Articular ultrasonography revealed skin thickening around previously described joints, with no sinovitis, hydrarthrosis or bone changes.Skin biopsy showed epidermal acantosis with compact hiperkeratosis, thickening of dermis collagen fibers and increased interstitial mucin.Pachydermodactyly is a rare pathology that can be mistakenly diagnosed as Juvenile Idiopathic Arthritis or Knuckle Pads syndrome. Figure  and 2