Background: Choledochal cysts are an uncommon entity. Surgical management is considered standard of care to prevent morbidity and to avoid the potential risks of malignant transformation. Our aim is to describe the clinical presentation, evaluation, management, and outcome of these patients.
Methods: A retrospective review of clinical, imaging, surgical, and outcome data of patients undergoing surgical management of choledochal cysts from 1982 to 2006 at a single institution.
Results: A total of 68 patients (57 female, 11 male) with a mean age of 30 years (range: 2mo-79y) were identified. Symptoms prompted evaluation in 96% of patients with abdominal pain (81%), jaundice (21%), and cholangitis (18%) being the most frequent. Cysts were classified as Type I (79%), II (6%), III (4%), or IV (12%). Operative management was based on cyst type and included complete excision (n=56, 82%), incomplete excision (n=9, 13%), or biliary diversion (n=3, 4%). Reconstruction after excision included Roux-en-Y hepaticojejunostomy (n=42, 62%), hepaticoduodenostomy (n=15, 22%). Decision to avoid complete cyst resection was based on cyst type, operative findings, and patient comorbidities. Median cyst size was 4.5 cm (range: 1-14). Neoplastic changes of cyst epithelium were identified in 4 (6%) patients, including cholangiocarcinoma in 2. Perioperative major morbidity or mortality occurred in 19% and 0% patients respectively. Follow-up was available in 78% of patients for a mean of 5 years (range: 1-242 months). Recurrent symptoms of cholangitis (n=3), pancreatitis (n=1), or chronic liver disease (n=1) were observed in 9% of patients having undergone complete cyst excision. Of two patients with cholangiocarcinoma at cyst excision, both underwent an R0 resection. An additional patient developed intrahepatic cholangiocarcinoma 7 years after complete cyst excision of a benign type I choledochal cyst. When comparing children (<18 years) (n=19) with adults (n=49), no major differences were notable in presentation, surgical management, or outcome, although associated neoplasia was not observed in children.
Conclusion: Choledochal cysts are an uncommon biliary tract abnormality which my cause significant morbidity and risk of malignancy. While an uncommon harbinger of malignancy, cholangiocarcinoma was identified in 4% of patients at initial cyst resection or on subsequent follow-up. Compared to earlier series, this incidence appears to be decreasing in an era where early complete cyst excision is more widely accepted. Thorough preoperative evaluation, surgical treatment, and follow-up are recommended in patients with choledochal cysts.