The report presents a rare case of carcinoid tumor in a 17 year female who presented with epigastric pain of one week duration. She was diagnosed to have type I choledochal cyst on abdominal ultrasound and MRI. She underwent total excision of choledochal cyst with roux-en-Y hepaticojejunostomy. Histopathological examination revealed a neuroendocrine tumor within choledochal cyst which was immunoreactive for Chromogranin A. Patient is well at 6 months of follow up. These tumors are characteristically slow-growing, therefore awareness of its presence preoperatively can facilitate optimal management by performing surgical resection with negative margins which offers the best chance of long-term survival.
Type IVa choledochal cysts with cylindrical dilatation of the intrahepatic ducts constitute a relatively less recognized variety of choledochal cysts, and differ from cystic dilatation of intrahepatic ducts in their clinical manifestations and response to treatment. Five patients with type IVa choledochal cysts and cylindrical dilatation of major intrahepatic ducts who underwent cyst excision and Roux-en-Y hepaticojejunostomy are reported. The duration of symptoms was less than 1 year in all patients. Palpable abdominal mass and abdominal pain were present in 3 patients. The traid of jaundice, abdominal pain and mass was present in only 1 patient. The intrahepatic dilatation regressed after excision of the extrahepatic cyst just below the hilum of the liver. The surgical technique is described and the need for excision of the cyst is emphasized.
Situs inversus totalis is the complete transpositioning of thoracoabdominal viscera into a mirror image of the normal configuration. Choledochal cyst is the congenital cystic dilation of the biliary tract. Both these conditions coexisting in a patient is extremely rare. We hereby present a case of type IC choledochal cyst in a patient with situs inversus totalis presenting with biliary sepsis secondary to choledocholithiasis. Also detailed are the management and operative strategies employed to deal with this rare entity.