Case Report
Year: 2023 | Month: September | Volume: 13 | Issue: 9 | Pages: 201-204
DOI: https://doi.org/10.52403/ijhsr.20230929
Congenital Hepatic Fibrosis - Case Report
Dr. Kumudavalli Muthukumarappan1, Dr. Suganthi K2
1Department of Pathology, 2Department of Pathology,
Apollo Hospitals, Chennai, Tamil Nadu, India
Corresponding Author: Dr. Kumudavalli Muthukumarappan
ABSTRACT
Congenital hepatic fibrosis is a heterogenous group of autosomal recessively inherited disorder due to defective remodelling of bile ductal plate. It is often seen in association with hepatorenal ciliopathies and syndromic fibrocystic abnormalities. Age of presentation and symptoms varies among the individuals which plays an important role in prognosis. Liver biopsy is the gold standard for diagnosis, characterized by abnormal excessive small bile duct proliferation with persistence of embryonic bile duct termed as Ductal plate malformation and periportal fibrosis of variable thickness and preserved hepatic lobules. We present a case of Autosomal recessive polycystic kidney disease and Congenital hepatic fibrosis in 11 years male child with growth retardation.
Key words: Congenital hepatic fibrosis, Ductal Plate Malformation.