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Pediatric Liver Center
Congenital Hepatic Fibrosis
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What is congenital hepatic fibrosis?
Congenital hepatic fibrosis is a rare condition that some children are born with. Congenital fibrosis can affect the liver and the kidneys, causing polycystic kidney disease and hepatic fibrosis (scarring on the liver).
Serious scarring can make the liver become enlarged and cause portal hypertension, which is increased pressure in the veins that carry blood through the liver and spleen.
What causes congenital hepatic fibrosis?
Congenital hepatic fibrosis is an inherited disorder that causes the bile ducts and blood vessels to form improperly. It is an autosomal recessive condition. Bile ducts excrete bile from the liver. Bile is a fluid that helps to digest fats. Bile ducts carry bile from the liver to the gallbladder and small intestine.
The hepatic portal system is a network of veins that carry blood to the liver to be cleaned. This inherited fibrocystic liver disease can also affect the formation of these blood vessels.
Who gets congenital hepatic fibrosis?
Children inherit congenital hepatic fibrosis from their parents, who must both carry a faulty gene to pass on this condition. The parents themselves may not have the disease.
What are the signs and symptoms of congenital hepatic fibrosis?
Some children with this condition may be completely asymptomatic, meaning they don’t show any signs or symptoms.
Other children develop an enlarged liver or spleen, which may cause their abdomen to swell. Some children may also develop bleeding in their gastrointestinal tract, which can cause them to vomit blood or have bloody stool.
Portal hypertension is another complication often linked to congenital hepatic fibrosis and can cause increased blood pressure. Children with portal hypertension may also have an enlarged spleen. They may also have swollen or dilated veins in their esophagus, which can lead to vomiting blood.
What tests are used to diagnose congenital hepatic fibrosis?
Your child’s doctor diagnoses congenital hepatic fibrosis based on several factors:
- A review of clinical history and physical exam allows doctors to rule out many common conditions
- Imaging tests, such as an ultrasound, CT scan or MRI, allow doctors to see if your child has an enlarged liver and spleen
- Blood tests help your child’s doctor tell if the liver is functioning properly
- A liver biopsy will help doctors see if the liver has scarring
What to expect from a liver biopsy
A board-certified pediatric hepatologist and pediatric anesthesiologist from our gastrointestinal procedure center perform the liver biopsy. Your child is under general anesthesia, so they don’t feel pain or discomfort. We remove a small piece of the liver, and a pathologist checks it under a microscope for signs of disease.
How do we diagnose congenital hepatic fibrosis?
Your child’s doctor makes the diagnosis based on medical history, clinical signs and symptoms, laboratory testing, radiology testing and a biopsy. We may also recommend genetic testing.
How is congenital hepatic fibrosis treated?
Treatment for congenital hepatic fibrosis is supportive. This means we work to prevent and treat complications of this disease, including infections, bleeding, poor growth and kidney problems.
We sometimes recommend endoscopy or surgery to treat complications, especially internal bleeding. While rare, children with this condition sometimes need a liver or kidney transplant.
Why choose us to treat your child’s congenital hepatic fibrosis?
Our Pediatric Liver Center is one of the largest in the country. We provide a multidisciplinary approach to diagnosing and caring for children with liver disease. Our liver team collaborates with kidney specialists, pathologists and radiologists to provide accurate diagnosis and comprehensive treatment.
Helpful resources
The National Organization of Rare Diseases (NORD) is a nonprofit patient advocacy organization. NORD is dedicated to people with rare diseases, such as congenital hepatic fibrosis, and the organizations that serve them.
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