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The presentation and management of 36 patients with cystic dilatation of the biliary tree seen over a 14-year period is reviewed. There were 28 females and 8 males, a ratio of 3.5:1. The classic triad of jaundice, pain and an abdominal mass was seen in only seven cases. Twelve patients suffered recurrent pancreatitis. There were three cases of variceal haemorrhage from portal hypertension secondary to biliary cirrhosis, and one case of biliary peritonitis from a ruptured cyst. Internal drainage (cystenterostomy) was performed in nine patients but five of these eventually needed cyst excision. Cysts have been excised primarily in the last 21 consecutive patients without mortality. Preliminary external drainage with T tubes was used before cyst resection in three patients who presented with complications of choledochal cysts. Cyst excision and hepaticojejunostomy is now the definitive treatment of choice.

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