Objectives: The presentation of choledochal cysts (CDCs) is altered by complications such as acute severe cholangitis, hepatolithiasis, spontaneous perforation, portal hypertension, pancreatitis, malignancy of the biliary tract and previous surgery in the form of internal drainage of the cyst. The management and the outcome of such complicated cysts differ from that of an uncomplicated CDC. This presentation is an analysis of our experience with complicated CDCs. Patients and Methods: One hundred and forty-four patients with CDCs were managed at a tertiary level referral hospital in North India, between January 1989 and June 2004. Thirty-three (23%) CDCs were associated with complicating factors: severe cholangitis requiring a biliary drainage procedure prior to definitive operative procedure (n = 11), spontaneous perforation (n = 3), hepatolithiasis (n = 6), acute cholecystitis (n = 1), recurrent acute pancreatitis (n = 1), chronic pancreatitis (n = 1), gastric outlet obstruction (n = 1), portal hypertension (n = 6), biliary tract malignancy (n = 4) and previous internal drainage of the cyst (n = 5). Five patients had more than one complication. The management outcome of complicated CDCs was compared with that of uncomplicated CDCs. Results: Complicated CDCs were significantly more common with type IV-A anatomy. Endoscopic, percutaneous or external surgical biliary drainage procedure was performed in 14 complicated CDCs prior to cyst excision. Desired definitive surgical procedure could be performed in 26/33 (79%) patients with complicated CDCs as compared to 107/111 (96%) patients with uncomplicated CDCs. Four (12%) patients with complicated CDCs had early postoperative complications as compared to 9/111 (8%) patients with uncomplicated CDCs. During a median follow-up of 13 months (2 months to 8 years), 2 patients with complicated CDCs died due to advanced secondary biliary cirrhosis and advanced gall bladder cancer, respectively. Six patients reported complications. Of the 6 patients, 3 required reoperation for a strictured hepaticojejunostomy (n = 2) and hepatolithiasis (n = 1). In contrast, there was no disease-related mortality and only 1 out of 111 patients with uncomplicated cysts had a complication during follow-up. Conclusion: Complicated CDCs merit a carefully planned management strategy including percutaneous, endoscopic and surgical procedures. The management may have to be staged. The outcome depends on the nature of complication and the management strategy adopted.

Keywords:
Choledochal cyst, Cholangitis, Hepatolithiasis, Pancreatitis, Biliary spontaneous perforation, Portal hypertension, Biliary tract malignancy
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2007
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