Congenital hepatic fibrosis

Below you will find more information about Congenital hepatic fibrosis from Medigest. If you believe that you are suffering from any of the symptoms of Congenital hepatic fibrosis it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition. There are medical conditions that carry similar symptoms associated with Congenital hepatic fibrosis and therefore the information provided by Medigest is offered as a guideline only and should never be used in preference to seeking professional medical advice. The information relating to Congenital hepatic fibrosis comes from a third party source and Medigest will not be held liable for any inaccuracies relating to the information shown.


Congenital hepatic fibrosis (CHF) is an extremely rare form of hereditary disorder that is characterized by the detection of a periportal fibrosis with some marked unevenly shaped producing bile ducts, as well as esophageal varices and intrahepatic portal hypertension. Congenital hepatic fibrosis is often associated with renal function disorder that is typically due to an autosomal recessive polycystic kidney disease.


Laboratory tests include examinations of the liver and the renal function. Ultrasonography, ultrasound examinations, CT scans, intravenous pyelography and splenoportography are among the possible imaging tools used to determine the extent of the disease. Liver biopsy and angiography may also be performed to derive a definitive diagnosis.


The medical therapy provided for congenital hepatic fibrosis patients is mostly during the detected presence of cholangitis. In cases of uncontrolled hemorrhage, surgical shunting or ban litigation may be performed. Surgical care also includes sclerotherapy and portosystemic shunt surgery.

Symptoms and Signs

Symptoms for congenital hepatic fibrosis hepatomegaly, evidence of some hypersplenism, experience of abdominal pain particularly on the upper right quadrant of the body. Abnormalities are usually diagnosed after the infant's birth.


Congenital hepatic fibrosis is categorized as an autosomal recessive disorder with no known causative agent or definite cause identified.

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