Portal hypertension secondary to choledochal cyst

Two patients with portal hypertension secondary to choledochal cyst are reported and combined with four others previously reported in the literature. Choledochal cysts may, by extrensic compression, cause partial or complete portal vein obstruction, portal hypertension and esophageal varices. Needle biopsy of the liver is probably not a reliable means of differentiating choledochal cyst from other intrahepatic causes of portal hypertension. Internal drainage of the choledochal cyst has been performed in four patients and in each instance resulted in satisfactory portal decompression.