Management of choledochal cyst with portal hypertension.

Portal hypertension (PHT) is a rare complication associated with choledochal cysts. Management issues of PHT patients are inadequately addressed, as its incidence is low and underlying causes variable. We report three cases of choledochal cyst with PHT. All patients had type IVa choledochal cysts, and the causes of PHT were secondary biliary cirrhosis (SBC) (two cases) and alcoholic liver disease (one case). Clinical presentation included jaundice, gastrointestinal bleeding and ascites. One patient with SBC successfully underwent excision with Rouxen-Y hepaticojejunostomy, while the patient with cholangitis was managed with endoscopic retrograde cholangiopancreatography stenting. The last patient with alcoholic liver disease was managed conservatively for seven years and died of liver failure. Management of choledochal cysts depends on the severity of liver disease in cases of cirrhosis of unrelated cause, while those with SBC should be considered for surgical management. Endoscopic stenting may be considered as a temporary measure in high-risk cases.