Congenital hepatic fibrosis

From Dog
Congenital hepatic fibrosis in a dog with characteristic tortuous bile ductules[1]

Congenital hepatic fibrosis is a rare genetic disease of young dogs characterized by juvenile liver disease. In humans it is an inherited condition, but the genetic cause in dogs is poorly understood.

Congenital hepatic fibrosis is a type of ductal plate malformation that affects the small interlobular bile ducts[2]. In this condition, the liver develops bridging bands of fibrous connective tissue that contain numerous small bile ducts, often characterized by irregular contours, contact of the ducts with hepatocytes, and ball-like protrusion of the duct wall into the lumen[3]. The fibrosis may be progressive. Typically, inflammation and cholestasis are absent.

This condition is quite rare in dogs and is associated with portal hypertension, acquired portosystemic shunts and ascites due to the effects of progressive fibrosis or to the typical insufficiency of portal veins; portal vein profiles are often hypoplastic with an abnormal branching pattern[4][5].

The liver is usually firm on clinical examination and there may be macroscopic or microscopic biliary cysts and/or acquired portosystemic shunts. Hepatic enzymes and function tests may be normal with no regenerative hyperplasia present, in contrast to end-stage liver or cirrhosis. However, cases of elevated liver enzymes, particularly ALT, ALP and GGT have been reported. Hepatic encephalopathy does not appear to be a feature of this disease.

Crystaluria, primarily urate uroliths are commonly observed.

Other unrelated disease may have similar gross lesions, such as congenital cystic liver and polycystic kidney disease in dogs in Cairn Terriers[6] and West Highland White Terriers[7], and Caroli disease[8], however none of these dogs were definitively diagnosed with congenital hepatic fibrosis, although all had macroscopically visible cystic lesions.

There is no reported treatment for this condition and affected dogs usually succumb to end-stage liver disease.


  1. Brown DL et al (2010) Congenital hepatic fibrosis in 5 dogs. Vet Pathol 47(1):102-107
  2. Giouleme O et al (2006) Ductal plate malformation and congenital hepatic fibrosis: clinical and histological findings in four patients. Hepatol Res 35:147–150
  3. Knisely AS (2003) Biliary tract malformations. Am J Med Genet 122A:343–350
  4. Desmet V (1998) Pathogenesis of ductal plate abnormalities. Mayo Clin Proc 73:80–87
  5. Desmet V (1998) What is congenital hepatic fibrosis? Histopathology 20:465–477
  6. McKenna SC & Carpenter JL (1980) Polycystic disease of the kidney and liver in the Cairn Terrier. Vet Pathol 17:436–442
  7. McAloose D et al (1998) Polycystic kidney and liver disease in two related West Highland White Terrier litters. Vet Pathol 35:77–81
  8. Gorlinger S et al (2003) Congenital dilatation of the bile ducts (Caroli’s disease) in young dogs. J Vet Intern Med 17:28–32