What is biliary atresia?
Biliary
atresia is a rare disease of the biliary tract that only affects infants. The
bile ducts are pathways that carry a digestive fluid called bile from the liver
to the small intestine. Once there, it breaks down fats and absorbs vitamins.
It then filters waste from the body.
In
biliary atresia, these ducts swell and become blocked. Bile lingers in the
liver, where it begins to destroy cells. Over time, the liver can become
scarred. Once this happens, it can no longer filter toxins properly.
Some
babies become infected while still in the womb. But most often, symptoms appear
between 2 and 4 weeks after birth.
Causes
Doctors
believe that a number of factors can cause biliary atresia, including:
- Change in a gene
- Problem with the immune system
- Problem with the way the liver or bile ducts develop in the womb
- Toxic substances
- Viral or bacterial infection after birth
It is
not passed from one family member to another, and children cannot get it from
someone else.
Girls
who are born prematurely are most at risk. The same goes for Asian and African
American children.
Symptoms
If
your child has biliary atresia, the first thing you will notice is yellowing of
the skin and whites of the eyes. This is called jaundice. Jaundice is very
common in babies, especially those born before 38 weeks, but it usually
resolves within 2 to 3 weeks. Jaundice caused by biliary atresia lasts longer.
Their
belly may also swell, they will have gray or white stools, and their urine will
be dark. This is because their liver cannot process bilirubin. This is what
gives poop its brown color.
Some
children may also have frequent nosebleeds or severe itching.
Diagnostics
Many
liver diseases have the same symptoms as biliary atresia. To make sure he has
found the right cause, your child's doctor may test his blood for high levels
of bilirubin. They may also do some or all of the following:
- X-rays: A small amount of radiation
creates an image that is recorded on film or a computer. This checks for an
enlarged liver and spleen.
- Ultrasound: high frequency sound waves show detailed images of their organs.
- Liver scan: Special x-rays use chemicals to
create an image of the liver and bile ducts. This can show if and where bile
flow is blocked.
- Liver biopsy: The doctor will take a small
sample of tissue for examination under a microscope. This can show if they have
biliary atresia and help rule out other liver problems such as hepatitis.
- Diagnostic surgery: They will receive medication to
put them to sleep and their doctor will make a small incision in the stomach
area so they can examine the liver and bile ducts.
Treatment
The
most common treatment is the Kasai procedure. This is done if the blocked bile
ducts are outside the child's liver. During surgery, your child's surgeon will
replace the blocked bile ducts with part of their intestines. This allows bile
to flow from their liver through a new "duct" into the intestines.
If
surgery is done before your baby is 3 months old, the success rate of the
surgery is about 80%. If this fails, children usually need a liver transplant
within 1-2 years.
If the
blocked bile ducts are inside the liver, medications can help clear the bile,
and vitamin A, D, and E supplements may also be given. But a liver transplant
will likely be needed.
If the child successfully undergoes the Kasai procedure, he can recover and live a full and active life. But in most cases, they will need specialized medical care for the rest of their lives. Eventually, they may also need a liver transplant.