J Korean Pediatr Soc.
1981 Jun;24(6):577-583.
A Case of Congenital Hepatic Fibrosis
- Affiliations
-
- 1Department of Pediatrics, College of Medicine, Seoul National University, Korea.
- 2Department of Pathology, College of Medicine, Seoul National University, Korea.
Abstract
- Congenital hepatic fibrosis is a relatively rare liver disease in children and young adults,that is characterized by stony hard hepatomegaly and portal hypertension with relative preservation of liver function and underlying architecture. Since this Condition was first delineated by Kerr et al in 1961, approximately over 150 cases have been reported in the literature. However, congenital hepatic fibrosis was not described in our country, except for 4cases of portal fibrosis seen in adults. Recently we have experinced a case of congenital hepatic fibrosis in a 4-year-old girl. At the time of admission this patient showed growth retardation, accompanied by marked hepatosplenomegaly. The liver was stony hard and relatively smooth on palpation, however, the liver function test being with in normal limits. There was evidence of esophageal varices on esophagography. PathologicaIly liver wedge biopsy showed an extremely hard gray white liver tissue with a thickened capsule and even surface. Microscopically the liver was characterized by broad bands of mature fibrous tissue with relatively normal lobular architectures in between. These fibrous septa contained numerous well-formed bile ducts and were slightly :infiltrated with chronic inf1ammatory cells. Most bile ducts were small and were empty. Reduction in the number of portal branches or abnormal arterial branches were not seen. Liver needle biopsy done one year prior to the present wedge biopsy showed only moderate portal fibrosis and small round cell infiltration with minimal bile duct proliferation.
