A Complete Guide on Pediatric Biliary Atresia

The liver performs almost 500 functions to keep a person healthy. Still, researchers are discovering ways in which this hard-working organ benefits human health. The liver is one of the most essential and second-largest organs in the human body. Its main function is to process nutrients from the food a person eats and flush out toxins from it. However, that is not the only function it does. It builds proteins, makes bile, maintain blood sugar levels, and much more.

That is why a healthy liver is important for the overall health and wellbeing of a person. When one’s liver is overburdened or sluggish, his/her entire body will suffer. Some diseases such as biliary atresia, fatty liver, or hepatitis can severely affect the liver and ultimately lead to poor health. Sometimes parents have to opt for liver transplant in children to cure such diseases.

Before diving deep into its symptoms, causes, treatment, and diagnosis let us first understand what biliary atresia is.

What is biliary atresia?

Bile is a vital fluid produced by a kid’s liver, and it is stored in the gallbladder. It mainly helps in digesting food. When a child has biliary atresia, his/her common bile duct is blocked or damaged. Therefore, fluid cannot flow through it. It is tough for it just as it is hard for water to pass through a clogged pipe.

This biliary atresia is a rare liver disease of the bile ducts that affects only infants. It only occurs in young infants less than 3-4 months of age. The damage that is caused by biliary atresia can lead to scarring, loss of liver tissue and function, and cirrhosis.

Types of biliary atresia

There are mainly two types of biliary atresia.

• Perinatal biliary atresia

This is the most common type of biliary atresia. It appears after the birth of a baby, mostly when a baby is about 2 to 4 weeks old.

• Fetal biliary atresia

This type of biliary atresia is not so common. It appears while a baby is still developing in the mother’s womb.

Symptoms of biliary atresia

Generally, symptoms of biliary atresia usually begin to appear between 2 to 6 weeks after birth. These symptoms include:

• Jaundice that does not improve within 1 to 2 weeks
• Dark yellow or brown urine
• Poor weight gain
• Clay-colored, gray, or pale stools
• Enlarged liver
• Frequent nosebleeds or intense itching

Causes of biliary atresia

The roots of biliary atresia are still unknown. For some infants, it may occur because the bile ducts did not form appropriately during pregnancy. For others, it may be caused due to viral or bacterial infection, immune system problems, a genetic mutation, or exposure to toxins.

Risk Factors

Biliary atresia is a rare disorder. Its risk factors include:

• It seems to affects girls more than boys
• Within the same family, it is common for only one child in a pair of twins.
• Asians and Africans-American babies are at higher risk for the disorder.
• Premature infants are at higher risk for this disease than infants who are born at full-term.

Diagnosis

When it comes to diagnosis of biliary atresia, doctors will ask about the infant’s medical and family history, perform a physical examination, and order a series of tests. Several tests are needed to get the correct diagnosis of biliary atresia.

• Blood tests
• X-ray
• Abdominal Ultrasound
• Hepatobiliary Scan
• Liver Biopsy
• Diagnostic Surgery

Treatment

Usually, biliary atresia cannot be treated with medication. Doctors can operate this disease with a surgery called the Kasai procedure and eventually, in most cases, doctors operate this disease with liver transplant in children.

In the Kasai procedure, doctors removed the blocked bile ducts and then a segment of the small intestine is directly connected to the liver instead of the bile ducts. It is a kind of surgical method, in which re-establishing of the bile flow from the liver to the intestine is done by joining the two directly.

Usually, approximately 80 percent of infants who undergo a biliary atresia Kasai procedure survive to adulthood. The remaining 15-20% who are not helped by the Kasai procedure, they require liver transplantation. It will be the only cure for such infants suffering from biliary atresia.

Eating, Diet, and Nutrition

Kids who suffer from biliary atresia may have reduced bile flow to the small intestine. It may lead to malnutrition. To make sure infants and children with this disease get enough nutrients and calories, doctors may prescribe a particular eating plan and supplements.

Outlook

If a baby has a successful Kasai procedure, he/she may recover and have a full, active life. However, in most cases, he/she will need specialized medical care for the rest of his/her life. In the end, he/she may need a liver transplant.