Choledochal cysts: a retrospective review of 28 patients and a review of the literature.

Retained choledochal cysts have been associated with recurrent cholangitis, portal hypertension and malignant lesions of the biliary tract. The authors reviewed the cases of 23 females and 5 males who had congenital cystic dilatation of the biliary tree; 26 were seen primarily and 2 were referred because of complications from previous surgery. Patient age at presentation ranged from 6 weeks to 46 years. The presenting complaints in 25 patients were pain or jaundice, or both; the classic triad of pain, jaundice and an abdominal mass was present in only 3 patients. Primary cyst excision was performed in 11 patients. Internal drainage procedures were performed in 12 patients, external drainage procedures in 3 patients and no direct operative procedure in 2 patients. Follow-up ranged from 1 to 20 years. Nine of 11 patients with primary excision were asymptomatic; 2 had recurrent cholangitis and required treatment for bile duct stricture. Five patients with retained cysts were asymptomatic. Recurrent cholangitis occurred in seven patients. Six patients required at least one reoperation, but only two had secondary cyst excision. The long-term complication rate for patients with retained cysts was 66.7% and for those with primary excision was 18%. The findings of this study support the current recommendation of primary excision of choledochal cysts. Symptomatic patients with retained cysts should have secondary cyst excision. Asymptomatic patients with retained cysts are at risk for malignancy and should undergo lifelong periodic radiologic examinations and liver-function testing.