Abstracts
2002 Digestive Disease Week

# 102186 Abstract ID: 102186 Clinical Presentation and Surgical Management of Choledochal Cysts in Adults
Leyo Ruo, Ronald P Dematteo, Sam Obiekwe, William R Jarnagin, David Klimstra, Yuman Fong, Leslie H Blumgart, New York, NY

Purpose: Choledochal cyst is a congenital dilatation of the biliary tract usually diagnosed in childhood, and presentation as an adult is uncommon. Treatment by biliary bypass or drainage without complete cyst excision may be associated with biliary stasis, recurrent cholangitis, and malignant degeneration. This study reviews the clinical presentation, surgical management, and outcome of patients with choledochal cysts diagnosed in adulthood. Methods: From 1992-2000, 24 adult patients with choledochal cyst were evaluated at our institution. Data on presenting symptoms, preoperative imaging, pancreatobiliary anatomy, surgical treatment, pathology, and clinical outcome were obtained from a prospective database. Results: The median age was 34 (range 17-67) years and 19 of the 24 patients were female. Presenting symptoms included abdominal pain (N=22), mass (4), weight loss (11), pancreatitis (10), jaundice (8), and cholangitis (8). Initial diagnostic imaging was primarily with CT (23) or US (21). The biliary tract was studied preoperatively by ERCP (23), MRCP (13), and PTC (3). Abnormal pancreatobiliary anatomy was identified in 7 (29%) patients (pancreas divisum in 2, long common channel in 5), and evidence of biliary tract stones was found in 11 patients. Of the 22 patients treated with surgery, 19 had type I, 1 had type II, and 2 had type IV (Caroli's disease) choledochal cysts based on the Alonso-Lej-Todani classification. In addition to biliary resection with Roux en Y hepaticojejunostomy, 4 patients required hepatic resections (2 trisegmentectomies, 1 left hepatic lobectomy, 1 wedge resection) while 3 underwent pancreaticoduodenectomy (1 subsequent to hepatectomy) to achieve complete cyst excision. Benign histologic diagnoses included choledochal cyst (15), hyperplasia (2), and dysplasia (1). Each of the 4 patients with cancer had incomplete treatment (exploratory laparotomy in 1 and biliary bypass in 3) elsewhere 6-34 years earlier, and all died within 3 years of follow up. There have been no deaths in the other patients at a median follow up of 27 months. Conclusion: Complete cyst excision, including hepatic resection and pancreaticoduodenectomy if necessary, is recommended to avoid the development of cancer in a choledochal cyst, which carries a grim prognosis.