Ten year experience with choledochal cysts: Lessons learned

Abstract

Presenter: Morgan Pfeiffer BS | Washington University, St. Louis Background: Choledochal cysts (CC) are rare congenital anomalies characterized by dilation of extra and/or intra-hepatic bile-ducts. Cyst excision with biliary reconstruction is the recommended treatment of choice because of risk of subsequent biliary malignancy which can persist even after resection. Limited data is available on natural history and long term outcomes in patients diagnosed and treated for CC. Methods: A single center retrospective review of patients diagnosed with CC between 2008 and 2018 was preformed. Patients were identified through institutional databases, and data pertaining to demographics, presenting symptoms, management, malignant transformation and long term outcomes was reviewed. Post-operative complications were classified using Clavien-Dindo scale. Results: Seventy-nine patients were diagnosed with CC during study period. 54 (68%) were type 1, 5 (6%) were type 2, 2 (2.5%) were type 3, 8 (10%) were type 4, 3 (4%) were type 5, and in 7 (8.5%) patients the cyst type was unknown. Sixty-one (77%) were female, and mean age at diagnosis was 27.2 years (SD= 24.21). The most common presenting symptoms were abdominal pain (29; 36.5%) and jaundice (10; 12.7%) Sixty-four (81%) patients underwent definitive surgical resection, 4 (6%) patients required endoscopic or percutaneous biliary decompression for symptoms, and 11 (14%) had no intervention and were followed with surveillance cross sectional imaging. In the surgery group, 59 (92%) had surgical resection of the extrahepatic bile duct with a cholecystectomy and Roux-En-Y hepaticojejunostomy. 4 (6.25%) patients had cholecystectomy for stone related symptoms. 1 (1.5%) had a Whipple. Thirty (48%) patients developed 49 postoperative complications. Twenty-six significant complications (Clavien-Dindo >3) were seen in 18 (28.5%) patients; 8 (26%) required PTC, 6 (16%) required ERCP, and 10 (30%) required an additional operation. There were no surgery related deaths. The average length of time with a PTC drain was 8.4 months (SD=10.2). The median length of follow up was 8.02 years (SD=3.47). Biliary malignancy was seen in 2 (2.5%) patients. One patient had an incidental diagnosis of T2 gallbladder cancer at age 55. One patient developed cholangiocarcinoma 12 years post-operatively at age 45. None of the resected patients had high grade dysplasia or invasive carcinoma present in excised cyst. Conclusion: The risk of developing cancer related to CC is low, especially in children and adolescents. Due to high surgery-associated morbidity and low cancer risk, it may be favorable to follow patients with imaging if they are asymptomatic.