Congenital Hepatic Fibrosis

SUMMARY A family with a high incidence of hepatomegaly, normal liver function and stillbirths is described. In one member biopsy material confirmed the diagnosis of congenital hepatic fibrosis. This patient's sister probably died of the same disorder. Unusual hepatic sinusoidal prominence was present in the patient described and in one of her daughters, from whom biopsy material is available. Regional adiposity also occurred in three generations. Congenital hepatic fibrosis should be suspected in patients with firm hepatomegaly and normal liver function. It is a familiar vascular anomaly of the liver. It may be symptomless, or it may cause death in childhood or adult life from portal hypertension or renal failure secondary to polycystic disease of the kidneys.