This case describes an unusual presentation of an intracranial hemorrhage first thought to be metastatic disease on computed tomography and magnetic resonance imaging. The healthcare team completed an exhaustive search for a primary malignancy that was negative. Final diagnosis on brain biopsy showed intercranial hemorrhage secondary to cerebral amyloid angiopathy. With an increasing number of elderly patients and the rising cost of health care, this case can serve as a reminder to clinicians about their own responsibilities in limiting the cost of health care.This is a case report about a 72-year-old white woman with an intracranial hemorrhage secondary to cerebral amyloid angiopathy. The brain lesions on computed tomography/magnetic resonance imaging mimicked a metastatic process until a brain biopsy could give a definitive diagnosis that was completely unexpected. Cerebral amyloid angiopathy is a rare cause of intracerebral hemorrhage and this diagnosis is important to consider in older patients on anticoagulation.Cerebral amyloid angiopathy is a rare diagnosis but should be considered in elderly patients on anticoagulation presenting with imaging findings consistent with intracerebral hemorrhage. While metastatic disease is a more common cause of intracerebral hemorrhage, cerebral amyloid angiopathy should remain in the differential diagnosis. This case report serves as a teaching point to clinicians in cases involving an older patient on anticoagulation. Cerebral amyloid angiopathy (CAA) is characterized by amyloid deposition in cerebral blood vessels. This deposition consists of β-amyloid peptide in the media of small and medium-sized cerebral arteries. Marked deposition can lead to necrosis of the vessel walls and hemorrhage. It is commonly associated with Alzheimer’s disease (AD) but most patients with hemorrhage related to CAA do not have AD . It can A 72-year-old white woman with a medical history of recurrent pneumonia, pulmonary embolism 1 month ago (currently on warfarin therapy), and coronary artery disease presented from an independent living facility with altered mentation. She had no known family history of CAA or ICH and had not been brought into the Emergency Room (ER) for confusion previously. At baseline, she was very independent, living on her own, and was able to perform all her activities of daily living (ADL). Her family indicated that she started to have worsening altered mental status, to the point of being found profoundly confused by staff in the lobby of her living facility, prompting her urgent transfer to a local hospital by the Emergency Medical Service. On presentation, she was febrile to 39.9 °C and had right-sided deficits with repetitive speech. A computed tomography (CT) scan Fig. . FurtherAs the medical field has developed more advances and pharmaceutical options for hypertensive control, the expected decrease in the incidence of ICH has not followed. The overall incidence is actually stable, which could be secondary to orally administered anticoagulation-associated bleeds in our ageing population . WarfariOf interest, in this clinical scenario CT and MRI actually led the primary team toward chasing after the wrong underlying etiology of the ICH. The reports from radiology for both the CT and MRI stated that the lesions were most consistent with metastasis. After the primary team reviewed this with radiology, a complete and very expensive workup to find the primary lesion followed. This exhaustive workup creates a huge burden and contributes to the rising cost of health care. Although in this scenario it was appropriate to attempt to find primary malignant lesions, this case can be used as a reminder to keep in mind the possibility of ICH due to CAA especially in the presence of an older patient on anticoagulation. It is important to remember ICH is not always associated with hypertension or Alzheimer’s dementia.Another topic in this clinical scenario is whether to restart the orally administered anticoagulation. There is an elevated risk of CAA and ICH in the elderly, which is a given reason to hold anticoagulation. This is counterbalanced by a parallel increased risk of thromboembolic stroke with increasing age . The benCAA is a rare cause of intracerebral hemorrhageThis diagnosis is important to consider in older patients on anticoagulation presenting with ICHA lack of hypertension can be a clue to CAA causing ICHThe use of anticoagulants increases the risk of brain hemorrhage in patients with CAAWith the knowledge of this case report, clinicians should now be able to devise broader differential diagnoses in similar situations


Kasabach–Merritt syndrome represents the association of hemangioma with thrombocytopenia and consumptive coagulopathy. We present a case of Kasabach–Merritt syndrome treatment with orally administered propranolol.A 4.5-month-old caucasian female infant with congenital giant hemangioma in the posterior region of her neck presented to our Institute for Childhood Diseases where she underwent clinical, laboratory, and radiological investigations. A low blood platelet count indicated the use of corticosteroids and blood components as first-line therapy. The lack of therapeutic response induced the introduction of orally administered propranolol as additive therapy. A 3-week treatment led to a reduction in the size of hemangioma and a rise in platelet count which enabled surgical treatment and definite healing.Orally administered propranolol as monotherapy or in combination with other therapeutic modalities may play a key role in the treatment of Kasabach–Merritt syndrome. Kasabach–Merritt syndrome (KMS) shows wide variation in its response to different treatment modalities. Therefore, optimal therapy for this rare syndrome has not yet been established. Guidance for clinicians encountering patients with KMS is limited and because of the life-threatening and heterogeneous presentation of KMS, randomized controlled trials of these therapies, to yield evidence-based management protocols, are difficult to perform. Management strategies include use of corticosteroids, interferon, chemotherapy, propranolol, embolization, laser therapy, radiation and/or surgery. As the primary mechanism of platelet destruction is intravascular coagulation within the tumor, therapy should be aimed at controlling the coagulopathy by reducing the size of the tumor.Thrombocytopenia associated with giant hemangiomas was first described in 1940 by Kasabach and Merritt . EstimatWe report the successful treatment of a patient with KMS with propranolol, prednisolone, and surgical intervention, aiming to emphasize the importance and potential efficacy of propranolol in the treatment of KMS but also the challenges clinicians face in managing this rare and life-threatening syndrome.A 4.5-month-old caucasian female infant was presented in April 2016 to the Department of Hematology of the Institute for Childhood Diseases with an extremely low platelet count. The finding was a component of the evaluation prior to surgical treatment of the tumor on her neck, which seemed to be a congenital hemangioma., from third healthy pregnancy, born at term by cesarean section: birth weight (BW) 4260 gr, birth length (BL) 57 cm, head circumference (HC) 37 cm, and Appearance, Pulse, Grimace, Activity and Respiration (APGAR) score 9/9. She was fed on adapted milk formula from the beginning. Her family history is unremarkable.She was the third child of non-consanguineous parentsIn the first hours after birth, a tissue mass in the cervical thoracic part of her vertebral column, which looked like a hematoma, measuring 1.5 × 1.5 cm was detected. In the further course of treatment the mass was defined as a tumor, 2 × 2 cm in size, which seemed to be a hemangioma  were within the normal range prior to preoperative evaluation.On admission, the child was conscious, body mass (BM) 5900 gr, with adequate spontaneous and provoked activity, afebrile with a body temperature (BT) of 36.7 °C, and with normal respiratory and heart rate; she was acyanotic, anicteric, and had normal osteomuscular status. Her vital signs were in the normal ranges. In her neck, in suboccipital region and medially, existed a tumor of hemangioma type, size 6 × 6 cm, of firm consistency. The skin above the tumor was cyanotic, with petechiae, without signs of devitalization. Other physical findings were normal.9/l; red blood cells (RBC) 3.06 × 1012/l; hemoglobin (Hgb) 87 g/l; platelets 7 × 109/l; partial thromboplastin time (PTT) 12.6 seconds; international normalized ratio (INR) 0.98 seconds; activated partial thromboplastin time (APTT) 41.6 seconds; fibrinogen 1.0 g/l; and D-dimer 26.9 mg/ml.The values of some laboratory parameters were: white blood cells (WBC) 9.21 × 10Doppler ultrasound of the soft tissue of the posterior aspect of her neck revealed an extensive, soft tissue heterogeneous mass, predominantly hyperechogenic, with areas of calcification and enhanced color Doppler (CD) signal, which may correspond to a hemangioma.A magnetic resonance imaging (MRI) examination revealed cutis and subcutaneous tissue of the posterior aspect of her neck, and a soft tissue, irregular, lobular mass, size 58 × 53 × 35 mm , which was completely hyperintense on T2, with single isointense to hypointense areas and isointense signal on T1, compared to surrounding structures. It was a completely nonhomogeneous structure, clearly bounded, not propagating toward the spinal canal with intensive inhomogeneous contrast enhancement. In conclusion, on MRI the mass had characteristics of benign soft tissue mass , fibrinogen, and D-dimer. The re-planning of surgical management was also indicated.As a result of our patient’s low platelet count and laboratory signs of consumptive coagulopathy, substitution with fresh frozen plasma (FFP) 15 ml/kg, along with intravenously administered corticosteroid (methylprednisolone) 2 mg/kg, were carried out daily during 7 days. The laboratory parameters improved: platelets 30 × 109/l; RBC 3.2 × 1012/l; Hgb 90 g/l; platelets 9 × 109/l; PTT 13.5 seconds; INR 1.05 seconds; APTT 36.4 seconds; fibrinogen 2.1 g/l; and D-dimer 12.7 mg/ml. Urine culture was sterile, while Staphylococcus aureus was isolated from chemo-culture. A cardiologist was consulted. Her electrocardiogram and echocardiographic findings were normal.Six days after discharge, the child was readmitted. Her parents noticed enlargement of the tumor in her neck and laboratory tests revealed a low platelet count. Compared with previous clinical examination, the tumor was larger and was 8 × 8 cm in size. The values of some laboratory parameters were: WBC 9.5 × 109/l; RBC 4.2 × 1012/l; Hgb 112 g/l; platelets 25 × 109/l; PTT 13.5 seconds; INR 1.05 seconds; APTT 36.4 seconds; fibrinogen 2.4 g/l; and D-dimer 10.9 mg/ml.Intravenously administered corticosteroid therapy (methylprednisolone) 2 mg/kg, FFP 15 mg/kg, and symptomatic therapy were administered. Ten days after admission, a rise in her body temperature occurred. Her inflammatory parameters such as C-reactive protein (CRP) 6.8 mg/l were monitored and the symptomatic treatment was continued. The child also received RBC transfusion because of low Hgb values. The values of some laboratory parameters, 7 days after discontinuation of therapy, were: WBC 8.5 × 109/l to 100 × 109/l) within 2 weeks, along with an improvement of coagulation status and reduction of hemangioma size to 5 × 5 cm. After 30 days of hospitalization, she was transferred to Department of Pediatric Surgery for further treatment  was given postoperatively. The wound was cleaned and the dressing changed on a regular basis. The wound was clean and dry, and was healing by first intention. Sutures were removed on 7th postoperative day. The laboratory tests on the 3rd postoperative day showed normalization of CBC parameters and coagulation status.She was discharged in good general condition on the 7th postoperative day with follow-up appointments with hematologist every 7 days for the first month, and then once a month for up to 12 months, along with the results of laboratory tests, platelet counts, and coagulation status parameters, which were within physiological ranges , computed tomography (CT), and MRI reveal the size, appearance, and layers of hemangioma, as well as its relationships with peripheral blood vessels, and distinguish hemangioma from vascular malformations. In cases of unclear clinical presentation, along with etiologically unexplained thrombocytopenia, scintigraphy with chromium-51/indium-111 oxine-labeled platelets or iodine-131-labeled fibrinogen, increases sensitivity in making a diagnosis. KMS is associated with kaposiform hemangioendothelioma in over 90% of cases and uncommonly with infantile (cavernous or capillary) or congenital hemangioma [The management of KMS is very challenging because of its rarity and unstandardized therapeutic strategies. Systemic corticosteroids are considered to be first-line therapy for this syndrome . If the Since the first report in 2008, propranolol has showed many advantages in the treatment of infantile hemangioma .There are still controversies regarding the effect of propranolol on KMS. There are only a few literature reviews on good , 11 and 9/L), at a dose of 2 mg/kg per day, divided into three doses [Choeyprasert and associates suggested that propranolol as monotherapy may be useful in mild KMS (initially small size of the primary tumor, 5 × 5 cm; and the mild severity of coagulopathy, platelets 55 × 10ee doses . This isee doses . It was ee doses .The size of hemangioma in our case is in line with the conclusions found in the literature and referred to above, but that is not the case with low platelet count. Despite the severity of thrombocytopenia, our patient had an excellent response to the treatment which was not expected according to the above mentioned data from the literature. Therefore, our case report suggests that propranolol therapy should be taken into consideration regardless of significant thrombocytopenia.Dhandore and associates reported inefficiency of steroids and propranolol in the treatment of KMS: a 24-hour-old full term male child with giant hemangioma over right thigh since birth along with thrombocytopenia and coagulopathy. Vincristine and interferon were not taken into consideration due to the child’s clinical condition. An emergency surgical excision was performed to prevent life-threatening multiorgan hemorrhage .This case report emphasizes the importance and potential efficacy of propranolol in the treatment of KMS. An excellent treatment response enabled surgical intervention and definite management of tumor, along with normalization of platelet count and coagulation status. Considering the fact that the treatment of KMS is still empirical, it is extremely important to publish individual experiences in management of this rare and life-threatening syndrome.


Hemangiomas are common benign tumors of the liver. Spontaneous rupture is a rare complication, occurring most commonly in giant hemangiomas. Rupture of a hemangioma with hemoperitoneum is a serious development and can be fatal if not managed promptly.The present study reports the unique case of a man who experienced rupture and hemorrhage of a hepatic hemangioma (HH) due to perforation of the gallbladder fundus. After en block resection of the hemangioma and gallbladder using the Pringle maneuver, the patient made an uneventful recovery without complications.To our knowledge, spontaneous rupture of HH secondary to gallbladder perforation has not been reported in the literature. This case highlights a unique, rare cause of ruptured HH and the need to consider appropriate treatment for some hemangiomas to avoid this potentially fatal complication.The current case may provide additional support for treatment of HH due to the potential for spontaneous rupture. For patients with ruptured HH, enucleation with the Pringle maneuver is recommended. Spontaneous rupture of HH is considered a life-threatening situation. Thus, appropriate treatment of HH must be determined to avoid this complication. We presented an extremely uncommon case of spontaneous rupture of HH where perforation of the gallbladder caused the tumor capsule to tear. En block resection of the hemangioma and gallbladder was performed using the Pringle maneuver. The patient recovered without complications.Hemangioma is the most common solid benign tumor of the liver.2This study was approved by the Institutional Review Board of The First Affiliated Hospital, College of Medicine, Zhejiang University. Informed consent was obtained from the patient for publication of this case report.A 44-year-old man presented to the emergency department with severe right upper quadrant abdominal pain for 1 hour. There was no history of trauma. The onset of pain was sudden, and it was persistent in nature and worsened with movement. He denied nausea or other gastrointestinal symptoms.Medical history included HH and gallbladder stones without any symptoms that were detected incidentally during an ultrasound examination 1 year prior. The patient reported no significant family history, alcohol intake, or smoking history.9/L with 84.5% segmented neutrophils (machine count), and platelet count of 155 × 109/L.On examination, he was found to be tachycardic (112b/min) with upper abdominal peritonism. His blood pressure was 95/63 mm Hg. Initial blood tests showed a hemoglobin of 81 g/L, white cell count of 7.86 × 10Urgent computed tomography (CT) of the abdomen with intravenous contrast demonstrated a giant HH at segment V and segment VI and free high attenuation fluid around the liver and spleen Fig. . The patThe patient was taken emergently to laparotomy, which was performed via an incision of the right mid-upper abdominal line. A large quantity of fresh and clotted blood was found around the liver and in the abdominal cavity. The fundus and body of the gallbladder completely overlaid the surface of the hemangioma. Gallbladder perforation at the fundus was identified, which caused a tear in the capsule of the hemangioma; this was the main source of active bleeding. En block resection of the hemangioma (enucleation) and gallbladder was performed using the Pringle maneuver with 12 minutes of ischemia time. The patient was transferred to the intensive care unit for 24 hours, where he received 9 units of red blood cell transfusions.After transfer back to the ward, he made an uneventful recovery and was discharged home on postoperative day 8 with no complications. His postoperative histological findings revealed hepatic cavernous hemangioma and chronic cholecystitis with gallbladder perforation Fig. .3 occurring mostly in giant HH. Pietrabissa et al stated that the spontaneous rupture of HH is an exceptional phenomenon. Although rare, it is associated with a high mortality rate. Jain et al reported a mortality rate ranging from 60% to 75% for spontaneous rupture with an operative mortality rate of 36.4%. Patients can die of massive hemorrhage in a short period of time. Thus it is challenging to appropriately treat HH rupture, because it is an emergent life-threatening situation. Spontaneous rupture of HH is often considered to be related to the size of the lesions. As the size of the hemangioma increases, so does the chance of rupture, especially if the HH is located on the surface of the liver. Yamagata et al concluded that lesions exceeding 10 cm in diameter may have greater internal bleeding and further growth or rupture. If the patient receives steroid therapy, the chance of rupture increases further.Hemangiomas are common benign tumors of the liver, generally detected incidentally during radiographic studies performed for other reasons. Based on the literature, it is clear that spontaneous rupture is a rare complication of HH. The rate of spontaneous rupture ranges from 1% to 4%, Factors contributing to spontaneous gallbladder perforation in the absence of trauma include acute cholecystitis, infection, or malignancy. The fundus is the most common site for perforation to occur. The combination of spontaneous HH rupture secondary to gallbladder perforation has not been reported in the literature, highlighting the unusual nature of this case.Gallbladder perforation is a rare complication of gallstone disease.Prior to laparotomy, we failed to diagnose the gallbladder perforation. After CT demonstrated a giant HH and free high attenuation fluid around the liver, spontaneous rupture of HH was quickly diagnosed. Indeed, we ignored some suspicious findings indicating gallbladder perforation that were present on the preoperative CT. Thrombi in the gallbladder and contrast extravasation from the hemangioma to the gallbladder Fig.  provided Ruptured HH leads to excessive hemorrhage and blood loss. Bleeding from the ruptured site is extremely difficult to stop. In case of hemodynamically unstable patients with spontaneous rupture of HH, enucleation is the preferred operation and can be performed rapidly and safely, with a potentially lower operative morbidity. The packing management technique for ruptured HH has a low rate of success and a high failure rate, with rebleeding and high perioperative mortality. Enucleation with temporary inflow occlusion (Pringle maneuver) is the first choice, and is associated with fewer postoperative complications and reduced blood loss. In some cases, partial resection is a safe choice that saves lives in urgent situations where HH involves large vascular structures of the liver. Recent studies have emphasized the role of transcatheter arterial embolization (TAE) as an effective treatment of HH. However, the use of TAE as an alternative to surgery in the management of ruptured HHs remains controversial.,14,16 If the patient is stable, TAE prior to surgery may decrease intraoperative blood loss. In hemodynamically unstable patients with spontaneous rupture of HH, operative treatment is the preferred choice.Rupture of HH with hemoperitoneum is a serious situation and often fatal if not promptly managed; thus, surgical intervention should be considered as soon as possible. Particular attention should be given to patients with unique clinical situations, as in the currently reported case.Generally, giant HH (primary diameter ≥4 cm), when peripherally located and exophytic, should be considered for treatment due to its risk of rupture.In conclusion, the current case may provide additional support for treatment of HH due to the potential for spontaneous rupture. For patients with ruptured HH, enucleation with the Pringle maneuver is recommended to achieve both hemostasis and hemangioma resection.


Hernias of the posterior rectus sheath are very rare abdominal wall hernias with only a handful of cases reported in the literature to date. As an uncommon disease, it is important to recognize and report this case in order to enhance scientific knowledge of this disease.Salmonella-related gastroenteritis. A computed tomography scan of his abdomen and pelvis showed abdominal wall hernia with loops of small bowel extending into his rectus abdominis muscle. In this case, it was decided to leave the situation alone for now due to no evidence of bowel obstruction and the low risk of this hernia getting strangulated, which otherwise would have warranted urgent surgery.This case report presents a spontaneous posterior rectus sheath herniation in a 79-year-old white man with previous abdominal surgery for appendicitis. His herniation was discovered incidentally during an examination for his chief complaints of lower abdominal pain and diarrhea which were later diagnosed as This report adds to the limited stock of available literature on this unusual issue and strengthens the evidence base on the best approach to support informed clinical decision making. The significant clinical implication of such case reports is increased identification rate of rare clinical conditions which otherwise often go unnoticed. Hernias are an abnormal protrusion of an organ or tissue through a defect in its surrounding walls. A hernia can be congenital or acquired and the common types of hernia include inguinal, femoral, ventral, and incisional. Less common hernias include interparietal, Richter, and Littre hernias of the abdominal wall and sciatic, obturator, and perineal hernias in the pelvis .Posterior rectus sheath hernias are a type of interparietal hernias where the hernial sac lies between the various layers of the abdominal wall muscles and does not exit into the subcutaneous tissue , 2. The This case report presents a summary of the case of a patient who presented with posterior rectus sheath hernia and discusses presenting features, diagnosis, and management in light of the current evidence. An informed consent has been obtained from the patient to present this case.9/L), slightly low red blood cell count (3.12 × 1012/L), and slightly raised level of creatinine (124 μmol/L). His medical history included recent completion of radiotherapy treatment for a Gleason score 9 prostate adenocarcinoma, previous cerebrovascular event, asbestosis, atrial fibrillation, and chronic lymphoid leukemia. His abdominal surgical history included only an open appendectomy 40 years prior. He is a retired marine engineer and lives with his wife. He reported that he did not drink alcohol and had quit tobacco smoking 30 years ago. His family history is noncontributory.A 79-year-old white man arrived by ambulance to our emergency department with chief complaints of lower abdominal pain and profuse diarrhea. On examination, general signs of poor health were observed. His blood pressure was 150/60 mmHg and pulse rate 90 beats per minute (bpm). His temperature was 37.3 °C and he did not appear to be in distress. A physical examination did not reveal any bowel obstruction. His laboratory findings were within normal range except for an elevated white blood cell count (14.5 × 10Computed tomography (CT) of his abdomen and pelvis was performed and demonstrated a peritoneal recess containing multiple small bowel loops over the anterior side of the intraperitoneum lying between peritoneal lining and the transverse abdominis fascia consistent with a pseudoherniation of a preperitoneal subtype Fig. . There wSalmonella and our patient improved on antibiotics with resolution of abdominal pain. He was discharged and followed up 2 weeks later at out-patient clinic and had no abdominal discomfort; therefore, no elective hernia repair was planned and further follow-up was not indicated.In view of CT abdomen findings, a surgical opinion was sought. As there was no evidence of bowel compromise, he was managed non-operatively. Fecal polymerase chain reaction (PCR) tested positive for Spontaneous posterior rectus sheath hernias were first documented in 1937. They are exceedingly rare with a case series in 2009 identifying only eight cases in the literature with two additional cases reported in 2014 and 2017 . Among tTo date, the pathophysiology of posterior rectus sheath hernia is not well understood , 6. ExamA majority of posterior rectus sheath hernia cases are post-surgical , 10 or pPosterior rectus sheath hernia is usually diagnosed with imaging studies such as ultrasonography, magnetic resonance imaging (MRI), and CT. Out of these, CT is often considered the best diagnostic modality due to its higher sensitivity to highlight complications within the hernia sac such as obstruction, incarceration, and strangulation . LaparosIn our case, posterior rectus sheath hernia was an incidental diagnosis with no significant clinical course and was thus managed conservatively. Posterior rectus sheath hernias are uncommon and they may present with severe abdominal pain and incarceration, which can lead to an increased risk of bowel obstruction and strangulation if left untreated. Prompt diagnosis with or without surgery is needed for a successful outcome. This case report adds to the limited stock of available literature on this unusual issue and strengthens the evidence base on the best approach to support informed clinical decision making. The significant clinical implication of such case reports is increased identification rate of rare clinical conditions which otherwise often go unnoticed.


The keratocystic odontogenic tumor is a benign odontogenic cystic neoplasia characterized by its thin, squamous epithelium with superficial parakeratosis. It has the potential for infiltration and local aggressiveness and has a high rate of recurrence. This neoplasia is predominantly found in males and people of white origin. The mandible is the most frequently involved site, in particular the third molar region, mandibular angle, and ramus. It has a mandible-maxilla ratio of 2:1. Only about twenty cases of peripheral keratocystic odontogenic tumors (PKCOT) have been reported in the international literature. This study presents a case of PKCOT localized in the anterior region of the maxilla, on the vestibular side of the upper left lateral incisor and the upper left canine. The diagnosis and treatment procedures, as based on the literature, are also discussed. Key words:Odontogenic cysts, odontogenic tumors, keratocyst, keratocystic odontogenic tumor. The keratocystic odontogenic tumor (KCOT) is a benign odontogenic cystic neoplasia characterized by its thin, squamous epithelium with superficial parakeratosis. It has the potential for infiltration and local aggressiveness and has a high rate of recurrence -3.This neoplasia is predominantly found in males and people of white origin. It occurs mainly in the mandible, in particular the third molar region, mandibular angle, and ramus, with a mandible-maxilla ratio of 2:1.1 It can appear at any age; however, it is more frequent between the ages of 20 and 30. Its incidence rate ranges from 3 to 12% of odontogenic tumors . Similaret al. (Philipsen coined the term “odontogenic keratocystic” (OKC) for the first time in 1956 . The hiset al. . On palpation, the lump was fluctuant and not painful Fig. . Teeth 2Clinical and radiological aspects led to a presumptive diagnosis of non-inflammatory odontogenic cyst causing cortical perforation in the maxilla, which was also consistent with a gingival cyst of adult and keratocyst odontogenic tumor.A cystectomy was performed under local anesthesia without conducting a root canal treatment of adjacent teeth, and the obtained material was sent for histopathologic testing.A full-thickness incision was made, preserving the papilla to avoid future gum recession (papilla-base incision), beginning on the mesial part of 22 and ending on the distal part of 23. This incision continues with a vertical incision on the distal area of 23, passing the deformity, and penetrating the mucogingival junction to have better visibility and access. A full-thickness flap was carefully elevated to avoid tearing the flap or the cyst capsule. Once localized, the cyst was removed via curettage of the bone tissue.After removal, the cyst was placed in 10% formalin and was sent to a pathological anatomy lab for histologic diagnosis. Subsequently, the flap was repositioned using a 6/0 non-absorbable suture.The patient was prescribed antibiotic treatment (one tablet of amoxicillin/clavulanic acid 875mg/125mg every 8 hours for 7 days), as well as nonsteroidal anti-inflammatory drugs . The patient was told not to brush in areas near the incision for the first 24 hours, and to use mouthwash with 0.12% chlorhexidine twice a day for two weeks. In addition, the patient was prescribed a soft, cold diet to avoid heavy chewing for at least two or three days. The stitches were removed two weeks later.The histopathological findings revealed a cystic lesion with a loose connective tissue wall, an inner lining of well-defined simple squamous epithelium (4-8 cells in thickness), basal cells arranged in a palisaded pattern with a superficial parakeratinized focally corrugated surface. The epithelium was focally detached from the connective tissue, and keratin was found inside the cystic le-sion. The collected data established a peripheral keratocyst odontogenic tumor as the diagnosis Fig. .Periodic check-ups were carried out after one month, three months, six months and one year post-surgery, as well as a CBCT, which confirmed no signs of recurrence Fig. . The patRegarding the case’s clinical presentation, the cyst was surrounded by a smooth contour similar to a gingival cyst of adult, while the contour of the peripheral keratocyst odontogenic tumor (PKCOT) is usually irregular and undulating. The histological examination was successful, as there was evidence of “keratin scales.” In the majority of cases, this is very difficult to achieve. The lesion in this study revealed the histological characteristics of a PKCOT, including parakeratinization -21.In the systematic review carried out in PubMed using the keywords “peripheral,” “keratocystic,” “odontogenic,” and “tumor,” only 22 cases of PKCOT were found, seventeen of which were localized on the gum tissue (77%), three on the oral mucosa (14%), and two in the lateral facial deep region (9%) .Regarding the tumor’s location, the proportion of maxillary KCOT, with respect to those occurred in the mandible, is 1 to 2 or 1 to 3. The lesion is localized in the maxillary tuberosity in only 10% of cases, and with an even lower percentage in the canine area . PKCOT iIn addition, the tumor’s specific characteristics must be emphasized. Even though it was of relatively small size (approximately 4 mm), the tumor had perforated the vestibular cortical area. The size of PKCOTs can vary, but the most common dimensions are between 3 and 5 mm, and they are rarely larger, reaching even 3 to 4 cm . The linet al. (Controversy exists over whether PKCOT is a locally destructive lesion with a high rate of recurrence like KCOT, or whether it is an indolent lesion more similar to gingival cyst of adult -13. Ide et al. ,13 affirThe question of whether or not the PKCOT is a counterpart of KCOT must be answered through exhaustive examination on a case-by-case basis. The PKCOT of the patient in this study seems to be a true expression of a KCOT in soft tissue ,5,8.In the cases linked to Gorlin-Goltz syndrome, PTCH1 mutations were identified, and the immunohistochemical results suggested that the KCOT and PKCOT lesions were caused by a genetic alteration in the patients’ PTCH1-GLI gene . As all Currently, there is no consensus on KCOT treatment due to its high rate of recurrence. As the literature shows, treatment of this tumor can range from marsupialization to a resection in-bloc, from most conservative to most aggressive treatments, respectively ,7,10.PKCOT cannot be completely compared to KCOT, as the clinical and biological behavior is not necessarily the same for both. Therefore, choice of treatment will depend on the age of the patient, the location and size of the tumor, and whether it is a primary or recurrent tumor. These factors will justify the differences found in the scientific literature .In the present clinical case, a complete exeresis of the lesion was conducted with posterior curettage and a slight bone drill, thus avoiding the possibility of any remains of the lesions remaining in the affected area. To date, the patient presents no signs and symptoms of recurrence after a year.


Orbital metastasis from lung cancer as an initial presenting symptom is a rare entity, which may paradoxically delay the diagnosis and initiation of correct management, due to the confusion of it being primary orbital pathology. Herein we report a case of a 58 year old woman, who presented with painful orbital swelling along with diminution in her vision. The patient was initially thought to have a primary eye lesion; however chest X-ray was suggestive of a lung mass, which was confirmed by chest computed topography followed by ultrasound guided fine needle aspiration cytology. The patient was then referred to a cancer centre for further management. This case report aims to increase the knowledge about this metastasis as a probable cause of orbital symptoms in certain subsets of patients, so that correct therapeutic decisions may be made in the future. However, this should be kept as one of the differentials in any patients presenting with orbital symptoms, so as to frame an accurate and effective plan of management. Occasionally such rare presentations would invariably lead to a delay in the correct diagnosis, thereby increasing the risk of loss of vision, which decreases the quality of life of patients3. Poor management also increases the odds of progressing the tumor stage. Herein, we report one such case in a 58 year old woman, who presented with unilateral peri-orbital swelling and diminution of vision. Following detailed examination and investigations, the patient was found to harbor a malignant lung lesion.Orbital metastatis as the initial presenting symptom from a metastatic lung lesion is a rare entity, occurring at an incidence of approximately 7%A 58 year old woman from central Nepal presented to our outpatient clinic with a history of painful swelling around her right eye for two months. The patient also complained of diminishing vision in the same eye. The vision in the patient’s left eye had been previously lost following an injury during childhood. There was no other relevant family information or any significant past medical or surgical illnesses of the patient. Local examination revealed presence of peri-orbital swelling in the right eye with restricted eye movements . The patRadio-imaging of the patient’s orbits revealed the presence of hyperostotic changes in theright orbit, with presence of enhancing lesions on the right globe with extension to the para-nasal sinuses and also invasion along the dural base in the anterior cranial fossa . The iniA chest X-ray was performed as a routine work up, which inadvertently revealed the presence of an elevated right hemi-diaphragm with presence of right para-hilar mass . FurtherWe discussed with the patient and her relatives the possibility of the eye findings to be related to the lung lesion and recommended approaches to obtain a definitive diagnosis. Ultrasound guided fine needle aspiration cytology (FNAC) from the lung lesion revealed findings suggestive of a malignant lung disease . DiagnosThe patient was started on a steroid therapy (injection dexamethasone at 8mg stat followed by 4 mg every eight hours), which decreased the swelling on the patient’s eye and improved visual acuity to finger counting within a period of 1 week. This further hinted at compressive rather than infiltrative effect on the optic nerve by the lesion. The patient was counseled and then immediately referred to the National Cancer Centre, Kathmandu, Nepal for further management with systemic chemo-radiation therapy after evaluation. Since the patient had a single and minimally functioning eye left, the decision was taken not to surgically decompress the lesion from the orbit. The patients was initially started on chemotherapy with a further plan of management to be tailored as per the clinical response seen in the patient.Initially, metastatic deposits causing eye swelling in the patient was not suspected. It was serendipity that the routine chest X-ray gave a clue to the presence of a lung mass. Even a small delay may have had a disastrous impact on the outcome of the vision in the patient.2. Of these, symptoms related to orbital metastasis presents earlier to that of the primary lesion in around 20% of patients2. Breast, prostate and lung carcinomas are the usual primaries in many cases of metastatic lesions to the orbit5. Lid swelling are a common presentation in such metastatic lesions5, which can paradoxically delay the actual diagnosisaccounting for the benign orbital lesions. Diplopia is the most common presenting symptom in metastatic lesions, while proptosis or visual loss is seen in patients with primary orbital neoplasms6. Loss of vision can be due to either direct infiltration to the optic nerve or subsequent to the mass effect. Rarely, is it subsequent to paraneoplastic phenomenon mainly from lung carcinoma. Pain resulting from perineural invasion is typical for metastatic orbital lesions6.Metastatic disease to the orbit is a rare epiphenomenon occurring in only 7% of all cancers7. Further investigations need to be carried out to stage the tumor before embarking on the management option; PET scan is a rapid viable model for assessment tumor staging8.Diagnosis can be confirmed with FNAB, which has a diagnostic accuracy of more than 90%6. However, chemo-radiation is usually preferred to surgery because it is non-invasive. Chemotherapy, especially platinum base regimes, is chosen for small cell lung cancer over radiation because of the risk of damage to the eye lens. For non-small cell cancers, either photon radiation of 30–40 Gy, or newer frontiers, such as tyrosine kinase inhibitors, are the mainstay of treatment. However, overall prognosis, despite systemic therapy, is poor with a median survival of little over 1 year, and only 27% of patients surviving for more than two years13. Compared to breast cancer, lung cancers metastasize early to the orbit and also have shorter median survival time14.Surgical debulking is the cornerstone of management in patients with diminished vision subsequent to optic nerve compression. This was not attempted in our case, since it was the only functioning eye in the patient and that was functionally impaired as well. Surgical removal of the lesion may be locally effective in few patients having symptoms, due to compression on the optic nerve following raised intra –orbital pressureIt is prudent to provide a strategy for management of cases presenting with eye symptoms, so that rare causes, such as metastatic lesions, are not omitted. Such a strategy would certainly help in providing an early and effective treatment plan in such patients with metastatic orbital lesions. This would increase the chance of improving vision, escalate quality of life and also initiate early cancer therapy following appropriate work up and staging.Both written and verbal informed consent for publication of images and clinical data related to this case was sought and obtained from the patient. An interesting case of orbital metastasis of lung adenocarcinoma is presented. Lung cancer is one of the leading causes of death globally. Adenocarcinoma occupies the first place in epidemiological frequency (50%) is also one of the most frequent types of tumors in non-smokers. They are classified into 4 histological types: acinar, papillary, bronchialveolar and the mucin secretory variety. The adenocarcinoma originates from mucoproductive cells. Adenocarcinoma metastasis to the orbit is infrequent. In the orbits most of the tumors are primary, but they can also reach the orbit by contiguity. At least 50% of patients with orbital metastasis are unaware of the existence of a primary tumor. Metastasis is less frequent than ocular metastasis. Apparently, there is no predilection for any specific orbit and their bilateral appearance is rare. The most frequent orbital metastasis are breast, lung and orbit. They have been described a 5 types of clinical syndromes associated with orbital metastasis. The first type is mass syndromes. In more than 50% of cases. This syndrome causes displacement of the eyeball. The second type is infiltrative. The third type is an inflammatory type. The fourth type of metastasis is called functional and is frequently located in apex orbital. The last type is silent, does not produce symptomatology. The use of fine needle aspiration biopsy is an excellent option when orbital metastasis is suspected. Finally, the patients with orbital metastasis are not candidates for orbit surgery for extirpation of the tumor mass. The realization of surgery does not offer a cure. In cases of slow-growing tumors, the extirpation of metastasis and the primary tumor may improve the prognosis. Management strategies include radiation therapy. Sometimes the use of radiation therapy results in vision recovery.We have read this submission. We believe that we have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. A major deficiency of the report is the lack of characterization of the tumour. Whilst the CT imaging is convincing for a lung primary it should at the very least be commented whether the tumour was non-small cell  or small cell -the H&E slide is suspicous of the latter, although I am not a pathologist. Ideally it would have been established if it were TTF1 positive supporting a lung origin. I am surprised that the smoking status of the patient is not reported. The sentence: "Orbital metastatis as the initial presenting symptom from a metastatic lung lesion is a rare entity, occurring at an incidence of approximately 7%" - the denominator 7% of what? - I suspect orbital tumours. Metastasis spelt incorrectly The sentence: "Poor management also increases the odds of progressing the tumor stage." is incorrect: the disease stage is already IV. What I think they mean is that patients may become too unwell for anticancer treatments and the visual impairment worse and harder to palliate. Funduscopy should be fundoscopy "PET scan is a rapid viable model for assessment tumor staging8" PET scanning would only be considered if simpler imaging modalities failed to identify a primary site, as in a lung cancer, staging is already M1b I remain troubled by the 7% figure which is repeated in the discussion. If orbital metastasis really occurred in 7% of all cancers it would not be regarded as that rare. Reference 2 does not give any evidence for the 7% figure quoted, does not give the denominator (either) and I can't see any evidence for the 7% figure quoted in reference 1. The outcome for the patient is not described with sufficient detail, including length of survival.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above. This is an interesting case report, about an unusual site of metastasis in lung cancer patient. There are details about the symptoms, diagnosis and symptomatic treatment of the orbital metastasis.  I would only add if there was a history of tobacco exposure (not cited in the text).I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.


Mitotane  is established in the adjuvant and advanced-stage treatment of adrenocortical carcinoma and counteracts both tumor growth and tumor-related steroid production. Both the adrenal glands and the gonads are steroidogenically active organs and share a common embryogenic origin. Here, we describe the effects of mitotane in two patients with metastatic Leydig cell tumor (LCT) of the testes and associated severe androgen excess . Both men suffered from severe restlessness, insomnia and irritability, which they described as intolerable and disrupting normal life activities. Urinary steroid profiling by gas chromatography–mass spectrometry (GC–MS) confirmed excess androgen production and revealed concurrent overproduction of glucocorticoids and glucocorticoid precursors, which under physiological conditions are produced only by the adrenal glands but not by the gonads. In a palliative approach, they were commenced on mitotane, which achieved swift control of the hormone excess and the debilitating clinical symptoms, restoring normal quality of life. GC–MS demonstrated normalization of steroid production and decreased 5α-reductase activity, resulting in decreased androgen activation, and imaging demonstrated disease stabilization for 4–10 months. In conclusion, mitotane can be highly effective in controlling steroid excess in metastatic LCTs, with anti-tumor activity in some cases. Testicular Leydig cell tumors (LCTs) are rare stromal tumors, comprising 1–3% of all testicular neoplasms . LCTs reDuring human foetal development, gonads and adrenal glands both derive from the urogenital ridge and after separation they develop distinct steroidogenic features, with gonadal sex steroid production and adrenal production of glucocorticoids, mineralocorticoids and adrenal androgen precursors. Mitotane  is routinely used in the treatment of adrenocortical cancer, where it has been shown to control adrenal steroid excess and, to a degree, tumor proliferation . MitotanUrinary steroid metabolome profiling at baseline and during mitotane treatment was carried out by gas chromatography–mass spectrometry, utilizing selected-ion-monitoring analysis for identification and quantification of 32 distinct steroid metabolites reflective of 24-h net steroid output, as previously described . Serum sWe carried out immunohistochemistry for sterol-O-acyl transferase 1 (SOAT1) as described previously , using aSupplementary Table 1, see section on Supplementary Fig. 1). Alongside the decrease in androgens, the patient reported a significant improvement of his previously debilitating clinical signs and symptoms. He returned to full-time work and enjoyed good quality of life. After 10 months of mitotane treatment, he died suddenly of a suspected myocardial infarction; no post-mortem examination was carried out.A 51-year-old patient presented with severe restlessness, insomnia, impaired concentration, increased aggressiveness, redness of the face and body hair growth, all gradually developing over the last six months. Fifteen years previously, he had undergone an orchidectomy for LCT, and thirteen years later, excision of a retroperitoneal mass, confirmed on histology as LCT metastasis. Imaging revealed multiple lesions consistent with liver, lung and retroperitoneal metastases. Immunohistochemistry of a tissue biopsy confirmed vimentin-positive, inhibin-negative metastatic LCT. Serum testosterone was very high at 93 nmol/L . Urinary steroid profiling by gas chromatography–mass spectrometry (GC–MS) showed increased androgen metabolite excretion  as well as increased excretion of DHEA, metabolites of pregnenolone, progesterone, 17-hydroxypregnenolone, 17-hydroxyprogesterone and cortisol  . PrognosSupplementary Table 1)). Mitotane decreased serum androgen production within four weeks. Six months after treatment initiation, plasma testosterone had decreased to 29.1 nmol/L and oestradiol to 177 pmol/L, while androstenedione and DHEAS had normalized. Urinary steroid profiling 4 months after initiation of miotane showed a decline in all previously raised steroid metabolites and decreased 5α-reductase activity. This was paralleled by significant clinical improvement in signs and symptoms, specifically reduced restlessness, aggressiveness and insomnia. Imaging four months after initiation of mitotane revealed a mixed response, with regression of some previous lesions, but emergence of new metastatic deposits in lung and abdomen. The patient passed away 12 months after his second recurrence, i.e. six months after the start of mitotane treatment.A previously fit-and-well 59-year-old man presented with a right testicular mass and underwent orchidectomy; histopathology revealed malignant LCT. Three years later, he presented with lower back pain, and imaging showed a large retroperitoneal mass, confirmed as disease recurrence by transcutaneous biopsy. He underwent laparoscopic removal of the mass together with retroperitoneal lymph node dissection. One year later, follow-up imaging revealed disseminated metastases, including liver, kidney and peritoneal deposits. He was unwell, with agitation, anxiety and insomnia. Biochemical work-up showed increased serum testosterone , oestradiol , androstenedione  and DHEAS . GC–MS profiling showed increased steroid excretion including androgen metabolites  and cortisol  . He rejeHere, we used mitotane, an established drug in adrenocortical carcinoma, in two patients with metastatic testicular LCT associated with severe androgen excess, clinically manifesting with severe restlessness, insomnia, irritability and impaired concentration. Both patients experienced significant improvement in signs and symptoms with mitotane therapy, swift normalization of steroid excess and some stabilization of radiologically quantified tumor load.In a comprehensive PubMed search , we identified eight cases of LCT treated with mitotane . Four paDuring human fetal development, adrenals and gonads both arise from the urogenital ridge and they both develop steroidogenic capacity, albeit with distinct features, i.e. sex steroid synthesis in the gonads and glucocorticoid, mineralocorticoid and androgen precursor synthesis in the adrenal glands. Benign testicular adrenal rest tumors, which are regularly found in men with congenital adrenal hyperplasia, have been shown to display features of both adrenal and gonadal steroidogenesis , 22. UsiRecent studies have implicated sterol-A-acyl transferase 1 (SOAT1), previously also termed ACAT-1 for acyl-coenzyme A cholesterol acyltransferase, as a target of mitotane action . SOAT1 iBased on our current findings, the use of mitotane in the palliative treatment of metastatic LCTs of the testes appears feasible and useful, with effective control of tumor-related steroid excess and possible beneficial effects on disease progression, a viable treatment option in a rare endocrine cancer that is not responsive to cytotoxic chemotherapy or radiotherapy.The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of this case report.This work was supported by the European Union under the 7th Framework Program , the Wellcome Trust  and the Mayo Foundation for Medical Education and Research .


Background: Duplication cyst is a rare cause of neonatal intestinal obstruction. Their most common location is the small intestine. The clinical presentation is extremely variable depending upon its size, location and type and the age of the patient and are mainly encountered during infancy or early childhood. The diagnosis is very difficult in neonates. This study was undertaken to study their presentation, diagnostic modality of choice and further management in neonatal age group. Materials and Methods: This was a retrospective study performed at the Department of Paediatric Surgery, J .N Medical College Hospital, AMU Aligarh from July 2008 to June 2014. The data was analyzed with respect to demographic profile of the neonates, their initial clinical presentation, radiological features and subsequent event leading to intervention, operative features and outcome.Results: There were a total of seven neonates between ages of 3 days and 21 days who were diagnosed as cases of intestinal obstruction due to duplication cyst. The majority of the patients were having ileal duplication cyst (n=4). Ultrasonography played important role in majority of the cases for diagnosis. There was one patient in which the diagnosis was confused with ileal atresia. All the patients underwent excision with restoration of bowel continuity.Conclusion: The diagnosis of intestinal obstruction in neonate due to duplication cyst is difficult. It has varied presentation and preoperative diagnosis at times may be challenging. Surgery is the mainstay of the treatment. Duplication cysts are rare congenital anomalies of gastrointestinal tract (GIT) causing various symptoms which are nonspecific leading to difficulty in management. They are most commonly observed in the small bowel but they can be present in any part of GIT. It may present at any age but the most common age is childhood. The most common presentation is intestinal obstruction. It may also present as perforation and gastrointestinal bleeding which are mostly seen as its complications. We have done a retrospective study about duplications cysts presented with neonatal intestinal obstruction.We performed a retrospective analysis of the data available in the unit of Paediatric Surgery, J.N. Medical College Hospital, AMU, India. We studied the medical record file of the babies who presented as intestinal obstruction in neonatal period from July 2008 to June 2014. Out of these patients we found seven cases of neonatal obstruction whose final diagnosis was duplication cyst. In these patients we studied the demographic profile, initial clinical presentation, complication or event leading to surgery, radiological investigations, operative findings and outcome.Demography: There were a total of seven cases of neonates who were diagnosed as cases of intestinal obstruction due to duplication cyst or its complications during the study period. There was male preponderance and the male female ratio was 5:2. Most of the babies presented during first week of life (n=4). Table 1 depicts the demographic profile and other parameters studied in the patients. Presentation: The most common presentation was abdominal distension associated with vomiting. It was noted that three patients had complaints suggestive of incomplete obstruction. One patient (Patient no.5) presented with incomplete obstruction which on conservative treatment showed improvement on first day but later on developed bleeding inside the cyst which became evident due to increasing abdominal distension and associated pallor demanding urgent surgical exploration. Investigations: All the patients underwent routine biochemical and specific investigations. Plain X-ray abdomen was done in all the patients as preliminary investigation. This was suggestive of multiple air-fluid level in all except two patients. Ultrasonography was suggestive of some cystic pathology in all except one patient. There was one patient in whom antenatal diagnosis was suggested by ultrasonography. Management: All the babies underwent exploratory laparotomy with cyst excision and restoration of bowel continuity by end to end anastomosis. In four patients bowel compression leading to obstruction was the reason for laparotomy. While one patient each had volvulus, intracystic bleed and perforation as the reason for exploration. The most common region affected was ileum (n=4). There was one patient each of pyloro-duodenal and jejunal duplication cyst (Table 1). In all seven cases it was the cystic variant which was found and measured from 3 to 10 cm in size. All cysts were lined by normal bowel mucosa except one which has additionally ectopic gastric tissue in it. Outcome: One patient died in the postoperative period because of septicemia. This patient had pyloro-duodenal duplication cyst. Four patients had uneventful recovery while two patients had delayed recovery due to wound and abdominal sepsis.Follow up: All six patients were healthy in their first follow up visit within 4 weeks of discharge. One patient (Patient no.4) had poor weight gain. Overall follow-up is of 1 year except in one patient (6months follow-up).The duplication cyst was first reported by R H Fitz in 1884. This was later described by other authors as well [1]. The most important criterion for the diagnosis of duplication cyst is the presence of normal gastrointestinal epithelial lining in it. Other criteria include the presence of surrounding smooth muscle and continuity with the alimentary tract. In our series all criteria are met.Duplication cyst can be found anywhere in the alimentary tract from the mouth to the anus. They are most commonly seen in the ileal region of the small bowel as was observed by Puligandla et al [2]. In our study also ileum was the most common site. Pyloro-duodenal, colonic and rectal cysts are rare. They can be cystic or tubular in shape and always lie on the mesenteric side of the bowel. Although they can cause symptoms at any point in life, they usually present during infancy. They cause symptoms depending upon their size, shape, location and presence of complications. The complications include bleeding into the cyst, intestinal obstruction, intestinal or duplication cyst perforation, volvulus, cyst torsion, cystic rupture, and malignancy  [3].The diagnosis of duplication cyst in the neonatal period is extremely difficult because the symptomatology is nonspecific. Partial intestinal obstruction in a neonate is an important symptom that should let the suspicion of the entity. The diagnosis is mainly by excluding other more common causes of neonatal intestinal obstruction with radiological diagnostic aid. Ultrasound is the most widely used and the first imaging modality used in the investigation of suspected cases of duplication cysts. CT and MRI scans can give better anatomic localization. On USG, the diagnosis is suggested by the presence of a hypoechoic outer muscular layer and echogenic internal mucosal layer [4]. Peristaltic muscular contractions of the cyst wall are highly suggestive of an intestinal origin of the cyst. Antenatal diagnosis of duplication cyst is suggested by some authors but it is difficult [5]. In our study one patient presented with antenatal USG which was suggestive of lump in mid abdomen.There are other differential diagnoses which should be considered in neonates presenting with abdominal lump with features of incomplete obstruction. These include ovarian cysts, mesenteric and omental cysts [6]. However in neonates, the main differential diagnosis that needs to be ruled out is intestinal atresia which sometimes leads to such gross proximal bowel dilatation which may mimic cystic lesion on USG leading to diagnostic dilemma as happened in the patient no 1 in our study.The treatment of choice is surgical excision with restoration of bowel continuity. This is easily achieved in cystic variants. However in tubular duplication cysts there is involvement of large portion of bowel which on resection might result in short bowel syndrome. Therefore in these cases mucosal stripping is suggested thereby preventing risk of peptic ulceration or carcinogenesis [7]. In our series, all cases had cystic variety and excision with bowel continuity restoration was achieved uneventfully.Duplication cysts are rare cause of neonatal intestinal obstruction. Diagnosis can be achieved keeping a high index of suspicion in a neonate with symptoms suggestive of incomplete or complete bowel obstruction and sonographic evidence of an intra-abdominal cystic swelling located in close proximity to mesenteric border. Management is straightforward surgical with good outcome.Source of Support: NoneConflict of Interest: None


Objective: Lipomas are very common benign tumors located in any part of the body in which fat is normally present, but lipomas containing both osseous and cartilaginous elements are rare. Methods: A case of osteochondrolipoma in a 72-year-old man is reported. The tumor in the mental region was 2×1.5×1.5 cm. Results: After resection of the tumor, there has been no recurrence during the 6-month postoperative follow-up. Histological examination confirmed the definitive diagnosis. Conclusions: Osteochondrolipoma is an extremely unusual lesion that should be kept in mind in the differential diagnosis of soft-tissue tumors. Lipomas are common benign soft-tissue tumors that are composed of mature adipose cells, with uniform nuclei identical to the cells in normal adult fat and cause few clinical problems. Lipomas occasionally contain other mesenchymal elements such as blood vessels, fibrous tissue, and, less frequently, bone, or cartilage.A 72-year-old man presented with a 20-year history of a slow-growing painless mass at the mental region. On examination, there was a single, well-defined, mobile, relatively hard mass, with normal surrounding skin and mucosa .Computed tomographic scan showed an elliptically shaped smooth mass of fat density measuring 2×1.5 cm with partial ossification. The mass was not in contact with the mandible, and a clinical diagnosis of lipoma with calculus was made .Under local anesthesia, the tumor with no adhesions to the periosteum of the mandible was easily dissected through the intraoral incision. Gross examination showed a whitish, partially yellowish mass measuring 2×1.5×1.5 cm, with a well-circumscribed smooth surface . Histolo1,2 They occasionally contain other mesenchymal components such as bone, cartilage, and vessels, and they are called osteolipoma, chondrolipoma, and angiolipoma, respectively. Among them, osteolipoma and chondrolipoma are rare, especially lipoma with both osseous and cartilaginous components. That is, osteochondrolipoma is extremely rare and to the best of our knowledge, only 14 cases have been reported in the English literature, including the present case (5-2,4,9,12,1,3,10,Lipomas are very common benign soft-tissue tumors that are normally found at the back, neck, shoulders, abdomen, or proximal extremities.ent case ; 5 tumorThe nomenclature of osteochondrolipoma is controversial. Jones et al,17,The pathogenesis of osteochondrolipomas remains uncertain. Different theories have been proposed to explain the formation of cartilaginous and osseous tissues in lipomas.3The differential diagnosis should include osteocartilaginous choristoma, metastatic chondrosarcoma or osteosarcoma, liposarcoma with metaplasia, and posttraumatic chondrification.


The modern surgical endodontic treatment is a safe and predictable procedure with high success rate. However, several factors can retard or impede the proper healing process. Use of a high speed handpiece during hard tissues management  can potentially be one of these factors. Formation of metallic debris from the surgical diamond burs, production of necrotic local tissue due to overheating and the direct liberation of air from conventional handpiece into the working area are potential irritants able to delay the tissue healing. The aim of the present article is to report the histopathological findings of the trans-operational accidental contamination with oil in the surgical area during an endodontic surgery. The chief complaint was moderate spontaneous pain that increased upon biting. The patient was subjected to endodontic emergency treatment 1 year earlier, but she never went back to finish the procedure. The medical history revealed no history of systemic problems or allergies. Upon clinical inspection, the access cavity was found without any restoration and the pulp chamber was exposed to the oral fluids. No response was elicited upon buccal or lingual mucosa palpation, contrary to the intense pain caused by horizontal and vertical percussion. The probing depth and mobility were within the normal limits. The radiographic examination revealed narrow and curved root canals, which were surrounded by a diffuse periapical lesion of approximately 6 mm in width and 4 mm in length on the apical portion of the mesial root . TherefoOnce signing a written informed consent by the patient, regional anesthesia was applied by buccal infiltration with Articaine . The left mandibular first molar was isolated with rubber dam and the decayed tissue was completely removed prior to access cavity preparation. The whole treatment procedure was performed using dental operating microscope. The orifices of three root canals  were identified with a DG-16 endodontic explorer. The operatory field was disinfected with gauze soaked in 5.25% sodium hypochlorite  and the root canals were explored with a #15 K-file and abundant 3% NaOCl irrigation. Cervical flaring was carefully performed with #2 and #3 Gates Glidden drills . After determining the working length with radiography, the cleaning and shaping of the apical thirds were completed with S1, S2 and F1 Universal ProTaper rotatory files , irrigating with 3% NaOCl between the instruments. The mesial and distal canals were manually widened up to #40 and 60, respectively using hand K-Flexofiles . Calcium hydroxide paste was placed as an intracanal dressing and the coronal access cavity was temporary sealed.After 7 days, the patient returned with persistent symptoms, especially acute pain upon percussion in the mesial zone of the treated molar. Therefore, it was decided to refresh the intracanal calcium hydroxide. Ten days later, the signs and symptoms were still present. As a result, it was decided to complete the endodontic treatment and schedule a complementary surgical procedure. When the intracanal medication was removed, a flow of purulent exudate was present in the mesial root canals. In response, they were abundantly irrigated with 5.75% NaOCl and were dried with absorbent paper points. All the canals were filled with gutta-percha embedded in a calcium hydroxide sealer, using the Tagger technique. The access cavity was sealed with temporary cement and a final radiography was taken .Endodontic surgeryTen days later, inferior alveolar nerve block with two cartridges of articaine  was performed and after waiting 15 min for anesthesia, a full-thickness triangular flap was raised. Access to apex of the mesial root was performed using a #4 long shank round carbide bur mounted on a conventional high speed handpiece, under abundant irrigation of saline using a hypodermic syringe. After locating and exposing the periapical lesion, all the tissue within the bone crypt was enucleated with a #86 Lucas curette and then was immediately submerged in 10% formalin solution for fixation and subsequent routine histopathology. Once the bleeding was under control by the use of gauze soaked in ferric sulphate, the remaining 3 mm of the root apex was cut with a Zecrya bur  mounted on a high speed handpiece, accompanied with saline irrigation. The ultrasonic retrograde preparation was made with ultrasonic tips , to low intensity and abundant saline irrigation was provided drop by drop at distance. After drying the retrograde cavity with absorbent paper points, MTA was transported using MAP system  and compacted in both mesial canals, followed by radiographic verification . The flaHistopathological analysis of the sampleIn randomized microscopic evaluation of micro-slices of the sample , granuloTo the best of our knowledge, this case is the first histopathological evidence that reports the presence of oil contamination within the enucleated periapical granulomatous tissue which must be integrated to the contraindications of use of conventional handpiece in any dental surgical intervention. It is well demonstrated that non-surgical root canal treatment is a reliable therapy in short- and long-term. According to Salehrabi and Rotstein , high peDuring an endodontic surgical procedure, some complications could occur that may retard or prevent the adequate periapical healing. Various operational accidents related with inappropriate use of the high speed handpiece have been reported. Formation of metallic debris from the surgical diamond burs  or produ° angled head which improves operator’s visibility, although it’s principal advantage is the rear air liberation, including the oil. Furthermore, the application of ultrasonic devices and more recently piezoelectric instruments during the endodontic surgery, allow the management of bone tissue and the root apical third in a safe way. This last system is extremely advantageous because it has the ability to cut the hard tissues (bone and root) without affecting the soft tissues  [A precautionary measure to avoid these such events is the use of a surgical high speed handpiece. This device has a 45 mucosa) . HoweverThis case shows histopathological evidence of operational accidental contamination of periradicular tissues with oil during endodontic surgery, related with the use of high speed nonsurgical handpiece with direct air liberation towards the working area.


Dear Editor,physallis, or bubble), consistentwith a diagnosis of chordoma  of the dorsal spine  and 1B schordoma .-5. Chordomas are slow-growing malignancies derived from primitiveremnants of the notochord. They typically occur in the fifth and sixth decades oflife,7, with a slight predilection for males and preferentialinvolvement of the sacrococcygeal region (50%), followed by the spheno-occipital region(35%), cervical spine, and lumbar spine, occurring only rarely in the dorsal spine andposterior mediastinum-8. Symptoms often appear only after the lesion hasreached large proportions, with local invasion affecting neurovascular structures. Localrecurrence is common when complete resection was not possible.Recent studies in the radiology literature of Brazil have highlighted the importance ofimaging methods in improving the diagnosis of intrathoracic alterations.The differential diagnoses of chordoma include metastases, chondrosarcoma, multiplemyeloma, neurogenic tumors, among others. Although imaging methods help delineate thelesion, the diagnosis is made on the basis of the histopathological analysis-8. Gadolinium contrast enhancement tends to be moderate andheterogeneous,8. Lesions are often accompanied by bone erosion, whichwas not observed in the case reported here. Recent studies have highlighted the use ofdiffusion-weighted imaging in the differentiation between chordomas and chondrosarcomas,reporting that the latter show higher apparent diffusion coefficients,10.On MRI, most chordomas show isointense or hypointense signals in T1-weighted sequences,whereas they show hyperintense signals in T2-weighted and short-tau inversion-recoverysequences, reflecting their high water content, some lesions containing fibrous septaand therefore showing low signal intensity in T2-weighted sequences,8,11,12, there is no specific criterion for differentiating between amulticentric chordoma and metastatic dissemination. We believe that our case couldrepresent dissemination to the cerebrospinal fluid, because there was involvement of thevertebral canal.In addition to an unusual site of involvement, our patient presented the peculiarity of asynchronous lesion. Although some authors have reported similar cases.Because chordoma is resistant to conventional radiotherapy, other modalities, such asstereotactic radiosurgery, are used. Chordoma does not respond well to chemotherapy,antitumor activity having been observed, in small studies, only with the use of imatinibmesylate.The treatment of choice for chordoma is surgical resection with adjuvant radiotherapy,resulting in a disease-free period approximately 2.5 years longer than that achievedafter surgical treatment aloneAlbeit rare, a diagnosis of chordoma should be considered in patients with lesionsaffecting the posterior mediastinum. In addition, the possibility of synchronous lesionsshould be investigated in such patients.


Double gallbladder is a rare biliary anomaly. Perinatal diagnosis of the disorder has been reported in only 6 cases, and in 5 of them the diagnosis was based on ultrasound imaging only. However, the ultrasound technique alone does not provide a sufficiently precise description of cystic ducts and biliary anatomy, an information that is crucial for a correct classification and for a possible future surgery.At 21 weeks of gestational age of an uneventful pregnancy in a 38 year old primipara mother, a routine ultrasound screening detected a biliary anomaly in the fetus suggestive of a double gallbladder. A neonatal abdominal ultrasonography performed on postnatal day 2 confirmed the diagnosis. On day 12 the newborn underwent a Magnetic Resonance Cholangiopancreatography (MRCP) that clearly characterized the anatomy of the anomaly: both gallbladders had their own cystic duct and both had a separate insertion in the main biliary duct.We report a case of early prenatal suspected duplicate gallbladder that was confirmed by a neonatal precise diagnosis of a Type 2, H or ductular duplicate gallbladder, using for the first time 3D images of Magnetic resonance cholangiopancreatography in a newborn. An accurate anatomical diagnosis is mandatory in patients undergoing a possible future cholecystectomy, to avoid surgical complications or reoperations. Therefore, in case of a perinatal suspicion of a double gallbladder, neonates should undergo a Magnetic resonance cholangiopancreatography.A review of the Literature about this variant is included.The online version of this article (10.1186/s12887-018-1043-9) contains supplementary material, which is available to authorized users. Double gallbladder is a rare biliary anomaly with a reported incidence of approximately 1 in 4000 individuals . In adulWe describe a very early prenatal diagnosis of double gallbladder - at the 21st week of gestational age - confirmed soon after birth by Magnetic resonance cholangiopancreatography (MRCP) 3D images. This technique, employed for the first time in this condition, is very helpful and extremely precise. A review of the literature about this rare disorder is likewise provided [At 21 weeks of gestational age of an uneventful pregnancy of a 38 yr. old primipara mother, a routine ultrasound (US) screening of the fetus detected a biliary anomaly allegedly considered a double gallbladder .A neonatal abdominal US performed on postnatal day 2 confirmed the presence of two saccular structures in the gallbladder fossa, without stones and with a normal intrahepatic biliary system.On postnatal day 4 the baby was admitted to the Unit of Pediatric Surgery of the Ospedale Mangiagalli in Milan, Italy.The patient underwent a second abdominal ultrasound that confirmed the existence of a double gallbladder. On day 12 the patient was subjected to a MRCP Fig.  that preThe patient was discharged in good health (see Additional file Double gallbladder is a rare anatomic malformation of the biliary tract, arising from duplication of either 1 primordium or 2 primordia , 4. In tThe anomaly was described for the first time in 1674 by Blasius, at autopsy of a 2-year old boy in Amsterdam. The first report of surgical removal of a double gallbladder was made by Sherren in 1911. Boyden in 1926  proposedPreoperative diagnosis is problematic . UltrasoDouble gallbladder can be discovered in either symptomatic , 6, 9–11Prenatal or early postnatal diagnosis of double gallbladder has rarely been reported. Abdominal US of the fetus is usually performed in order to detect biliary anomalies, first of all a biliary atresia .We found only 4 reports of prenatal or early postnatal diagnosis  describiIn our patient, the anomaly was suspected during pregnancy as early as the 21st week of gestational age. The diagnosis was thereafter confirmed by ultrasound soon after birth although a precise anatomical definition was only obtained by MRCP. To our knowledge, the use of MRCP for this diagnosis has been reported in adults  but onlyOur case consists of a Type 2 H or ductular duplicate gallbladder. Identification of a dual duct as an anatomic variant is particularly important  as this The indication for surgery for double gallbladders is uncertain in asymptomatic patients. Safioleas  states tIn symptomatic patients, laparotomic  or laparOur little patient was asymptomatic so we decided to avoid surgery.In conclusion, six cases of prenatal duplicate gallbladder have been reported so far. Diagnosis in five of them was based on sonography only. As the type of duplicate gallbladder can be correctly characterized only if the insertions of the cystic ducts are identified, this happened only in one newborn through a neonatal MRCP .Duplicate gallbladder is rare but we wish to emphasize that ultrasound images in the fetus or soon after birth are not sufficient. Indeed, in view of a possible surgical treatment, the precise detection of the anatomy of the biliary anomaly is mandatory. Absence of this information exposes to surgical complications or reoperations. Therefore, in case of a suspicion of diagnosis of double gallbladder, the neonate should undergo a Magnetic resonance cholangiopancreatography.We report a case of very early prenatal suspicion of duplicate gallbladder performed with ultrasound on the 21st week of gestational age, and a neonatal very precise diagnosis with 3D images of a MRCP, never reported before, of a Type 2, H or ductular duplicate gallbladder.


Small bowel diverticulosis is a rare finding within the general population and jejunal diverticulosis, specifically, is even rarer. Clinical manifestations can range from post-prandial pain, constipation and malabsorption to serious complications, such as gastro-intestinal hemorrhage, perforation and acute intestinal obstruction. Here we describe the case of an 81-year-old gentleman who presented with a three-year history of abdominal pain and weight loss. Despite unremarkable physical examination and laboratory tests, persistent pneumoperitoneum and dilated loops of small bowel were found on imaging. Having been given a diagnosis of small bowel bacterial overgrowth, the patient underwent capsule endoscopy study for further evaluation of his small bowel. The capsule did not reach the colon and the patient never noted passing the capsule in his stool so, six months post-procedure, a computed tomography (CT) scan seemed to reveal the retained capsule. Subsequent exploratory laparotomy revealed 200 cm of atonic, dilated jejunum with impressive diverticula along the anti-mesenteric border. This case report is an example of an unusual set of presenting signs and symptoms of jejunal diverticulosis, including persistent pneumoperitoneum, pseudo-obstruction and small bowel bacterial overgrowth. A literature review has revealed that these signs have been present in other cases of jejunal diverticulosis, although the etiology and pathophysiology is not clearly understood. Diverticular disease is a disease of old age and of Western society , 2. UncoIn this report, we describe the case of an elderly gentleman with a history of long-standing abdominal pain, weight loss, and small bowel dilation on imaging. He was found to have impressive jejunal diverticulosis on exploratory laparotomy for what was believed to be endoscopic capsule retention.An 81-year-old man presented for evaluation of vague abdominal pain, dyspepsia, and weight loss over the previous three years. His past medical history was significant for prostate cancer, treated more than two decades previously by prostatectomy and adjuvant radiation therapy. Prior to our evaluation, he had been diagnosed with small bowel bacterial overgrowth and was started on amoxicillin/clavulanic acid. This provided some relief of the patient’s symptoms; however he continued to have significant post-prandial pain, which led to progressive weight loss.Physical examination on his initial evaluation revealed a thin, nearly cachectic, elderly male who appeared to be his stated age. His abdominal exam was remarkable for a scaphoid abdomen, no tenderness to palpation, and a well-healed, lower midline surgical incision without hernia. Laboratory evaluation was unremarkable. Imaging included a computed tomography (CT) scan of the abdomen and pelvis with enteral and intravenous contrast. This revealed a small amount of extraluminal air between loops of small bowel and mild, diffuse small bowel dilation, suggestive of a small bowel ileus . The celColonoscopy and esophago-gastroduodenoscopy produced no adverse findings, so his evaluation proceeded with capsule endoscopy to determine the cause of the pneumoperitoneum demonstrated on the prior CT scan. This demonstrated severely inflamed proximal small bowel mucosa and what appeared to be large diverticula. The capsule had not reached the colon and the patient denied noticing that the capsule had passed in his stool. Abdominal X-ray did not reveal that any capsule was retained in either the small bowel or colon. Push enteroscopy was recommended and also demonstrated impressively large diverticulae, beginning at approximately the fourth portion of the duodenum and extending beyond the last visualized area of the proximal jejunum with significant inflammation of the jejunum . A waterApproximately six months after the capsule endoscopy, the patient was seen for worsening abdominal pain, early satiety, and bloating. General Surgery service was consulted for what appeared to be visualization of the endoscopic capsule on a follow-up CT scan . After eSeveral times during the exploratory laparotomy, the small bowel was thoroughly interrogated from the ligament of Treitz to the ileocecal valve, with no sign of a retained capsule. Examination of the small bowel was remarkable for diverticula extending from the ligament of Treitz about 200 cm on the antimesenteric edge of the small bowel . This poThe patient was found to be doing well and gaining weight on follow-up approximately fifteen weeks after surgery and he denies abdominal pain, nausea or vomiting.vasa recta) penetration [Small bowel diverticula are rare, with the prevalence at autopsies ranging from 0.6–1.5%, and imaging studies reporting an incidence of 0.002–0.7% , 9. Micretration .Jejunal diverticulosis manifests in a variety of ways but is fortunately asymptomatic in most cases. When symptomatic, clinical manifestations of this disease may include chronic epigastric or periumbilical abdominal pain—especially post-prandial, constipation, diarrhea and malabsorption , 11. In Recurrent pneumoperitoneum—without peritonitis secondary to jejunal diverticulosis—has been recorded in the literature only a handful of times. Wright and Lumsden described a case where a patient with jejunal diverticulosis developed spontaneous pneumoperitoneum on three separate occasions . Nason aet al. analysed ten patients specifically with jejunal diverticulosis and found that nine had had symptoms of intestinal pseudo-obstruction anywhere from five to forty-three years in duration. They concluded that it is a major clinical presentation of jejunal diverticulosis [Mechanical obstruction can occur from volvulus or intussusception at the site of the diverticulae, or from adhesions due to diverticulitis complication . Pseudo-iculosis . Anothericulosis .The patient presented in this case report had been preliminarily diagnosed with small intestinal bacterial overgrowth (SIBO) and treated with oral antibiotics, resulting in some relief of his symptoms. It is important to note that some of the clinical presentations of small bowel diverticulosis discussed above, such as pseudo-obstruction and dysmotility, can contribute to the development of SIBO ; the bacIn conclusion, jejunal diverticulosis is an uncommon disease that is associated with dysmotility of the small intestine, leading to mucosal and submucosal herniation at points of blood vessel perforation into the intestinal wall. The presentation of the disease is clinically difficult to discern and can often lead to dire complications due to late diagnosis. One should consider such a diagnosis when no organic cause of pneumoperitoneum is found on imaging. The inability of the bowel to contract properly can also lead to pseudo-obstruction and SIBO. Surgical resection of the involved bowel is the definitive treatment in patients presenting with intractable abdominal pain—as in our patient—or for those with bleeding, perforation or mechanical obstruction .Conflict of interest statement: none declared.