A Case of Congenital Hepatic Fibrosis with Variceal Bleeding.
- Author:
Dong Soo SHIN
1
;
Si Hong LIM
Author Information
1. Department of Pediatrics, Hallym University College of Medicine, Gangwon, Korea. intern44@hanmail.net
- Publication Type:Case Report
- Keywords:
Congenital hepatic fibrosis;
Esophageal varix
- MeSH:
Bile;
Biopsy;
Child;
Congenital, Hereditary, and Neonatal Diseases and Abnormalities;
Dizziness;
Esophageal and Gastric Varices*;
Fibrosis*;
Gastrointestinal Hemorrhage;
Hematemesis;
Humans;
Hypertension, Portal;
Ligation;
Liver;
Male;
Tomography, X-Ray Computed
- From:Korean Journal of Pediatric Gastroenterology and Nutrition
2004;7(1):98-101
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Congenital hepatic fibrosis is an inherited, congenital disorder of the liver characterized by portal hypertension and hepatic fibrosis. We experienced a case of congenital hepatic fibrosis with esophageal varix in a 9-year-old male. He complained hematemesis, hematochezia, dizziness. In laboratory examination, AST/ALT was slightly increased. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and hypoechoic lesion of periportal area were seen by abdominal CT scanning. Histologic finding of liver biopsy showed fibrous tracts containing dilated bile ductules connecting adjacent portal spaces that were widened by mature fibrosis. Endocopic sclerotherpy and ligation was done. We summarized a case with review of literatures.
