Management of Choledochal Cysts: An Institutional Review

Abstract

Introduction: Choledochal cysts are congenital abnormalities characterized by cyst formation and dilation of the biliary tree. They are a rare condition more common in females with the highest incidence in the United States estimated at 1 in 13,500 live births (1). They can be clinically silent, cause biliary obstruction and/or transform to malignancy. Management is controversial because statistics regarding malignant transformation vary widely. Estimates from select studies vary from 10% (2) to 26% (3). This study was performed to review the natural history and management of choledochal cysts at our institution. Methods: Patient charts from 2009-2017 were obtained with the following ICD9 and ICD10 codes: Choledochal cyst (Q44.4); biliary cyst (K83.5); other specified disorders of the biliary tract (576.8); anomalies of gallbladder, bile ducts, and liver (751.69); biliary atresia (751.61); other congenital malformations of bile ducts (Q44.5); and intrahepatic bile duct carcinoma (155.1, C22.1). A total of 2,546 charts were identified. Charts were then reviewed for the presence of choledochal cysts. Results: Eleven patients with choledochal cysts were found. Ten out of the 11 were female. Five had Type I cysts (one of these patients also had a Type II cyst). Five had Type II cysts. There was only one patient with a Type III cyst and only one with a Type IV cyst. Five out of the 11 patients with choledochal cysts were managed with surgical removal with either a Roux-en-Y hepaticojejunostomy, Roux-en-Y choledochojejunostomy or Whipple procedure depending on other patient factors. The patient with the Type III cyst was lost to follow-up after diagnosis of the cyst. Conclusion: Management of choledochal cysts at our institution varied with about half opting for surgical removal and half opting for serial follow-up with imaging. Given the rarity of the disease and varied results regarding transformation to malignancy, an individualized patient approach should be taken. Future research aims could include creation of a database of patients with choledochal cysts, given that malignant transformation and management will likely vary by type of cyst, patient characteristics, and patient preference.