Management of adult choledochal cyst

Abstract

Introduction: Choledochal cyst is a rare benign biliary disease mostly presenting during childhood. Adult presentation is rare and associated diseases and complications are common. This paper aims to review the management of adult patients who presented to our institution with choledochal cyst, focusing on their presentation, preoperative investigations, surgical treatment given and postoperative course.

Methods: A retrospective review of all our choledochal cyst patients from January 2000 to August 2004 was performed. Data collected included demographics and clinical information.

Results: There were ten patients, eight female (80 percent) and two male (20 percent). The average age at presentation was 38.6 (range 16-81) years. The commonest presenting complaints were obstructive biliary disease (nine out of ten, 90 percent). There were seven Type I (70 percent), one type IVA (10 percent), one type IVB (10 percent) and one (10 percent) with Caroli's disease. Two patients had concomitant cholangiocarcinoma (20 percent). Three patients had associated cystolithiasis and one patient had pancreatitis. One patient had early cirrhosis due to her disease. Six patients underwent total cyst excision with a Roux-en-Y hepaticojejunostomy. One patient who previously had a biliary bypass underwent further resection of her cyst and Whipple's operation because of development of cholangiocarcinoma in the distal remnant cyst. They are currently well with no surgical complications. The average length of follow-up was 16 months (range six months to three years).

Conclusion: Adult patients with choledochal cyst have associated biliary problems such as the presence of cholangiocarcinoma, cystolithiasis, cholecystitis and liver cirrhosis with portal hypertension. They tend to present similar to obstructive biliary disease. The best surgical option for these patients is a total cyst excision together with a Roux-en-Y hepaticojejunostomy.