Congenital syngnathia is an extremely rare condition with no standardized treatment. We hereby report a case highlighting the difficulties faced in its management and the postoperative complications. Congenital syngnathia refers to the maxillomandibular fusion, a rare condition with 44 cases reported till 2012 [1]. It rarely occurs in isolation and is generally associated with other anomalies like cleft lip, cleft palate, aglossia, popliteal pterygium syndrome [2], van der Woude syndrome, and aglossia-adactylia syndrome [3]. There has been no standard treatment for this condition due to its rarity and long-term outcomes are not well documented.A 2.5 kg first born female baby was brought to our emergency on day 1 of life with complains of fused upper and lower jaw. On examination, the baby was active, crying and stable. Oral examination revealed complete fusion of mandible with maxilla . On general examination, there was no gross congenital anomaly apart from microcephaly. Feeding was started with nasogastric tube. Routine blood investigations were normal and baby was taken up for definitive surgery on day 4 of life. Release of the fused gums was attempted under intravenous fentanyl, ketamine and inhalational sedation. Release was only possible for 1cm from the midline on the right side to allow for one finger insertion. Complete bony fusion was present on the left side. As intubation was not possible procedure was abandoned with a plan to go for radiographic assessment of the fusion. Contrast enhanced computed tomography (CECT) was done, which showed maxillomandibular fusion along with left temporomandibular joint fusion, right side cleft palate, retrognathia and micrognathia. The child was again taken up for surgery on day 28 of life where nasal intubation with the help of fiber-optic bronchoscope was done and release of the syngnathia with feeding gastrostomy was performed. On the fourth postoperative day, the baby developed upper abdominal distention, bilious vomiting and blood in stools. Abdominal radiograph showed multiple air fluid levels. Following this, the child was explored; it revealed herniation of the proximal ileum between the gastrostomy and the abdominal wall with gangrenous changes in the herniated loop. Gangrenous part was excised and primary end-to-end anastomosis was performed. Intraoperatively, there were dense adhesions, which when released led to serosal tears that needed repair. Again, on the fourth postoperative day, the child developed signs of peritonitis that needed abdominal re-exploration which revealed jejunal perforation 10cms proximal to the previous anastomosis, from one of the sites of serosal tears. Primary repair of the perforation site was done along with central line placement for nutrition. Again, after 2days, the child had fecal fistula from the wound site and was explored, which revealed complete anastomotic dehiscence of the previous ileal anastomosis. Repair was done again but the child went into sepsis and finally expired on day 42 of life.Burket in 1936 first reported about syngnathia as a bony fusion of upper and lower jaw and since then very few cases have been reported in the English literature [4]. Though majority present at the birth but few cases have been reported in elderly . Dawson et al. in 1997 first classified it in two types as simple and complex syngnathia; was modified 4 years later into 4 types . Cleft palate is the most common association and it was present in our case too [1].Management has not been standardized due to the rarity of this condition but early surgery has been recommended. Nutrition should be started early using nasogastric tube. Various procedures have been done to release the fusion, but recurrence has been the most common problem despite all efforts [5-8]. Some authors also suggested the use of inter-positional flap using temporalis muscle or buccal mucosa and insertion of an implant such as silastic sheets between separated soft tissues to prevent recurrence [6]. The other issue is that of expert anesthetic management.. Nasal intubation with the help of fiber-optic bronchoscope under local anesthesia and sedation is the way out in such babies, though tracheostomy should be considered in cases of failure or where resources are not available. Similar approach of nasal intubation was done in our case during the second surgery, which was reported earlier[8]. Though in our case the release was possible, the child succumbed to multiple complications.Source of Support: NilConflict of Interest:The corresponding author is editor of the journal, but he was not involved in review and decision.


Background: We present a case of adenocarcinoma arising in the oncocytic subtype of intraductal papillary mucinous neoplasm (O-IPMN), with emphasis on the molecular findings in the adenocarcinoma component. Tissue microdissection and next-generation sequencing were performed using a 26 gene panel  of cancer-related genes.Case Presentation: A 69-year-old Caucasian female presented with chest pain and was found to have findings consistent with acute pancreatitis. During her work-up, computed tomography scan revealed a large cystic and solid mass in the tail of the pancreas. She recovered from her acute pancreatitis and was discharged home. She later returned for resection of her mass.Results: Evaluation of three microdissected regions of tumor demonstrated no identifiable nonsynonymous alterations in any of the three regions, within the targeted genes.Conclusion: This case demonstrates that the O-IPMN is a molecularly distinct subtype, and we conclude that adenocarcinoma arising in these neoplasms shows molecularly distinct tumorigenesis from traditional pancreatic ductal adenocarcinoma. These differences may help explain the improved survival with invasive adenocarcinoma arising from these lesions compared with traditional ductal adenocarcinoma. Infrequently, these tumors develop an associated low-grade invasive carcinoma that retains the oncocytic features. With the introduction of routine molecular genetic analysis, including next-generation sequencing (NGS), recent studies have shown that these lesions are, in fact, genetically distinct from the typical IPMNs, which likely contribute to prognostic differences. We present a unique case of a 69-year-old female with a high-grade signet ring cell carcinoma arising within an oncocytic IPMN (O-IPMN), with associated radiological studies, molecular testing, and a review of literature.Intraductal papillary mucinous neoplasms (IPMNs) are a heterogeneous group of tumors. The epithelial subtypes  have been shown to be independent predictors of biological behaviors, recurrence, and long-term survival rate. The rarest subtype, oncocytic, was first described by Adsay et al.The patient was a 69-year-old Caucasian, otherwise healthy female who initially presented to an outside hospital with chest pain and was found to have an elevated serum lipase consistent with acute pancreatitis. Notably, she had a history of pancreatitis 16 years prior, thought to be secondary to gallstones, and underwent cholecystectomy at that time. During her work-up for pancreatitis, computed tomography (CT) scan revealed a large, multilobular mass in the tail of the pancreas; the mass had mixed cystic and solid components, which is concerning for a mucinous cystic neoplasm. Follow-up magnetic resonance imaging (MRI) corroborated CT findings. She recovered from her acute pancreatitis during an inpatient stay and was ultimately discharged home. After discharge, the patient remained largely asymptomatic.She was referred to the University of Colorado Hospital Pancreas Multi-Disciplinary Clinic for evaluation and surgical management. A complete blood count and complete metabolic panel were within normal limits. Carcinoembryonic antigen and carbohydrate antigen 19-9 were also within normal limits. Imaging studies were reviewed and management discussed with more than 20 physicians from a broad range of specialties. Ultimately, given the radiological appearance of the lesion and concern for high-grade dysplasia versus invasive carcinoma, the recommendation was made to proceed with resection. The patient underwent open distal pancreatectomy and splenectomy.The patient was taken to the operating room for distal pancreatectomy and splenectomy. Upon exploration, there was no evidence of metastatic disease or lymphadenopathy. Therefore, it was decided to proceed with planned distal pancreatectomy and splenectomy. The entire tail of the pancreas was easily exposed, revealing a softball-sized cystic neoplasm along the inferior edge of the pancreatic hilum. It was a well-circumscribed cyst without any inflammation. The rest of the pancreas was soft with normal architecture. It was a very classic appearance of a cystic neoplasm. The spleen was then mobilized into the operative field easily, and the retroperitoneal attachments extending to the ligament of Treitz were easily taken down. The splenic artery and splenic vein were then encircled and ligated. The pancreatic neck was then thinned out and stapled across with an Endo-GIA stapler. The specimen was sent to pathology with negative margins. The procedure was well tolerated by the patient and without complications. Estimated blood loss was 100 cc.Her postoperative course was unremarkable, and she was discharged home 6 days later. Intraoperative pancreatic margin was negative for malignancy. Final pathology showed an intraductal oncocytic papillary neoplasm (IOPN) measuring 11 × 9 × 7 cm, with a component of invasive adenocarcinoma. Microscopic margins, as well as 17 sampled lymph nodes, were negative for malignancy. The invasive component of the tumor was difficult to assess owing to extensive necrosis, but was 2.2 cm in greatest diameter for the viable portion of the tumor.Initial CT scan showed a multilocular, mixed solid, and cystic mass, which measured 9.0 cm in the greatest dimension . Follow-The distal pancreatectomy specimen contained a well-encapsulated 11-cm multilocular, cystic lesion, which caused dilation of the main pancreatic duct. The cyst contained watery mucin contents and numerous solid-to-papillary excrescences. Microscopically, the cysts were composed of edematous, paucicellular stroma lined by simple columnar cells with abundant granular eosinophilic cytoplasm and prominent nucleoli with scattered goblet cells. These cells formed complex arborizing papillae, cribriform patterns, and varying degrees of solid growth. Multiple foci of low-grade and high-grade invasive carcinoma were present. The low-grade areas were characterized by small nests of tumor cells within abundant extracellular mucin. The high-grade areas were composed of signet ring cells with invasion into the stroma. Cytologically, the low-grade tumor displayed the same densely eosinophilic cytoplasm of the cystic lining  and 3. NCDKN2A, TP53, and MADH4,2 and activation of the protooncogene KRAS. To evaluate the invasive component of this tumor for these recognized molecular abnormalities, NGS was performed.Typically, well-documented events are responsible for the biological behavior of pancreatic ductal carcinomas. Most cases show inactivation of tumor suppressor genes AKT1, ALK, APC, BRAF, CDH1, CTNNB1, EGFR, ERBB2, FBXW7, FGFR2, FOXL2, GNAQ, GNAS, KIT, KRAS, MAP2K1, MET, MSH6, NRAS, PDGFRA, PIK3CA, PTEN, SMAD4, SRC, STK11, and TP53. Libraries were sequenced on the Illumina MiSeq platform for a targeted depth of no less than 500 × for any individual amplicon. A custom-built bioinformatics pipeline utilizing GSNAP for sequence alignment and FreeBayes for variant calling was employed for data analysis. All genomic regions were verified to be covered by at least 500 sequencing reads, and identified variants were manually inspected using Integrative Genomics Viewer (Broad Institute).Ten-micron cut sections were deparaffinized, hematoxylin counterstained, and microdissected by scalpel point under a microdissecting microscope. The microdissected material was washed with 70% ethanol, air dried, and resuspended in lysis buffer, and DNA was extracted (Qiagen QIAamp DSP DNA FFPE Tissue Kit) using manual extraction with elution into 30 μL of elution buffer. Library preparation was performed through the Illumina TruSight Tumor 26 kit per the manufacturer's instructions (with minor modifications). This kit amplifies selected regions of 26 cancer-related genes: Evaluation of three separately microdissected regions of the tumor demonstrated no identifiable nonsynonymous alterations in any of the three regions, within the targeted areas of our cancer-related gene panel.3 Recent molecular studies suggest that, when strict morphological criteria are applied, the oncocytic subtypes are genetically distinct lesions. These lesions lack the most common molecular abnormalities found in IPMNs. Specifically lacking are KRAS mutations that have been detected in the majority of IPMNs,4–6 activating GNAS mutations at codon 201 that have been identified in approximately half of IPMNs,7 and inactivating mutations in the RNF43 gene, a tumor suppressor in the Wnt signaling pathway. Instead, molecular studies have shown somatic mutations in ARHGAP26, ASXL1, EPHA8, and ERBB4. The differences suggest that these oncocytic subtypes have a distinct pathway of tumorigenesis that likely contributes to the relatively indolent nature of the lesion. In addition, genetic testing of the associated adenocarcinoma has failed to demonstrate CDKN2A, TP53, MADH4,7 or activation of the protooncogene KRAS found in the majority of pancreatic ductal adenocarcinomas.The formerly named IOPN is an exceedingly rare lesion that has been recently reclassified as an O-IPMN.1 with or without an invasive component.Our case of high-grade adenocarcinoma arising in association with an O-IPMN is morphologically unique in that all of the documented invasive tumors associated with O-IPMNs exhibit low-grade morphology with or without extracellular mucin. Our tumor is composed of low-grade elements but also contains areas of high-grade, signet ring morphology. Although this high-grade morphology might suggest some homology with invasive ductal adenocarcinoma, NGS failed to show similar molecular changes. This provides additional evidence that O-IPMNs have a distinct genetic tumorigenesis. This may explain why, empirically, these lesions have an improved 5-year survival compared with gastric, pancreatobiliary, or intestinal IPMNs


We describe a giant gastric phytobezoar in a child with repaired congenital esophageal atresia. At age two, a gastric interposition (pull-up) procedure was performed for severe and recurrent esophageal strictures. For 12 months post-gastric interposition, he experienced frequent respiratory illnesses requiring hospital admissions but it was not initially appreciated that these episodes were likely secondary to recurrent aspiration from a gastric bezoar with “spill-over” aspiration. A bezoar is a tightly packed mass of foreign indigestible material in the gastrointestinal tract, most often in the stomach but they can occur elsewhere in the intestine. The composition of bezoars varies with different types described including phytobezoars which comprised most commonly indigestible fruits but also vegetable fibers, skin, or seeds .Bezoars can develop in patients with altered gastrointestinal anatomy, motility, and even in those with otherwise normal gastrointestinal tract. Most bezoars are found in children and young females with pica, and particularly when associated with psychiatric disorders or mental retardation. By contrast, in adults, most bezoars are related to previous gastric surgery that reduces gastric motility , 3.Our patient was born with esophageal atresia without a fistula and underwent a successful primary anastomosis on day 2 of life. Beginning in his second year of life, he became symptomatic with feeding difficulties due to recurrent stricture formation at the anastomotic site. Repeated dilatations proved ineffective; therefore, a gastric interposition procedure was performed. Following gastric interposition, he began experiencing recurrent acute respiratory symptoms including wheeze and had repeated acute hospital admissions. He also experienced persistent “noisy” breathing, vomiting up to 20 times per day, and persistent halitosis. Examination at the time reportedly revealed decreased breath sounds over the right middle and right lower lobes.® (250–500 ml daily) lead to dissolution of the bezoar, and ultimately successful gastroscopic removal of the bezoar. Of note on the fourth postoperative day, the patient had a significant hematemesis requiring fluid resuscitation. This was a self-limiting event and the previous endoscopic instrumentation and possible concurrent gastric ulceration related to the bezoar was suspected to be the cause. Later surgery was performed to remove a fold of redundant gastric mucosa obscuring the pylorus which was above the diaphragm and part of the stomach was reduced into the peritoneal cavity. His most recent chest radiograph has markedly improved  and as in our case it was successful (Bezoar management involves lavage or dissolution and fragmentation of gastric materials, and/then retrieval of as much material as possible. This usually involves forceps and suction removal of the various materials. Many substances have been utilized to effect dissolution of the gastric materials including Coca-Colaccessful , 7–9. Faccessful .While the giant bezoar in this case is of interest, there are other important messages to be drawn from this case. A gastric interposition procedure may impair gastric motility. In gastric interposition, the stomach is mobilized through an epigastric incision and proximal esophagus through a neck incision. The esophageal stump is excised and pyloroplasty is performed. A trans-hiatal posterior mediastinal tunnel is created. The gastro-esophageal anastomosis is performed in the neck using the apex of the stomach .In any child who has had surgical repair of esophageal atresia either by primary anastomosis or, as in this case, by a secondary gastric interposition procedure, who experiences recurrent or persistent respiratory symptoms, aspiration into the airway should be central in the differential diagnosis. With hindsight, this boy’s chest radiograph was grossly abnormal some 12 months before his referral. The significance of the radiographic changes and the likelihood of recurrent aspiration as the cause of his repeated acute respiratory events were not appreciated with these presentations misdiagnosed as pneumonia or asthma exacerbations. While an intra-thoracic bezoar is uncommon, the recurrent acute respiratory symptoms associated with frequent daily vomiting were strongly indicative of recurrent pulmonary aspiration and indications for further investigation.Children who have had surgical repair of esophageal atresia are at later risk of pulmonary aspiration through stricture formation at the site of the primary anastomosis. In this case, gastric interposition surgery performed because of recurrent stricture formation was subsequently complicated by recurrent vomiting, episodes of acute respiratory distress due to pulmonary aspiration. These were all clues to disordered gastric function and emptying. While gastric bezoar formation would not, necessarily, have been “at the top of mind” as a diagnosis, the chest radiograph prior to referral was strongly suggestive of this diagnosis.Parental consent given to present case.AC, BM, and AI contributed to the writing and editing of the case report.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Percutaneous endoscopic gastrostomy (PEG) has gradually gained the popularity since its invention and become the most preferred method for gastrostomy insertion in recent years. PEG is associated with lower morbidity and mortality and has the advantages of being minimally invasive and more convenient over the conventional open gastrostomy. However, significant rates of major complication still occur. Enterocutaneous fistula is one of the key complications that can be easily neglected due to its asymptomatic nature. We present a case of small bowel enterocutaneous fistula which was only found 8 years after the PEG insertion, being diagnosed after the longest duration of delay in diagnosis reported in literature. Percutaneous endoscopic gastrostomy (PEG) has dramatically renovated the practice in gastrostomy placement since it was first described in by Gauderer et al. at the University of Cleveland in 1980 –6. We heA 35-year-old female patient suffering from severe mental retardation and epilepsy since birth was a long-term resident of the mentally incapacitated unit of the hospital. She was bedridden with a small body build of a weight of 32 kg. She had a PEG insertion done 8 years ago and then received regular PEG tube change every 3 months by the nurses and maintained well on the gastrostomy bolus milk feeding. Her body weight was static over the past years and there was no sign or symptoms of malnutrition or diarrhea.She presented to our gastroenterology unit for failure to deflate the PEG balloon during a regular PEG exchange due to blockage of the inflation/deflation catheter. Upon admission, the physical examination of the abdomen was normal with active bowel sound and negative for any distension or tenderness. The routine blood tests including complete blood picture, electrolytes, and liver and renal function tests did not review any abnormality. An oesophagogastroduodenoscopy (OGD) was arranged with an attempt to examine and deflate the balloon in the stomach endoscopically. An OGD examination failed to identify any gastrostomy tube in the upper gastrointestinal (GI) lumen with the scope passed down to the second part of the duodenum. An urgent computer tomography (CT) of the abdomen revealed the distal end of the PEG tube was likely situated at the proximal jejunum distal to the duodenojejunal junction with a normal fill-filled balloon intraluminally. There was no radiological feature of pneumoperitoneum . The PEGA nasogastric tube was inserted and used as a temporary measure for nutritional support and the fistula was allowed to heal spontaneously. A laparoscopic gastrostomy insertion was arranged for the patient. Intraoperatively, the previous PEG site at the jejunum was found to be adhered to the antrum of the stomach and the anterior abdominal wall. Another new gastrostomy tube was inserted by the operation and the patient tolerated gastrostomy feeding well afterwards.Since its innovation in the early 1980s, PEG has gained the popularity in gastrostomy insertion over laparoscopic gastrostomy as it avoids the need of general anesthesia and can be performed much less invasively. Overall it carries a successful rate as high as 98% and a lower risk of mortality compared to open gastrostomy of less than 1% , 3, 5, 7Enterocutaneous fistula occurs when a segment of small or large bowel entrapped between the stomach and the abdominal wall. It is highly unusual in adults; a large case cohort identified only 6 cases of colocutaneous fistula out of 2384 adult patients receiving PEG insertion  while anA proper technique of transillumination, finger indentation of the ventral abdominal wall, and observation of any gas bubble aspirated from the injecting needle before entrance into the stomach during the operation can minimize the risk of interposition of the colon or small bowel , 10, 15.The majority of the enterocutaneous fistula resulting from PEG insertion reported from the literature is colocutaneous fistula and most of them would be symptomatic. The presentation includes diarrhoea upon tube feeding, faecal discharge from the tube, weight loss, abdominal pain, intestinal obstruction, and difficulty in tube exchange and also acute peritonitis after procedure , 12, 15.In conclusion, small bowel enterocutaneous fistula is a rare complication of PEG tube placement which can remain silent, asymptomatic for many years, and neglected easily. Careful case selection to avoid procedure candidate with multiple high risk factors and proper endoscopic techniques are useful in avoiding this complication. Moreover, the healthcare workers responsible for the PEG care have to take caution on any gastrointestinal, abdominal, or tube-related symptoms of the patients to ensure an early diagnosis and management.


We present a unique case of a 16-year-old male patient who was eventually diagnosed with a large enterolith arising from a Meckel's diverticulum. The enterolith had caused intermittent intestinal symptoms for three years before resulting in small bowel obstruction requiring surgical intervention. Meckel's enterolith ileus is very rare with only few cases described in the literature. To our knowledge, this is only the second case of Meckel's enterolith which had caused intermittent symptoms over a period of time, before resulting in ileus, and the first case where the intermittent symptoms lasted several years before bowel obstruction. The patient had been evaluated with colonoscopy, computerized tomography (CT), and magnetic resonance imaging enterography (MRIE); a calcified pelvic mass had been found, but no further diagnosis other than calcification was established. The patient presented at our emergency department, with symptoms of obstructive ileus and underwent exploratory laparotomy, where a large enterolith arising from a Meckel's diverticulum (MD) was identified, causing the obstruction. A successful partial enterectomy, enterolith removal, and primary end-to-end anastomosis took place; the patient was permanently relieved from his long-standing symptoms. Consequently, complications of Meckel's diverticulum and enterolithiasis have to be included in the differential diagnosis of abdominal complaints. Meckel's diverticulum is a common embryological remnant that can be found incidentally during surgery for other pathology, or may manifest as acute abdomen, most commonly due to diverticular inflammation. More rare complications of Meckel's diverticulum, including bleeding, herniation, intussusception, and enterolithiasis, have also been reported , 2. We pA 16-year-old patient presented to our outpatient clinic for evaluation of a pelvic calcified mass, initially found three years earlier in an abdominal X-ray, during investigation of an episode of lower right quadrant pain .At that time, the patient did not require any surgical intervention and was managed conservatively. He was advised to have an outpatient workup of this mass. He was evaluated by a gastroenterologist and underwent an MRI enterography and colonoscopy. The MRI enterography revealed a calcified lesion in the distal ileum, located inside the pelvis, while the colonoscopy failed to recognize any lesion or inflammation in the terminal ileum. Meanwhile, the patient occasionally complained of nonspecific abdominal symptoms, which did not prompt him to seek further medical attention and resolved automatically.Two years later, we scheduled an abdomen computerized tomography (CT), as a follow up. Contrast-enhanced abdominal CT revealed the same calcified lesion, 5 cm in diameter, that was in contact with the small bowel in the pelvis. The lesion had not grown in size during the last years.Three years after the first onset of symptoms, the patient presented in our emergency department with symptoms of small bowel obstruction, namely, vomiting, intense colicky abdominal pain, and gas/flatus retention for 48 hours. Clinical examination revealed abdominal tenderness, distension, and hyperactive bowel sounds upon auscultation. Lab studies revealed mild leukocytosis. Plain X-ray showed multiple air-fluid levels. A new CT scan of the abdomen revealed obstruction of the small intestine, by the aforementioned mass .The patient underwent an emergency laparotomy, where a loop of small bowel was found to be adherent to the pelvic wall. Upon dissection and mobilization of this loop from the pelvic wall, an enlarged and inflammed Meckel's diverticulum was identified. The diverticulum contained an enterolith, which was obstructing the bowel lumen. A partial enterectomy and an end-to-end anastomosis of the small bowel were performed.The operation led to resolution of the obstruction, and the postoperative course was uneventful.The pathology report confirmed the presence of a large enterolith, 5 cm in diameter, and ulcerative transmural inflammation of the resected part of the small intestine.Six months following the operation, the patient was completely relieved from his occasional abdominal complaints and remained totally asymptomatic.Informed consent was obtained from the patient for publication of this case report.Meckel's diverticulum is usually asymptomatic (85–95%), but clinical presentations associated with Meckel's diverticulum are diverticulitis, rectal bleeding, and small intestinal obstruction . Small bMeckel's diverticulum is complicated with enterolithiasis in 3–10% of cases . The difEnteroliths can cause obstruction , divertiTo our knowledge, our case is only the second to describe a Meckel associated large enterolith that had been causing intermittent-type bowel symptoms for a certain period of time, before resulting in acute abdomen, due to obstructive ileus, thus requiring immediate surgical intervention . InteresIn conclusion, enterolithiasis is an entity that can be a diagnostic challenge for the clinician. Meckel diverticulum-associated enterolithiasis can produce bowel obstruction. The clinician should include Meckel's diverticulum complications and enterolithiasis in the differential diagnosis of abdominal symptoms, either acute or chronic.Meckel diverticulum-associated enterolithiasis is a rare condition that can be either asymptomatic or produce nonspecific symptoms but can also manifest itself as acute abdomen. Intestinal obstruction due to Meckel's enterolithiasis is very rare, with only few cases described in the literature. Our patient experienced intermittent symptoms for a course of several years before developing intestinal obstruction. Our case underlines that complications of Meckel's diverticulum and Meckel diverticulum-associated enterolithiasis need to be included in the differential diagnosis of abdominal complaints, as this diagnosis can be particularly challenging.


Agenesis of dorsal pancreas (ADP) is an extremely rare congenital anomaly that results from defective development of pancreas. Most ADP patients are asymptomatic; if symptomatic, they present with epigastric pain. About half of affected individuals develop diabetes mellitus (DM), resulting from reduced islet cell mass secondary to the absence of endocrine structures. Being very rare, it is generally not kept in mind while dealing these cases and are not suspected until imaging investigations are not done. In our case study, ADP was diagnosed during evaluation of the patient for recurrent pain abdomen and generalized weakness.How to cite this article: Jain A, Singh M, Dey S, Kaura A, Diwakar G. A Rare Case of Complete Agenesis of Dorsal Pancreas. Euroasian J Hepato-Gastroenterol 2017;7(2):183-184. Here we present a case of ADP diagnosed during workup of pain abdomen and DM.Agenesis of dorsal pancreas is an extremely rare congenital anomaly that results from defective pancreas development. The first case was reported in 1911 during an autopsy and around 100 cases have been reported since then.An otherwise healthy 37-year-old female presented to the outpatient department with complaint of recurrent upper abdominal pain, fatigue, dizziness, and pain in lower extremities for past few months. General physical and systemic examination was normal.Routine blood investigations revealed high blood sugar level (454 mg/dL) and she also had glucosuria. Patient was evaluated for recurrent abdominal pain and subsequently workup for DM was done. Ultrasound abdomen was done, in which only head of pancreas was seen and distal pancreas and duct were not visible. Magnetic resonance cholangiopancreatography (MRCP) was subsequently done in which distal body and tail of pancreas were not visible and diagnosis of ADP was made  and 2. P3 Agenesis of the ventral pancreas and complete agenesis of the pancreas are incompatible with life.4Abnormal embryogenesis can lead to developmental failure of the dorsal pancreas, resulting in complete ADP.5 The cause of ADP is currently not well understood. Familial transmission has been reported in the literature.6 Differential diagnosis of ADP includes pancreas divisum , pseudo-agenesis (atrophy of the body and the tail of the pancreas secondary to chronic pancreatitis and sparing of the pancreatic head), and congenital short pancreas.7The pancreas is formed by ventral and dorsal endodermal buds. The ventral bud gives rise to the major part of the head and uncinate process which drains through Wirsung duct. The dorsal bud forms the upper part of the head, body, and tail of the pancreas which drains through the Santorini duct.8 A case of pancreatic adenocarcinoma associated with dorsal agenesis has also been reported.9Most ADP patients are asymptomatic; if symptomatic, they present with epigastric pain. About half of the affected individuals develop DM, resulting from reduced islet cell mass secondary to the absence of endocrine structures, which are normally predominantly located in the body and tail of the pancreas. A connection to pancreatitis has also been proposed. Potential mechanisms include sphincter of Oddi dysfunction, compensatory enzyme hypersecretion, hypertrophy of the ventral gland, and higher pancreatic duct pressures.10 Moreover, ERCP or MRCP can define the anatomy of the pancreatic ducts more precisely.11 Treatment is usually symptomatic and supportive.The most frequent imaging techniques used ultrasound and computed tomography scan, but they are suggestive of ADP. Further confirmatory imaging techniques are magnetic resonance imaging, endoscopic retrograde cholangiopancreatography (ERCP), MRCP, and, recently, endoscopic ultrasound.Being very rare, it is generally not kept in mind while dealing with such presentations and not suspected until imaging investigations are done. In our case study, ADP was diagnosed during evaluation of the patient for pain abdomen and generalized weakness.In patients with unexplained pain abdomen and with constitutional symptoms, a thorough evaluation should be done and anomalies such as, ADP should be kept in mind.


Pediatric pancreatic injuries are rare. We present an atypical case that occurred in a 4-year-old male. The child presented with a twenty-four-hour history of vomiting that had progressed to right lower quadrant abdominal pain on examination in the emergency department. The initial differential was gastroenteritis versus appendicitis. An abnormality on the ultrasonography and an elevated lipase level eventually led to an MRI showing a complete transection through the posterior margin of the pancreas. The patient was admitted to pediatric surgery and underwent a successful distal pancreatectomy with preservation of the spleen. On further inquiry specific to trauma, the child disclosed that his older brother had punched him in his abdomen the night before. The child's parents were separated due to intimate partner violence, and this older sibling recently had been very stressed. The sibling was referred for mental health evaluation and counseling, and the case reported to the county children and youth investigative services system. A low threshold for considering trauma and child abuse in the pediatric population is recommended when significant intra-abdominal injury is diagnosed. A 4-year-old male presented to the emergency department complaining of a 1-day onset of nausea and vomiting that had progressed to periumbilical abdominal pain radiating to the right lower quadrant. Physical examination revealed a blood pressure of 117/79 mm Hg, pulse 118 beat/min, respiratory rate 22 breaths/min, oxygen saturation 100%, and temperature 99.3°F. He appeared well developed and well nourished. He was alert and presented no neurologic deficits. He produced mucous vomit during examination. He exhibited tenderness upon palpation over the periumbilical and right lower quadrant regions of the abdomen. There was mild guarding with no rebound. Bowel sounds were normal, and he exhibited no distention and no mass.Laboratory results were significant for lipase 9373 U/L (80–360 U/L), hemoglobin 8.1 (10.3–14.9 g/dL), hematocrit 26.2 (32–42.0%), aspartate aminotransferase (AST) 70 (26–55 U/L), and alanine aminotransferase (ALT) 257 (<56 U/L). His total bilirubin at 0.3 (0.0–0.4 mg/dL) and alkaline phosphatase at 199 (256–369 mg/dL) were both normal. His urinalysis was significant for ketones and protein. Ultrasonography of the abdomen showed a normal appendix, a normal gallbladder, no focal liver lesion, no biliary ductal dilation, and no focal splenic lesion. There was a moderate amount of complex intraperitoneal fluid seen and an incomplete visualization of the pancreas. Heterogeneous nodules were seen related to the posterior margin of the body of the pancreas. Abdominal CT  showed lThe patient was admitted to pediatric surgery and underwent a successful distal pancreatectomy with preservation of the spleen. On further inquiry specific to trauma, the mother stated that she recalled the patient had been playing with his brothers upstairs the night before and might have fallen off the bunk bed. Further history by a child advocacy physician on second inquiry was told by the mother that, the evening prior to presentation, she was aware that the child was crying because he claimed he was hurt by his brother. As he had calmed quickly, she did not think anything serious happened as they often “rough house.” In a later interview, the child disclosed that his older brother (described by their mother as a 12-year-old who was approximately 5 1/2 feet and weighing 120 lbs) punched him in his abdomen causing his injury. The child's parents were separated due to intimate partner violence, and this sibling had been recently very stressed. Previously, he had witnessed significant violence towards his mother.Pediatric pancreatic injuries are rare and account for less than 2% of all abdominal injuries . Most paMajor pancreatic injuries are uncommon in children, and the diagnosis often is delayed , 4. HoweChildren exposed to intimate partner violence (IPV) are at increased risk of being abused and neglected . They arOf additional interest, our patient did not have bruising or signs of trauma to the abdomen. Significant visceral injury may present with little or no specific signs, where as few as 12% may have abdominal bruising, emphasizing the need for early consideration as some children may present with nonspecific symptoms, for example, vomiting and irritability . Child aA severe intra-abdominal injury such as pancreatic transection can present with nonspecific symptoms and signs. A low threshold for diagnosing occult trauma is required. Child abuse should be strongly suspected when the clinical history does not match with the severity of injury.


We present a case report of recurrent disseminated peritoneal leiomyomatosis in a 30-year-old female with a past history of laparoscopic myomectomy by a technique of morcellation for a large fibroid in 2014. After one year she presented in 2015 with a well-defined oval shaped fibroid along the anterior abdominal wall, which was supplied by the 10th intercostal artery and a branch of right internal mammary artery. She was again presented after 1 year in 2016 with a large pelvic-abdominal fibroid with blood supply from the omental artery, a branch from the right gastroepiploic artery, and sigmoid branches of inferior mesenteric artery. Uterine fibroids affect 20%–30% of older women. Extrauterine fibroids are very unusual and they present with a greater diagnostic dilemma. These are histologically benign tumors and originate from smooth muscle cells, usually from the genitourinary tract . Unusual growth patterns and locations can also be seen including disseminated peritoneal leiomyomatosis, benign metastasizing leiomyoma, parasitic leiomyoma, intravenous leiomyomatosis, and retroperitoneal growth. The disseminated leiomyomatosis growth out of subperitoneal mesenchymal stem cells, which undergo metaplasia under the stimulation of hormones, has been widely accepted theory . HoweverA 29-year-old female patient presented in 2014 with complaints of heaviness and pain in the lower abdomen for the past 3 months. Ultrasound was done outside of our hospital which revealed a large heterogeneously isoechoic mass lesion in the myometrium of fundus displacing and splaying of the endometrial cavity. She underwent laparoscopic myomectomy by morcellation technique for removal of uterine fibroids. Surgical specimen is sent for the histopathological evaluation, which confirmed the leiomyoma.After one year, she again presented with abdominal pain and underwent ultrasound imaging, which revealed a well-defined oval shaped highly vascular lesion along the anterior abdominal wall in the right hypochondrium. CT angiography was performed for further evaluation, which revealed a well-defined oval shaped highly vascular lesion along the anterior abdominal wall. The blood supply to this lesion was from 10th right intercostal artery and the branch from the right internal mammary artery. In addition, multiple well-defined round to oval homogenously enhancing lesions were also noted in the mesentery in the left iliac fossa and along the superior border of urinary bladder . The patIn march 2016, again she presented with abdominal pain from the past 15–20 days. On imaging, she was diagnosed as a case of uterine fibroids and was taken for surgery. During the surgery it was found that the fibroid was extremely vascular and hence the surgery was abandoned and was referred to our hospital for embolization and further treatment. MRI  and CT aThe patient recovered satisfactory in postoperative period and was discharged in next seven days.Disseminated leiomyomatosis is rare disorder characterized by multiple vascular leiomyomas growing along the submesothelial tissues of the abdominopelvic peritoneum .Laparoscopic myomectomy is recommended as a procedure of choice for patients requiring removal of the fibroids in the young patients. Laparoscopic route has been proved to be safe, efficient, and cost-effective . HoweverBecause of nonspecific symptoms and atypical radiologic features diagnosis of disseminated fibroid is challenging. On CT scans they are usually observed as well-circumscribed multiple nodules with contrast-enhancement characteristic to that of myometrium or uterine fibroids. However, when necrosis, degeneration, or implantation of endometrial components occurs an enhancement is heterogenous, mimicking peritoneal carcinomatosis. Our case is presenting myometrial tissue with internal cystic areas suggestive of plentiful endometrial glands interspersed between the smooth muscle cells. What is important, these findings imply that there is a risk of dissemination of endometrial cancer or other uterine malignancies if they are not diagnosed before laparoscopic surgery. The superb contrast resolution and multiplanar capabilities of MR imaging make it particularly valuable for characterizing these tumors, which usually show low signal intensity similar to that of smooth muscle on T2-weighted images. Early diagnosis and knowledge of such disease may help the clinician toward timely appropriate management.Only few cases of recurrent disseminated fibroid after laparoscopic hysterectomy and laparoscopic myomectomy have been reported in the literature –5. AlthoIn summary, uterine morcellation may result in seeding of myometrial tissue throughout the abdominal cavity that leads to serious morbidity and causes diagnostic dilemma. And the disseminated leiomyomatosis can seek blood supply from any nearby vessel. Although laparoscopic hysterectomy is safe and widely accepted procedure, possibility of such a complication should be kept in mind.


Glomangiomyoma is a rare histological variant of glomus tumour. Clinically, it mimicks as a haemangioma and is challenging to diagnose. Its occurrence in the neck of a child has not been previously described.A 3 year old girl presented with the complaints of painless progressive neck swelling in the right side for one and half year. Sonography, computed tomography (CT), magnetic resonance imaging (MRI), CT neck angiography and fine needle aspiration cytology (FNAC) were suggestive of vacular malformation i.e. giant haemangioma or arteriovenous malformation. The mass was removed in toto under general anaesthesia without postoperative complications. The histopathology confirmed it to be glomangiomyoma with haemangiopericytoma like features.It’s an extremely rare variant of glomus tumour and may be the first report of a glomangiomyoma in the neck of a child. Despite a rare entity, it should be borne in mind during differential diagnosis. Glomus tumour, an uncommon neoplasm, arises from the glomus bodies, cells having resemblance of the modified smooth muscle cells of the normal glomus body. Glomangiomyoma is a rare variant of it. Glomus bodies possess peculiar fibrous perivascular structures and regulate body temperature by functioning as arteriovenous shunts . These aIn 1924, the first description about glomangiomyoma was given by Mason. It rarely occurs extradigitally particulary the neck region. And other extradigital sites where normal glomus bodies may be sparse or even absent, such as the patella, chest wall, bone, stomach, colon, nerve, eyelid, nose, mediastinum, small bowel, rectum, urinary tract, lung, cervix, vagina, oral cavity, mesentery, heart, lymph nodes, larynx, back and trachea have been reported –10. HereThis is a case report of a 3 year old female child presented with chief complaints of swelling in the right side of neck for around 1 ½ years in ENT OPD of Universal College of Medical Sciences, Bhairahawa, Lumbini zone. The onset was insidious and gradually progressive, painless and without aggravating and relieving factors. On examination, there was a 8 X 6 cm, ovoid, nontender, soft swelling, nonpulsatile, mobile in all directions, not fixed to overlying skin, smooth surface and prominent superficial neck veins and the transillumination test was positive. The swelling was extending superiorly at level of right angle of mandible, inferiorly 2 cm below the suprasternal notch, laterally 1 cm behind the posterior border of right sternocleidomastoid muscle and medially in the midline of neck . It was negative for malignancy. Plain and contrast enhanced CT was suggestive of high flow vascular malformation Figs. , 3, 4. MCT neck angiography was also suggestive of vascular malformation- giant haemangioma or arteriovenous malformation (AVM) Figs. , 7.Fig. She underwent excision of the mass under general anaesthesia. Preoperative endovascular embolisation would have been better anticipating the blood loss during surgery but it’s not available locally. Hence, we solely depended upon the ligatures and electrocautery. Intraoperatively, the mass was adhered to sternocleidomastoid muscle extending upto hyoid bone level superiorly and extended inferiorly upto the supraclavicular fossa, feeding vessels from right subclavian, external carotid, common carotid artery, thyrocervical trunk were ligated and also the draining vessels towards internal jugular vein were also ligated and the entire mass was removed in toto Figs. , 9, 10. Postoperatively she was free of complications and also at her subsequent follow ups Fig. . HistopaGlomus tumours are rare neoplasms, found typically in soft tissue of the extremities, notiably in the subungual region of the finger tip. However, extradigital identification have been done in different parts of the body , 6.HistoIn our centre, it was diagnosed with the help of imaging like USG, contrast enhanced CT, CT angiography, MRI, FNAC and the histopathological evaluation.The treatment of choice for glomus tumour is surgical excision. Several sclerosants like sodium tetradecyl sulphate, polidocanol and hypertonic saline has been reported to be effective. Ablative therapy with Argon and Carbon dioxide laser is of potential benefit for small, superficial lesions . Here, w


This confirmed the presence of a fistulous pathway between the gallbladder and the skin.  The patient underwent cholecystectomy surgery and open laparotomy with The natural history of the disease has changed from suppurative cholecystitis with spontaneous rupture to operative external drainage of an abscess2. Early and effective medical and surgical management of biliary tract disease can prevent this rare condition. Stone obstruction of the biliary tree plays a crucial role in the pathophysiology of the development of this condition; intra-gallbladder pressure can increase dramatically due to the obstruction of the cystic duct. The unresolved obstruction of the bile outflow compromise gallbladder wall blood circulation, as well as lymphatic drainage, resulting in necrosis of the gallbladder wall with the fistula formation. Once pierced, the gallbladder may drain into the peritoneal cavity, causing peritoneal localized abscess or the abscess can lead into an external fistula due to its adherence to the abdominal wall3.Spontaneous cholecystocutaneous abscess or fistula is an extremely uncommon complication of gallbladder disease. Less than 100 cases have been described in the literature. The first descriptions of cholecystocutaneous fistula was made by Thelisus in 1670. Later Courvoisier reported 169 cases of biliary fistula in the 19th centuryA 76 year old man was admitted to our University Hospital “Ospedale Vittorio Emenuele” and seen in our surgical department. He presented with a 3 cm tumefaction of the right hypochondrium, surrounded by an erythematous skin area, with small secretion of a yellowing-green material, attributable to a bile leaking . The patAn abdominal ultrasound examination revealed the presence of a lesion in the aponeurotic muscle wall, but the possible underlying pathology was unknown. No other signs of pathology were observed. Routine blood work was normal.Abdominal computed tomography scan with contrast media showed the gallbladder walls had diffuse thickened and blurred edges, and the right and transverse abdominal muscles were almost covered and embedded with minute hypo-dense ailments compatible with relapsing phlogistic processes . Hepatoben block aponeurotic muscle, skin and fistula orifice excision. In order to have a good abdominal wall reconstruction, a properly shaped Prolene Prosthesis was placed using fibrin glue7. The patient received broad-spectrum antibiotics after surgery .The patient underwent cholecystectomy surgery 10 days after diagnosis, with an open laparotomy with Considering the patient’s good general condition and good post-operative course, discharge was on the seventh day post-surgery. Surgical wound re-dressing was made one week after discharge in our facility, where the surgical stitches were removed. Scar appearance was good and without concern. No other dressing was needed and the scar was covered with a bandage. The first follow-up was scheduled 15 days after discharge, second one 60 days from discharge. For both follow-ups, routine blood work and surgical scar checking were performed, the results of which were normal and the scar was healing normally. A histological examination confirmed the diagnosis of chronic cholecystitis with gallstones and cholecystocutaneous fistula.11. Fistulas often represent the result of post-surgical12 or post-traumatic15 complications that generally involve the duodenum (77%) and colon (15%)16.Thanks to the progress made with medical imaging and surgical techniques, biliary fistula is today a very rare pathology20, although there are described cases of spontaneous cholecystocutaneous fistula in the absence of gallstones21. Rarely does cholecystocutaneous fistula evolve into a neoplastic process. Instead, fistula can be a sign of gallbladder cancer20. According to Sibakoti, polyarteritis nodosa with gallbladder vasculitis and prolonged use of high dose steroids can be considered predisposing factors21. Fistulaprimum movens is by cystic duct obstruction, which increases the pressure within the gallbladder, with wall distension and impaired vascularization, resulting in the formation of focal necrosis of the wall with perforation evolution and abscess formation to the surrounding area that will rupture in to the continuous structures. In the present case, the abscess drained through the abdominal wall and the fistulose pathway originated from the bottom of the gallbladder. This area is the most distant from the cystic artery and physiologically the least vascularized and therefore more susceptible to ischemia17.Spontaneous cholecystocutaneous fistula represents a truly exceptional event, as confirmed by the analysis of the literature, which revealed only 28 cases published over the last 10 years . This di47.The external fistular orifice is usually on the right upper quadrant, but other locations have been described, including the left hypocondrium, umbilical scar, right lumbar, and right iliac fossa, and rarely the right gluteus and breast regionhepatobiliary MRI to visualize the biliary tree. Considering that 11% of cholecystitis have concomitant presence of gallstones in the main bile duct, it is advisable to perform endoscopic retrograde cholangiopancreatography (ERCP)29. In our case CT with CM and hepatobiliary MRI confirmed the fistula presence and it was not necessary to execute the ERCP before surgery. Although an intraoperative cholangiogram was performed to check that the bile ducts were clear from gallstones. Cholecystocutaneous fistula has always been treated by two different strategies. The first includes a two-step approach: percutaneous drainage and antibiotic therapy, and subsequently cholecystectomy. The second directly involves laparotomy cholecystectomy execution withen block aponeurotic muscles, as well as skin and fistula orifice excision.The diagnostic process always begins with upper abdomen ultrasound and ends with29. The second strategy is the most commonly used since the two-step approach treatment is reserved for patients with sepsis and poor general condition30. In 1998, Kumar described the first case of gynecological fistula treated with laparoscopic technique, proposing to the scientific community the feasibility of this innovative approach34.Rarity of this pathology confirms the great quality of progress made by early diagnostic techniques and medical treatment to prevent complication of cholethiasis. Although cholecystocutaneous spontaneous fistula is not common, it can lead to a serious condition. If not quickly treated, it can rapidly evolve into a generalized septic state with severe impairment prognosis. In our case, the patient was in good health arguably because the fistula was draining the most of the abscess outside the body and not in the peritoneum space. Surgical treatment was, however, essential to restore the physiologic bile flow and adequate broad-spectrum antibiotic prophylaxis lowed the risk of post-operative infections. Although laparoscopic approaches have been described since 1998, this pathology is, in most cases, continuing to be treated with open technique, most likely because it is easier and with fewer risks of post-surgical complicationsWritten informed consent was obtained from the patient for the publication of the patient’s clinical details and related images. The article Case Report: Spontaneous cholecystocutaneous fistula, a rare cholethiasis complication covers a subject that nowadays is quite uncommon. It goes through the background of the case’s history, it provides details of diagnostic tests, treatment given and outcomes reached. It could be useful for other practitioners. It can be accepted for indexing with minor English revision.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. My compliments to the authors for the interesting and rare case report. I approve it for pubblication in F1000 Research. Please give more explanation for the 10 days delay between patient admission and operative surgical management. On the basis of rare case report the authors should clarify the operative technique with more details.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. The authors should clarify the reasons for the 10 days delay in the operative management of their patient. More insight should be provided on the choice of aggressive operative treatment as compared to a percutaneous drainage. Perhaps the drainage would have been more readily available and also less expensive as compared to elective surgery. Keeping in mind the age of the patient, it might have been a reasonable option proceeding with conservative management and percutaneous drainage trial. Did the authors investigate the use of somatostatin (provided in the form of Octreotide) in such case? It is currently a first line approach for uncomplicated pancreatic-cutaneous fistulas and might as well work with the Gallbladder considering the physiological functions of the hormone. All in all the case is interesting and, considering the rarity of the event, it deserves publication.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.


Sarcomas account for less than 1% of malignant neoplasms arising in the head and neck in adults. Laryngeal synovial sarcoma is an extremely rare form of laryngeal malignancy with less than 20 cases reported in the literature. We report the case of a 48-year-old man with synovial sarcoma of the larynx. He underwent excision of the tumor followed by radiation. He is alive in remission at 36 months. The literature on synovial sarcoma of the larynx is reviewed.  Sarcomas account for less than 1% of malignant neoplasms arising in the head and neck in adults and less than 5% of soft tissue sarcomas in adults occur in the head and neck region . Larynx A 48-year-old man presented with a history of hoarseness of voice since 5 months, dyspnea since 3 months, and dysphagia since 2 months. He consulted an ENT surgeon where a fibre optic laryngoscopy showed a pedunculated mass occupying the laryngeal inlet and extending up to base of tongue . A compuLaryngeal synovial sarcoma is an extremely rare form of laryngeal malignancy with less than 20 cases reported in the literature  is an important adjunct in the treatment of soft tissue sarcoma to diminish the incidence of local recurrence . The majAdjuvant chemotherapy has been utilized for high grade synovial sarcoma. Doxorubicin and ifosfamide have been shown to demonstrate improvement in disease specific survival in the treatment of soft tissue sarcomas , 32. OurIn summary, synovial sarcoma of larynx is a rare entity. Surgery is the mainstay of treatment with conservative surgery and organ preservation considered for early cases. Postoperative radiation is reserved for those with positive margins and high grade tumors and chemotherapy for high grade tumors.


Mucoceles represent one of the most common lesions of the oral cavity, developing as a result of saliva accumulation. The most frequent affected area is the lower lip, followed by floor of mouth, ventral tongue and buccal mucosa. Despite numerous reports of mucoceles originating on the ventral surface of the tongue, only scarce cases of such a lesion identified on the dorsal tongue surface have been described. In this report a mucocele developed on the dorsal tongue of a 74-year-old woman is described. Additionally a review of previously published mucoceles of the dorsal surface of the tongue is provided and discussed.A 74-year-old female patient was referred for a painless swelling on the dorsal surface of the tongue of 1 month duration. Possible clinical diagnosis included granular cell tumor and lingual thyroid gland. Proper blood testing for TSH, T3 and T4 as well as ultrasonography were requested, found to be within normal limits. An excisional biopsy was performed and tumor was removed with no intra-operative complications.Histopathological examination was consistent with a mucocele, exhibiting an amorphous material surrounded by granular connective tissue without epithelial lining on the periphery. Patient was examined on regular follow-up basis, with no signs of recurrence for the last 1 year.Mucoceles of the dorsal tongue surface represent rare clinical entities, necessitating the need for further case reports to be published in order to widen our understanding of their clinical features. Key words:Mucocele, oral cavity, tongue, minor salivary glands, oral and maxilloafcial pathology. Mucoceles develop in the oral cavity as a result of saliva accumulation, causing swelling of the affected area. These entities represent one of the most common lesions of the oral mucosa while they are likely to grow at a lesser frequency in other parts of the human body such as the nasal cavity and maxillary sinus .They are mostly subdivided into two categories: a. Mucus extravasation type; rupture of salivary glands’ duct as a result of injury can lead to leakage and concentration of saliva in the surrounding tissues. In this way a cavity is formed within the soft tissues, not surrounded by epithelial wall lining, developing a pseudocyst, and b. Mucus retention type; obstruction of the salivary duct leading to a true cyst covered by epithelium. About 90% of such lesions are classified as extravasation mucosal cysts while only 10% as mucous retention cysts .The incidence of oral mucoceles is difficult to be estimated due to the fact that a large percentage of these lesions are not referred for histopathological examination.It should be highlighted that mucoceles may develop at any age. Nevertheless, among the studies, a higher incidence of both the 2nd and the 3rd decade of life is reported with no sex predilection. Importantly, among the 2 subtypes, the mucous extravasation cyst occurs at younger ages compared to those from retention  . NormallAccording to collected data, a wide age range of patients ranging from birth to 74 years old was recorded.Mucous cysts were identified in the anterior as well as in the posterior area of the dorsal surface of the tongue, exhibiting no predilection.et al. suggested that a mucous cyst identified in a newborn most likely originated from an anterior lingual gland which drained through a small, closed ended, duct into the dorsal tongue (Regarding their aetiology, we can speculate that lesions of the anterior dorsal tongue may develop from Blandin-Nuhn glands while those of the posterior area may derive from Weber and von Ebner salivary glands. Wong Chung l tongue . In our According to the histopathological criteria there were 3 mucous extravasation cysts, 2 mucous retention cysts while in 1 case there were no data available specifying its type.Mucoceles on the posterior surface / base of the tongue are possibly more challenging to diagnose, necessitating special diagnostic examinations e.g. laryngoscopy while those in the anterior surface usually require a thorough clinical examination with no further exams. Also those lesions in the tongue base were found to provoke more severe symptoms including dysphagia and difficulty in swallowing while they were all removed under general anaesthesia.Although the limited number of intraoral mucoceles developed in the dorsal surface of the tongue precludes definitive conclusions, abovementioned discussion includes author’s remarks / observations from the collected cases.Mucoceles of the dorsal tongue surface represent rare clinical entities, due to the fact that there are no salivary glands located on this area. It seems that they develop due to pathophysiologic mechanisms, that further require to be discovered. Due to their scarce description the need for future case reports to be published is imperative in order to widen our understanding of their clinical features.


The goals of sarcoma management include both a cure and the functional preservation of involved tissues and adjacent critical structures with common opinions favoring immediate reconstruction. The question arises whether these goals are contradictory. This paper discusses the question based on the experience of 28 patients with different types of extremity sarcoma, with 24 surgically treated by the University of California San Diego (UCSD) orthopedic and plastic surgery team (2011–2016) and the collection of evidence from published practice guidelines, reviews, case studies, and clinical trials. Included are the impact of limb-sparing and functional reconstructive concepts, efforts regarding the adequacy of surgical margins, and the rationale of immediate versus delayed reconstructive approaches, and the disease-free status of sarcoma management. To adequately address upper extremity sarcomas, and the ability to cure and functionally preserve the extremity, a multimodal and complex, function-conscientious management is often offered. The clinical evidence points out that surgical intervention and adequate excisional margins are the key elements to reducing morbidity and mortality as a consequence of treatment or disease –3. WhileFunctional results of immediate major reconstructive procedures can be impressive, and they are technically possible in an immediate single-stage setting; however, there are reports regarding errors in and problems with the diagnosis which precipitate a cautious and delayed approach to reconstruction. In these cases, where the diagnosis was not firmly established or confirmed, or where the emphasis was set on sparing the extremity instead of focusing on a long-term cure or patient survival under an established diagnosis, delaying definitive reconstruction should be thoroughly considered. A review of material referred to the multispecialty sarcoma treating center revealed 37% of the histopathological diagnoses change upon histopathological evaluation, including the grade of the sarcoma, and 82% of margins, previously declared as negative, return positive . It is fFor the multidisciplinary plastic and orthopedic surgical team, the prospect of the best functional outcome is not only related to the objective of anatomical extremity salvage, but also to the ability to perform complex and definitive anatomical and functional reconstruction. In other words, if performed without a certain diagnosis or clear margins, or in the event of a change in the clinical situation, the possibility that such a reconstruction might have to be revised or discarded in the future presents an argument for delayed reconstruction or at least for a customized approach with defect temporization until all necessary information and parameters are known.Resecting sarcoma tumors to ensure negative surgical margins can be challenging. A full histopathological assessment including tumor grading may require time and may not be completed intraoperatively. A negative pressure vacuum-assisted wound closure (VAC) system sealing the defect for a few days after resection enables complete histopathological assessment of the margins prior to reconstruction, while also preserving acute surgical wound characteristics which enables a delay or staged reconstruction while still maintaining the advantages of immediate-like reconstruction. Some advocates of vacuum-assisted wound closure (VAC) devices for post-sarcoma-resection wound size reduction, granulation tissue induction for delayed or simple forms of repair, miss the by far most important VAC advantage: the fact that it can buy the necessary time for a full oncological wound assessment and the finalization of the reconstructive management planning. Using a VAC-system maintains not only acute wound biology, while retaining technical reconstructive options for immediate reconstruction but also enables the delivery of immediate brachytherapy followed by definitive reconstruction , 11.On the other hand, there are clinical scenarios that necessitate immediate-definitive soft tissue reconstruction. Post-ablative or post-amputative defects may be so massive that some form of immediate reconstruction is mandatory. Sometimes post-amputative extremity parts may be utilized as donor tissue for a flap. In such instances, there is no other technical maneuver or choice other than to proceed with immediate reconstruction , 11. AddThe authors’ combined database includes 28 patients with advanced or complex upper extremity reconstruction defined as those necessitating multidisciplinary planning and surgical interventions (resection by the Orthopedic team and reconstruction by the Plastic Surgery team). The 24 patients  were treated surgically between January 2011 and October 2016 with an average age of 45 years (range from 12 to 68 years). The tumor types within the study population are presented in Encrypted records of the multidisciplinary treatment planning conferences were reviewed blindly by two physicians (an orthopedic and a plastic surgeon) not involved in patient care. They analyzed individual patient age at diagnosis, sex, tumor site, type and duration of signs and symptoms, type of previous treatment (if any), initial diagnosis, local recurrences, regional or distant metastatic involvement, and the outcome as of November 2016. For each individual case, the treatment planning conference was re-enacted and factors determining the choice (timing) of post-resection reconstructive strategy were retrospectively analyzed. Specifically, utilizing the concept of decision analysis along a continuum regarding diagnostic certainty , and how predictable an intraoperative situation would be regarding the threshold for immediate reconstruction versus the need for further testing accompanied by delayed reconstruction with preparational wound temporization was determined  13–16].–16.13–16In eight cases  resection was followed by immediate-definitive reconstruction, while in 16 cases the definitive procedure was delayed. Whenever possible during the original treatment planning, immediate-definitive reconstruction, concurrent with ablative or amputative procedures, was offered. Four of them were treated for recurrent disease. Retrospective analysis revealed that all of these patients were categorized as meeting the threshold points 100%. The follow-up (from one month to five years) revealed fatal progress of the disease in five cases , while the remaining patients appear to be disease free. All five patients underwent immediate reconstruction to cover critical defects, and to accommodate the placement of brachytherapy catheters within the flaps (shoulder sarcoma). One case entailed defect coverage and functional restoration of elbow extension in an elbow-area defect  17]. Th. Th17]. Retroactive re-review of all cases revealed that, based on information at the time of original treatment planning , plans would be affirmed.per se or non-established clear or safe margins) during the original pre-surgery treatment planning conference. In three cases, delayed reconstruction was performed because the diseased area was infected. The remaining case was offered delayed reconstruction because of the need for tumor grading and determination of how to complete the reconstruction (ultimately the patient underwent amputative surgery due to the suspected progression to a high-grade undifferentiated liposarcoma).Of the 16 cases that underwent delayed reconstruction, 12 had been offered delayed reconstruction because of diagnostic uncertainty , segmental cortical bone resection was performed with autologous iliac crest bone grafts . No segmSurgical guidelines for the treatment of upper extremity sarcomas evolved from amputative approaches to limb-sparing designs, with – if possible – immediate functional restoration procedures with neo- and adjuvant multimodal complementary treatments. Although, the lowest rate of recurrence is found in patients with amputative or compartmental  resections, wide radical excisional surgery may result in a significant loss of tissue, especially if the tumor is large in diameter, crosses fascial planes, and/or invades neurovascular structures of extremities without survival benefit , 12–14. sine qua non for optimal long-term results. The definition “how much margin is safe” evolved on the basis of a better understanding of the biology of malignancies, utilizing the staging and grading systems while planning the management. As the expansion of available management options for advanced extremity malignancies, both surgical, including rehabilitative, and non-surgical therapeutic modalities, has increased the complexity of clinical decision making and is becoming more complex requiring specialized, multidisciplinary teams [Safe surgical margins are a condition ry teams , 13, 14.Surgeon consensus regarding the overall management plan for the group of patients analyzed in this study was 100%. Professional specialty and experience influences the treatment sequence preference with radiation oncologists, and practitioners experienced in sarcoma management lean toward preoperative neoadjuvant therapy while surgical intervention itself is not questioned  and 3 1. UsuallyThe pivotal element of reconstructive planning, with the ability to be controlled by the patient and the surgeons, are surgical margins and the commitment to immediate or delayed definitive reconstruction. Concerns about false negative margins and the general difficulty in intraoperatively assessing large, three-dimensional fields, confirm the reasoning regarding the delay of complex flap reconstruction or a staged approach. Immediate flap reconstruction and anatomical tissue rearrangement during the reconstruction may make secondary tumor bed re-excision – if indicated – very challenging. Therefore, it should not be a surprise that the majority of reconstructive dilemmas evolved from uncertain disease diagnoses, to margins, or issues regarding the timing of the reconstruction , 16. OneNew imaging techniques may be useful in avoiding “one-size-fits all” management strategies, determining safe margins, and evaluating the responses to neo adjuvant and adjuvant treatments. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) is a recently introduced tool that is helpful in mapping sarcoma affected tissues . Modern One case very vividly demonstrated the importance of being cautious and yet failing. The defect in the patient’s right arm after wide excision of the tumor with histologically clear margins was covered with Integra , a temporary wound coverage, while awaiting the results of tumor grading (margins were clear but the tumor turned out to be of high grade) . A VAC-sDefinitive closure may entail complicated reconstructive procedures which may need to be revised or re-radiated in the setting of positive margins or recurrent disease. In fact, the management of such situations is not well defined. It is unclear which approach is optimal to further limb salvage in case of a relapse near a prosthesis, allograft, or under a complex , 21. OneLimb-sparing and defect reconstruction in the treatment of primary bone sarcomas or sarcomas grossly involving bone is sometimes an alternative to amputative surgery . For marIn addition to functional objectives, reconstructive extremity surgery designs include the consideration of aesthetic aspects regarding the surface and the volumetric characteristics of a graft or a flap. For the correction of soft tissue contours, autologous fat transfer has been recently proposed as an option. Therefore, it is worthy to bring up some words of caution. A local recurrence of osteosarcoma, which occurred 13 years after the primary tumor removal, was reported following fat injections for contour improvement, with some evidence suggesting that fat grafts or progenitor cells may promote tumor growth .modus operandi of the orthopedic-plastic surgery team treating advanced upper extremity malignancies.Relative concordance is notable between the decisions regarding immediate versus delayed definitive upper extremity reconstruction and the reconstructive technique chosen at the time of pre-resectional planning or intraoperatively. Appreciation of difficulties in establishing truly negative margins should lead to a customized and individualized approach for the reconstructive management of each patient. The recognition of the value of staged or delayed definitive reconstruction should be the MD and GAM certify that they have no financial conflict of interest  in connection with this article.


There are few reported cases of flexor tendon sheath ganglion arising from the A2 pulley. We report a case of a flexor tendon sheath ganglion in a 17-year old female who presented with pain, triggering and a swelling at the base of her right ring finger. During the excision biopsy, a ganglion measuring 0.5×0.8×0.4 cm in size was removed from the A2 pulley area. Ganglion is the most common soft tissue tumour of the hand, representing approximately 50% to 70% of all upper extremity swellings but the exact aetiology remains obscure.2 The female to male ratio of ganglions in the flexor tendon sheath has been reported as 2.6 to 1 and is predominant in females in their third and fourth decades of life. The A2 pulley is rarely involved.2 We report a case of a ganglion originating from the flexor tendon sheath at the A2 pulley.Ganglions are commonly found in specific locations on the hand, wrist and feet. They are usually soft, solitary and asymptomatic, occasionally causing pain and neurologic symptoms if they are located adjacent to a peripheral nerveA 17-year old girl presented with pain on the palmar aspect of the metacarpo-phalangeal (MCP) joint of her right ring finger for the past few months. She was a pianist and had no significant past medical history. She complained of pain on finger flexion, and mild triggering was observed at the proximal interphalangeal joint (PIPJ). A small cystic mass was palpable in the subcutaneous layer over the proximal finger flexion crease. The mass was fixed to the underlying structures during flexion and extension of the affected digit. The excision of mass was done via a transverse incision parallel to the proximal finger flexion crease under loupe magnification. The mass was located on the proximal part of A2 pulley  & 2. No Ganglions have been recognized since the time of Hippocrates, who suggested that they represent mucoid flesh, thus differentiating them from ganglions of neural origin. Although much has been written about this common hand tumour, little attention has been given to flexor tendon sheath ganglions, which are often referred to as volar retinacular cysts or “sesamoid” or “pearl-seed”ganglia. This seems particularly surprising given that they are relatively common and often result in significant discomfort and loss of normal hand function.1. Eller suggested that they originate from a localized rupture within the flexor tendon sheath2. Doyle believed that they result from a rent in the synovial membrane that heals but subsequently leaves an extracapsular piece of membrane that continues to secrete synovial fluid. Nelson et al. have suggested a traumatic aetiology, with a preceding injury occurring in approximately half of all patients2. However, the findings in our case are similar to others in that we could not find a clear association between antecedent trauma and the subsequent development of a flexor tendon sheath ganglion.The aetiology of the flexor tendon sheath ganglion is unknown3. As has been reported with ganglions located in other areas of the hand, flexor tendon sheath ganglions seem to occur more commonly in women. The greater prevalence of young women with flexor tendon sheath ganglions has been attributed to this population’s concern about cosmesis3. This seems unlikely, given that a flexor tendon sheath ganglion is usually quite small and inconspicuously located on the palmar surface of the hand3. In our patient’s age group, cosmesis was not a major concern, which has been similarly noted by others.Involvement of the flexor tendon sheath occurs in approximately 5 to 16% of all hand and wrist ganglionset al. Certainly, the level of disability in the patient with a ganglion of the flexor tendon sheath can be disproportionate to the size of the cyst. However, the resulting discomfort did not appear to cause our patient to urgently seek care considering that the symptoms were present for about nine months prior to presentation. This delayed presentation has also been noted by Matthews, who reported approximately 10 months of symptoms prior to presentation in his series. Our patient noted associated triggering of the affected digit. The association between stenosing tenosynovitis (trigger finger) due to ganglion at the A2 pulley has not been previously reported4.Our patient presented with the clinical complaints of a painful, tender mass. The mass was small and firm in nature. The resulting discomfort was particularly troublesome with grasping activities such as holding a steering wheel or lifting objects. She also reported that the pain interfered with her hand function. Flexor tendon sheath ganglions may be painful because of local neural innervation of the cyst, which has been demonstrated histologically by DeOrsay 5. Theoretically, it should not be as successful for flexor tendon sheath ganglions because of their small size and tenderness. Radiation therapy was advocated in the early 1940s, but this modality was no longer utilized because of concerns regarding the long-term sequelae of radiating the hand5. Transfixion with a loop of catgut suture had also been advocated, with a reported failure rate of only 1%. Aspiration of the cyst followed by injection with sclerosing agents such as iodine, carbolic acid, or ethanolamine had also been utilized but no longer advocated because of the risk of soft tissue necrosis5. Currently, the most popular treatment method involves aspiration with steroid injection but the risk of recurrence is high. Excision has been advocated because of the potential for digital nerve injury and the high recurrence rates reported with aspiration5. However, the results of surgical excision of a flexor tendon sheath ganglion are difficult to interpret because most series have grouped them with other hand and wrist ganglions. There are only two studies that have specifically assessed the results of excision of flexor tendon sheath ganglion. Bittner et al. reported a recurrence in only one of their 43 patients who underwent cyst excision5. Resection with a surrounding portion of the flexor tendon sheath is recommended to minimize the chance of recurrence because it has been suggested that recurrence is the result of inadequate removal of all degenerative myxoid tissue. We have confirmed that surgical excision of a small part of the distal aspect of A2 pulley does not cause any bowstring of the flexor tendon and results in a low recurrence rate, minimal complications, and high patient satisfaction.A variety of treatment modalities had been advocated for ganglions in general. Manual compression was the simplest yet least effective treatment approach


Gastrointestinal duplications can affect any part of the alimentary tract and are notorious for their variable presentation. Their association with malrotation and midgut volvulus is rare. We describe an 8-year old boy presented with episodes of abdominal pain. Radiological workup showed whirlpool sign and abnormal relationship of mesenteric vessels. At operation, malrotation with chronic volvulus was found. Incidentally, a jejunal communicating duplication cyst was also noted. An 8-year-old boy presented with on and off abdominal pain for four months which increased in frequency and intensity 24 hours before presentation. Pain used to worsen on oral intake and was occasionally associated with non-bilious vomiting. There were no other significant symptoms. He had been evaluated elsewhere with a normally reported contrast follow-through study done three months earlier.On examination patient weighed 20 kg and was dehydrated. There was no pallor and the patient was afebrile. Abdomen was soft, non-distended and non-tender without any palpable mass. Bowel sounds were increased. Per rectal examination was normal. After admission, he had episodes of agonizing abdominal pain. Nasogastric tube revealed non bilious aspirate. Routine blood investigations were within normal limits with baseline hemoglobin of 10.4 g/dl. Abdominal x-ray did not reveal any pathology. Abdominal ultrasound (USG) showed positive whirlpool sign. CT scan of the abdomen confirmed the USG findings and showed the abnormal relationship of superior mesenteric vein and artery . Considering the acute symptoms and the radiological findings, patient was taken up for emergency surgery. At laparotomy, midgut volvulus with malrotation was found. Derotation of the twisted small bowel loops was done. Bowel loops were healthy and viable. Thick Ladd’s bands with narrow small bowel mesentery were present. Ladd’s bands were divided and mesentery widened. Approximately 40 cm distal to the duodeno-jejunal flexure, a 10cm x 5cm tubular jejunal duplication on the mesenteric side was found . It communicated with the normal jejunum through a narrow opening. Resection of the involved segment with end to end jejuno-jejunal anastomosis was performed. Resected specimen was sent for histopathological examination. Postoperative period was uneventful. Patient has been asymptomatic in the follow up period. Histopathological examination showed normal small intestinal features with ectopic gastric mucosa.Although both malrotation and gastrointestinal duplications are important causes of intestinal obstruction in the paediatric age group, finding them in the same patient has been described to be rare in literature. [1- 4] We could not find the exact incidence of this association.Despite having both malrotation and jejunal duplication, our patient seldom experienced any symptom before four months of presentation to the hospital. Chronic malnutrition leading to low weight for age and repeated episodes of pain abdomen can both be explained by chronic volvulus and malrotation.[5] Even when not causing frank Intestinal obstruction, malrotation has been found to delay gastric emptying and aggravate gastro-esophageal reflux.[6] This mechanism can explain non-bilious vomiting and aspirates in nasogastric tube. Jejunal duplication could also have added to the pain and relative intestinal obstruction. No pallor or history of melena signifies that the patient did not have bleeding despite the presence of ectopic gastric mucosa. It is possible that during episodes of volvulus the involved midgut emptied to some extent into the communicating duplication and derotated after becoming less bulky. Possibly, this mechanism of relief of obstruction may be the cause for the child to be asymptomatic till this age and also for the non-bilious vomiting and aspirate. Unfortunately, the patient did not have images of the contrast study done outside and it could not be repeated due to his acute symptoms otherwise a communicating duplication could be picked preoperatively. Finding of whirlpool sign on imaging and abnormal relationship of mesenteric vessels led us to preoperative diagnosis of malrotation and volvulus.We concluded that malrotation and jejunal duplication have rare association and USG coupled with a carefully done contrast study is indispensable in such cases to delineate the abnormal anatomy preoperatively.Source of Support: NilConflict of Interest: None declared


Kedney matrix stones are a rare form of calculi. Flank pain and urinary tract infections (UTI) are the most common presentations of matrix calculi. The diagnosis is usually made at surgery, but some preoperative radiographic findings might be suggestive. Open surgery was the method of choice for treatment. However, combination of ureterorenoscopy and percutaneous nephrolithotomy (PCNL) was found to be safe and effective. We report a rare case of renal and ureteral matrix stones that were diagnosed and treated by open surgery. We also describe its clinical, radiological and therapeutic features through a review of the literature. Kidney matrix stones (KMS) are a rare form of urinary calculi, presenting a diagnostic and therapeutic dilemma to the practising urologist . By contMr A.A. 45 years old, without medical history, complains of right back pain with several episodes of pyelonephritis. Clinical examination was normal. The urinalysis revealed a urinary infection due to Escherichia-coli. The renal function was impaired (Creatinine = 25 mg / l) . X-ray showed no calcium Image of the urinary tract. Ultrasound shows an important right hydronephrosis with multiple slightly echogenic formations in the renal pelvis. CT found an hydronephrosis reducing the renal parenchyma secondary to several large ureteric and caliceal obstructive stones with a low density (520-680 UH) . The lefKMS also known as fibrinomas, colloid calculi or albumin calculi, are a rare form of calculi first described in 1908 by Gage and Beal . The matThe computed tomography (CT) appearance of KMS varies, depending on mineral volume, composition and internal distribution. Some authors described egg-shaped matrix stones with a mineral rim and soft tissue center . MRI shoKMS are a rare entity. The diagnosis is usually made at surgery, but some preoperative radiographic findings might be suggestive. Open surgery was the method of choice for treating these patients in the past. However, recently it was replaced by endourological intervention. Washing the pelvis and prophylaxis with antibiotics are believed to be effective to avoid recurrences.


To present a coincidence of macular telangiectasia type 2 and solitary retinal astrocytic hamartoma in one patient.A 50-year-old woman was examined in the Department of Ophthalmology of University hospital Kralovske Vinohrady for complaints of metamorphopsia in her left eye. Her uncorrected visual acuity (VA) was 4/4 on Early Treatment Diabetic Retinopathy Study charts (ETDRS), on the retina of her left eye white, prominent, partially calcified tumour 1 disc diameter in diameter, 1,5 disc diameter from the foveola was detected on the retina. In the macular region of both eyes, parafoveal greying with crystalline deposits and changes in retinal vasculature were visible. We performed following examinations: fluorescein angiography (FA), B-scan ultrasound, spectral domain optical coherence tomography (SD-OCT) including photo documentation. FA showed partial hyperfluorescence of mulberry-like surface of the tumour typical for retinal astrocytic hamartoma. Parafoveally in both eyes, leakage from parafoveal telangiectasia was apparent. SD-OCT showed cystoid space in the macular region of both eyes as well as changes in inner and outer photoreceptor segment junction in left eye. SD-OCT of the tumour showed proliferation in retinal nerve fibre layer with normal structure of underlying retinal layers and choroid. Ultrasound examination of the tumour detected solid, highly echogenic prominent tumour with high reflectivity and acoustic shadow.A coincidence of two relatively rare clinical units, macular telangiectasia type 2 and solitary astrocytic hamartoma was detected as a unique and rare observation. Retinal astrocytic hamartoma is a benign tumour formed through proliferation of well differentiated astrocytes, mulberry-like based on ophthalmoscopy. It is most common in paediatric patients with tuberous sclerosis or neurofibromatosis . These tIn February 2014, a 50-year-old Caucasian female patient was referred to our department due to suspected chorioretinitis in the left eye. The patient reported metamorphopsia in the left eye lasting about 6 months. Both personal and ocular histories were free of any noteworthy facts. VA was 4/4 (ETDRS) natural in both eyes; intraocular pressure was 26 mmHg in the right eye and 23 mmHg in the left eye. The finding in the anterior segment of both the right and left eyes was quite physiological when examined using the slit lamp. Biomicroscopic examination of the fundus of the left eye revealed a clear, prominent whitish tumour with calcification at the centre Fig.  sized 1 Considering that currently no efficient therapy of macular telangiectasia type 2 is known, and given the stability of the astrocytic hamartoma without any signs of growth after 18 months of follow-up, the patient is only actively followed without any therapy for now.In most cases, astrocytic hamartomas are benign lesions showing no tendency to grow and cause complications and without any need for a therapeutic intervention. The ophthalmological presentation is very variable ranging from flat to elevated lesions of whitish or yellow colour. They tend to be slightly opaque or transparent, with not very well defined boundaries. Calcifications are common in the lesion. Spontaneous regression of the tumour has also been observed , 4. HoweFluorescein angiography clearly shows a network of small capillaries of the tumour in the arterial and venous phase, later accompanied by its homogeneous hyperfluorescence , 14, gaiAs regards differential diagnosis, several additional modalities should be considered. In particular, other glial tumours of the retina are concerned – presumed solitary circumscribed retinal astrocytic proliferation (PSCRAP) and acquired retinal astrocytoma .PSCRAP is found in elderly patients without a history of tuberous sclerosis or neurofibromatosis. Compared to astrocytic hamartoma, such lesions are usually well circumscribed, unilateral flat formations, shadowing deeper retinal structures. They do not include calcifications. SD-OCT scans clearly show their origin in the nerve fibres layer with the shadowing of deeper retinal layers. No edema is usually present at the lesion site or in the macula. They are stable lesions without any tendency to grow and cause complications .Acquired retinal astrocytoma is found in adult patients without a history of tuberous sclerosis or neurofibromatosis . It is aMacular telangiectasia type 2 is a progressive bilateral disease of the macula of unknown etiology, with characteristic alterations of the macular capillary network and with the formation of parafoveolar telangiectasias; in later stages it shows dilated capillaries and retinal atrophy . Other cConcurrence of astrocytic hamartoma and telangiectasias has been described . HoweverConcurrent incidence of solitary astrocytic hamartoma of the retina and of macular telangiectasia type 2 was present in our patient. We could not find any evidence of any relationship between these two diseases in available literature (PubMed). In this case, it is only an accidental concurrence of two diseases given that unilateral retinal astrocytic hamartoma is combined with a bilateral finding of macular telangiectasia type 2.


Dear SirAlimentary tract duplications are epithelial-lined cystic or tubular structures attached to bowel wall and supplied by mesenteric vessels. Association between intestinal malrotation and duplication cyst, especially of jejunal location is rarely mentioned in literature[1-5]. We are reporting a rare coexistence of isolated jejunal duplication cyst and malrotation in a neonate with intestinal obstruction.A 15-day-old male neonate was brought to emergency for multiple episodes of bilious vomiting. The baby was born full-term by spontaneous vaginal delivery at home and antenatal ultrasonography(USG) was not done. At presentation, he was 2500 gm, moderately dehydrated, pulse 160/min and respiratory rate was 46/min. Resuscitation was started immediately and the nasogastric tube inserted. Abdomen was soft, non-tender and a mobile mass was palpable in left upper abdomen. Plain X-ray abdomen showed distended stomach and duodenum. Rest of the abdomen was gasless, but there was presence of rectal gas in pelvis . USG revealed a well defined thick walled cystic lesion in left mid abdomen measuring 42х37mm suggestive of duplication cyst . Doppler imaging showed "Whirlpool sign" in epigastric region with superior mesenteric vein (SMV) curving from right to left of superior mesenteric artery (SMA) suggesting midgut malrotation . At surgery, a non-communicating jejunal duplication cyst, intimately adherent to jejunal wall and compressing the intestinal lumen was found . Malrotation was also present which was corrected. Duplication cyst was excised along with a portion of adjoining jejunum. End-to-end jejunal anastomosis was done. Postoperative period was uneventful. Combination of enteric duplication and intestinal malrotation is a rare entity and may present as an acute surgical emergency [1-5]. Gastric and duodenal distension on plain X-ray of abdomen is suggestive of duodenal obstruction. The presence of rectal gas in addition to dilated stomach and duodenum will commonly point towards partial duodenal obstruction. This can occur in malrotation due to compression of distal duodenum by Ladd's bands [3]. Similarly our preoperative suspicion was malrotation on plain X-ray abdomen. Color Doppler imaging showing Whirlpool flow pattern of SMV around SMA is suggestive of midgut malrotation. Ultrasonography is very reliable in diagnosing duplication cysts.It will show an inner hyperechoic rim of mucosa-submucosa and an outer hypoechoic rim of muscular layer [2]. Duplication cysts are thick walled because it is composed of smooth muscle and mucosa as opposed to other types of cysts such as mesenteric and omental cysts. Our preoperative diagnosis coincided with operative findings.Surgical removal of duplication cyst preserving normal bowel is possible in some cases, but is extremely difficult [1]. Resection of duplication with the native bowel is the usual procedure due to common blood supply . This case represents a rare coexistence of two congenital malformations of GIT. Ultrasonography is very helpful in detecting both duplication cyst and malrotation.Source of Support: NilConflict of Interest: None