Multiple normal deliveries in a woman with severe portal hypertension due to congenital hepatic fibrosis: the importance of preserved hepatocellular function.

We report a 26-year-old woman with congenital hepatic fibrosis (CHFib), adult polycystic kidney disease, type II diabetes mellitus, and von Willebrand disease, who despite persistent advice to the contrary because of severe portal hypertension, had 5 pregnancies delivering 6 healthy infants over 8 yr. During this time, she experienced several episodes of esophageal variceal bleeding and severe ascites with persistently normal liver and renal function. CHFib is one of the prototypes of portal hypertension with preserved hepatocellular function. Although pregnancy is ill-advised in most women with portal hypertension and particularly with severe manifestations, this report illustrates the importance of preserved hepatocellular function for the outcome of pregnancy in these patients.