Congenital Absence of the Common Bile Duct - A Rare Anomaly with an Evolving Association with Esophageal Atresia

Congenital absence of the common bile duct (CBD), also known as âcholecystohepatic ductâ or âinterposition of the gallbladderâ is a rare extrahepatic biliary anomaly characterized by the hepatic ducts entering the gallbladder directly and the cystic duct draining the entire biliary tree into the duodenum. There have been only been four reports of children with congenital absence of the CBD, with three of these also carrying a diagnosis of esophageal atresia, suggesting an association between the two entities. We report an additional case of a child with a history of tracheoesophageal fistula and congenital absence of the CBD as well a review of the literature. Keywords: Gallbladder interposition, Biliary tract anomaly, Pediatric cholecystitis, Acalculous cholecystitis, Roux-en-y hepaticojejunostom

Desmoplastic Small Round Cell Tumor with Primary Ovarian Involvement: Case Report and Review

Background. Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, malignant neoplasm that has recently been characterized. It has not been associated with a primary visceral organ. In women, cases are even more rare and often have some ovarian involvement. Case. An 11-year-old girl presented with abdominal pain, nausea, and vomiting. A CT scan revealed a large heterogeneous pelvic mass with cystic components and an 8-cm midabdominal mass. During exploratory laparotomy, the patient was found to have a pelvic mass measuring 12.9 cm replacing normal ovarian tissue. The midabdominal mass was also removed. Pathology, cytology, and immunohistochemistry confirmed a desmoplastic small round cell tumor. Even with aggressive surgical and medical intervention, the patient died 11 months after initial diagnosis. Conclusion. We present a rare small cell tumor that is associated with ovarian involvement. The prognosis in these patients is extremely poor and very few survivals have been reported

Lupus-associated vasculitis manifesting as acute appendicitis in a 16 year old girl

<p>Abstract</p> <p>A 16 year old female with systemic lupus erythematosus presents with acute appendicitis. Final pathologic analysis of the appendix describes a lupus-associated vasculitis.</p