Choledochal cyst associated with portal hypertension.

Portal hypertension is a known complication of delayed presentation of choledochal cyst. In the literature, choledochal cyst presenting with portal hypertension has not been addressed adequately. The aetiology of portal hypertension in these cases has not been well studied, but it may be related to compression of the cyst over the portal vein, secondary biliary cirrhosis, or even extrahepatic portal venous thrombosis. We present our experience with four cases of choledochal cysts with associated portal hypertension over a 10-year period (1991-2001). Gastrointestinal bleeding, splenomegaly or incidental discovery of oesophageal varices led to the diagnosis of associated portal hypertension. Excision of choledochal cyst and bilioenteric bypass may not be feasible in those patients with massive collaterals in the region of porta with secondary portal hypertension. An initial approach of internal drainage (endoscopic/operative) of the cyst may allow regression of collaterals and subsequent safe excision of the cyst in this difficult group of patients. The liver function and histopathology dictated the final outcome.