CONGENITAL HEPATIC FIBROSIS Case report

Zorica D Raskovic

doi:10.5937/sjecr14-4339

Zorica D Raskovic Faculty of Medical Sciences

DOI: https://doi.org/10.5937/sjecr14-4339

Abstract

Congenital hepatic fibrosis (CHF) is a rare developmental disorder of the portobiliary system, most commonly associated with polycystic kidney disease. Pattern of inheritance of this disorder is autosomal recessive. Exact prevalence are not known (estimated from 1:10 000 to 1:20 000). Sequelae of CHF and portal hypertension were found to be developing in less than half of the all patients and were related with age.

We report the case of a boy suffering from CHF complicated with portal hypertension, splenomegaly and hypersplenism. The disease was recognized as cholestatic syndrome at neonatal age. Transcutaneous liver biopsy was performed and repeated at the age of 9 months. Diagnosis was made on the basis of histopathological examination. The ultrasound examination showed polycystic kidneys, but global renal function remain preserved. At the age of 8 years and 6 months portal hypertension was confirmed by Doppler ultrasonography and endoscopic examination revealed esophageal varices of second and third grade, also seen at the splenic portography. Trombocytopenia due to hypersplenism was identified with platelets count 75, 2 x10 ^3.In order to prevent variceal bleeding splenorenal shunt and a partial spleen resection were done. In the differential of the cholestatic syndrome in infants should have CHF. This kind of disease may suggests early developing complications such as portal hypertension and hyperspenism. Surgical treatment by performing portosystemic shunt is justified in these cases.

Author Biography

Zorica D Raskovic, Faculty of Medical Sciences

Pediatric clinic

Clincal Centre Kragujevac

Pediatrician

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