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.
Twenty bilio-enteric anastomoses were performed or managed from May 1990 to December 1992. Recurrent pyogenic cholangitis (RPC) and pancreatic cancer were the commonest conditions which required drainage procedures. Roux-en-Y hepatico-jejunostomy (RHJ) was performed in 9 patients, 4 for RPC, one for pancreatic cancer, another for a cholangiocarcinoma, 2 following excision of choledochal cyst and one hepatico-jejunostomy was part of a Whipple reconstruction. Roux-en-Y side to side choledocho-jejunostomy (CDJ) was performed in one patient. Choledocho-duodenostomy (CDD) was performed in 6, 4 for obstructive jaundice due to choledocholithiasis, one for RPC and one in a choledochal cyst. One patient operated elsewhere presented with complications after a CDD. Palliative cholecysto-jejunostomy (CYJ) was carried out in 4 patients with pancreatic malignancy. All benign conditions were treated by hepatico-jejunostomy and choledocho-duodenostomy, while three patients with malignant conditions were treated by hepatico-jejunostomy. Permanent subcutaneous access loops were provided when recurrent problems were anticipated, 4 in RPC and one after subtotal resection of a cholangiocarcinoma. Based on this study, we found Roux-en-Y hepatico-jejunostomy a versatile drainage procedure, which was useful in both benign and malignant diseases.