Congenital hepatic fibrosis: CT findings in 18 adults

To evaluate the computed tomographic (CT) findings in adult patients with pathologically proved congenital hepatic fibrosis.

This was a retrospective review of congenital hepatic fibrosis cases identified at two institutions over the course of 8 years. Eight men and 10 women with an age range of 22-72 years (mean age, 39 years) were included. Contrast material-enhanced and unenhanced CT scans were obtained through the liver in all patients. Two radiologists evaluated size of and morphologic findings (atrophy or hypertrophy localized according to hepatic segments) in the liver; increased diameter or number of hepatic arteries at the hilum; presence of hepatic nodules, varices, spontaneous splenorenal shunts, and splenomegaly; and association with other hepatic ductal plate malformations and renal abnormalities.

Sixteen patients had morphologic abnormalities in the liver, 15 had splenomegaly (three underwent splenectomy for portal hypertension), and 14 had varices or spontaneous splenorenal shunts. An enlarged hepatic artery and a tangle of abnormally enlarged arterial vessels were identified in five and four patients, respectively, and four of these nine patients had large benign regenerative nodules. Ten patients had renal abnormalities and nine had an associated ductal plate malformation.

This retrospective study shows that certain findings (ie, liver morphologic and associated ductal plate abnormalities, varices, splenomegaly, and renal abnormalities) are frequently observed in combination in patients with congenital hepatic fibrosis.