Congenital Hepatic Fibrosis

The fibrosis would affect resistance in portal veins leading to portal hypertension.^{[citation needed]}

Congenital hepatic fibrosis
Congenital hepatic fibrosis has an autosomal recessive pattern of inheritance.
Specialty	Gasteroenterology

The condition is usually congenital, but sporadic cases have also been reported. It may be associated with other congenital defects, commonly with autosomal recessive polycystic kidney disease, the most severe form of PKD. Some suggest that these two conditions are one disorder with different presentation.

Embryogenically, congenital hepatic fibrosis is due to malformation of the duct plate, a round structure appearing in the eighth week of gestation that is formed by primitive hepatocytes, which differentiate into cholangiocytes. Congenital hepatic fibrosis usually presents in adolescent or young adulthood, but onset of signs and symptoms can range from early childhood through mid-life. Clinical features may vary but commonly include cholangitis, hepatomegaly and signs of portal hypertension.^{[citation needed]}

Liver biopsy is diagnostic. In biopsy there is diffuse periportal and perilobular fibrosis in broad bands, containing distorted duct like structure or microcyst formation.^{[citation needed]}

Management includes control of esophageal bleeding/varices and treatment of associated renal disease if present.^{[citation needed]}

• Caroli disease
• Polycystic kidney disease
• Von Meyenburg complex
• Biliary hamartomas