Choledochal cysts: Management and long-term follow-up

Abstract

Background: Choledochal cysts are congenital anomalies that can occur at any level of the biliary tree. They carry long-term risk of biliary complications and cancer development. Complete excision of all involved bile ducts is recommended.

Methods: Patients treated between 1995 and 2019 were reviewed retrospectively.

Results: Sixty patients; 46 female and 14 male with a median age of 41 years (range 13-83) were included in the study. Mild abdominal pain was the most common presenting symptom (60%). Majority of the patients had Todani type I cysts (67%). Concomitant biliary malignancy was diagnosed in five patients (9%). Eight patients were followed-up conservatively (13%). Twenty-five patients were treated by excision of the extrahepatic bile ducts and Roux-en-Y hepaticojejunostomy, liver resection was added in seven, pancreatoduodenectomy was done in three and liver transplantation in one. There was no perioperative mortality. Postoperative complications developed in 17 patients (34%), two requiring surgical treatment. Four of the five patients with malignancies died at a median 42 months (range 6-95) following surgery. Median 62 months (range 8-280) follow-up was available in 45 surgically treated patients, 19 followed-up for more than 10 years. None of the patients developed malignancy during follow-up. Four patients (17%) were readmitted for anastomotic strictures requiring treatment.

Conclusion: The majority of choledochal cysts are Todani type-I and early cyst excision is the mainstay of management, which may decrease the risk of malignant transformation.

Keywords: Cholangiocarcinoma; Cholangitis; Hepatectomy; Liver resection.