Congenital hepatic fibrosis is part of a group of conditions called “fibrocystic conditions of the liver”. These all start well before you are born and are caused by a problem with the way your liver first starts to grow. Doctors sometimes call this a “ductal plate malformation”.
Because all these conditions start in the same way, people will often have more than one of them. This is particularly true of congenital hepatic fibrosis.
A lot of these other conditions involve cysts growing in your liver or bile ducts. Congenital hepatic fibrosis is a bit different. It causes scarring and toughening of your liver.
Severe scarring can cause a condition called cirrhosis. You can find out more about cirrhosis here.
Congenital hepatic fibrosis can also cause a condition called portal hypertension. Your portal vein carries blood to your liver. If your liver is badly damaged your blood cannot move through it properly. This causes pressure to build up in your portal vein. Which can lead to other serious problems such as bleeding veins (varices).
Congenital hepatic fibrosis can cause a swollen tummy. But most of its symptoms come from the complications that it can lead to. In particular, portal vein hypertension.
Congenital hepatic fibrosis is often linked to other fibrocystic liver diseases. You can find out more about the symptoms of these here.
Congenital hepatic fibrosis is usually found in children. It is very rare for it to be found for the first time in an adult. Because of this it can take some time to get the correct diagnosis.
There are lots of more common conditions that will have a similar effect on your liver. So you may have a lot of tests before it is clear that you have congenital hepatic fibrosis.
The most common tests that you will have will be ultrasound and MRI scans. You might also have a liver blood test. In rare cases you might need to have a liver biopsy.
You can find out more about these tests in the drop-down boxes below.
An ultrasound scan is quick and should not hurt. A wand will be put on your skin over your liver. The wand will use sound waves to get pictures of your liver and bile ducts. An ultrasound scan will help doctors to find out if there are cysts in your bile ducts. You can find out more about ultrasound scans here.
An MRI scan uses magnets and radio waves to build up a picture of the inside of your body. It does not use x-rays and is very good at looking at the soft parts of your body, such as your liver.
An MRI scan usually takes about half an hour. The scanner is a tube. You will lie on a bed that moves into the tube. The scanner can be very loud, so you will be asked to wear headphones.
If your diagnosis is still not clear after your scans, your doctor might suggest a biopsy. This is a procedure where a needle is put through your skin and into your liver. It will take a sample of your liver that can be looked at in a lab to see what is going on.
If your doctor suggests a biopsy, ask them about the benefits and risks of the procedure. You can also find out more here.
Treatment for congenital hepatic fibrosis is all about controlling your symptoms. And dealing with any complications your condition causes. So it will be different for everyone.
You can find more information about treatments for common complications using the links below:
Can I drink alcohol if I have congenital hepatic fibrosis?
You should not drink any alcohol if you have congenital hepatic fibrosis. Your condition has caused your liver to become scarred and damaged. So it will not be able to cope with even small amounts of alcohol. Drinking will add to the damage and increase the risk of complications.
If you usually drink alcohol and it is part of your social life, it can be very difficult to stop. We have some advice here
Can I take other medicines if I have congenital hepatic fibrosis?
You will not be able to take some medicines. This includes some common over the counter medicines like ibruprofen.
You will also need to be very careful about supplements and alternative medicines. These can sometimes cause liver damage. You are more at risk if you already have congenital hepatic fibrosis.
Always talk to your doctor before trying any new medicines or supplements. Even if they have been recommended by another practitioner.
Diet and congenital hepatic fibrosis
There is no particular food that is good or bad for congenital hepatic fibrosis. If you are currently well you should aim to look after your liver by eating a well-balanced diet.
If you have developed serious liver disease you might have trouble eating. If this happens you should ask your doctor to refer you to a registered dietician for help. You can also read out page on coping with eating difficulties.
Support for you
Finding out that you have any type of liver condition can be very difficult. It might help to connect with other people in a similar position. You can find information about our support groups and patient forum here.
We also have a helpline run by specialist liver nurses. You can talk to them by calling 0800 652 7330
Published on 10/08/2023
Review date 10/08/2026