BACKGROUND: There are five types of choledochal cysts, which are anomalies that involve intrahepatic or extrahepatic bile ducts, or both. These lesions are found most frequently in patients who are Asian, female, infants but are recognized with increasing frequency in adults. METHODS: We have managed 16 patients with this anomaly. One patient was Asian, and 1 was a child. There were 3 males and 13 females. The mean age was 29 years. There were 9 type I, 1 type II, 1 type III, 4 type IV, and 1 type V cysts. Resection of cysts and hepatico Roux-en-Y jejunostomy were performed in 9 patients for type I cysts. Pancreaticoduodenectomy was performed for a type I and a type IV cyst. The extrahepatic portion of a type IV cyst along with a segment of liver was resected in 1 patient. Operation was terminated on 1 patient with a type IV cyst because of extensive involvement of the intrahepatic ducts. She will undergo liver transplantation. The type II cyst was resected. No surgery was performed on a type III and type V cyst. Four of these patients were previously treated unsuccessfully by internal drainage procedures. RESULTS: There was no mortality. Morbidity was limited to a patient who previously underwent incomplete resection of a cyst and a cyst Roux-Y jejununostomy. No cholangiocarcinoma has been encountered in our patients after a mean follow-up of 5.5 years from the time of initial discovery of the choledochal cyst. CONCLUSIONS: Management of choledochal cysts is successful after their complete removal. Partial cyst resection and internal drainage is less satisfactory because of occasional pancreatitis, cholangitis, and cholangiocarcinoma. Resection of the intrahepatic and intrapancreatic portions of the cysts reduces the risk of cancer even though this risk is low after incomplete cyst excision. Biliary continuity after cyst resection is best established by Roux-Y hepaticojejunostomy.
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