Diagnosis of Congenital Hepatic Fibrosis in Adulthood

A Multidisciplinary Approach

Mohammed I. Alsomali, MD; Martha M. Yearsley, MD; Douglas M. Levin, MD; Wei Chen, MD, PhD


Am J Clin Pathol. 2019;153(1):119-125. 

In This Article


Our study highlights the importance of keeping CHF in the differential diagnosis of portal hypertension, especially in adult patients with chronic liver disease since childhood with accompanied chronic kidney disease, ciliopathies, or without obvious etiology. Although imaging studies lack specific hepatic findings for CHF, they are helpful to detect the concomitant fibropolycystic disease of other organs and to exclude common biliary diseases seen in the adult population. Liver biopsy remains a gold-standard diagnostic tool for CHF but requires interpretation in the setting of clinical context and radiologic features.