Biliary atresia in children: All parents must know about this medical condition

Your infant’s skin looks a bit yellowish. But your mom-in-law and mom say it’s normal. He probably just needs a bit of sunlight, they suggest. However, your mommy instinct kicks in and because you suspect something unusual, you book a doctor’s appointment.

And the results reveal Biliary Atresia – a condition you may not have even heard about.

Biliary atresia in children is a rare neonatal medical condition, which can be fatal if left undetected and untreated. This disease of the liver and bile ducts reportedly affects one in 10,000 infants worldwide. It is also more common in girls.

Infants only show its symptoms after two or four weeks post birth. But once it is diagnosed, biliary atresia in children needs immediate attention and medical care.

Biliary atresia in children: All you need to know about this condition

As you know, our liver cells produce bile liquid. This liquid digests fat and carries all the waste from the liver to the intestines. The body then excretes the waste.

This entire network of ducts forms the biliary system. When this system functions properly, it drains the bile from the liver to the intestines with ease.

However, in biliary atresia, bile gets trapped inside the liver. It is unable to flow down to the gallbladder and eventually to the intestines. This damages and scars the liver cells and might eventually lead to liver failure.

Causes and risks of biliary atresia in children

Although researchers are still trying to understand the exact cause of biliary atresia in children, a few reasons have been attributed to this rare condition. Some believe that auto-immune mechanisms are to blame, though it develops differently depending on the child.

In some infants, biliary atresia occurs because bile ducts did not develop during pregnancy. This is also called embryonic (fetal) biliary atresia. In other children, a viral infection post birth, can damage the bile ducts. This is called perinatal biliary atresia.

Interestingly, studies suggest that it can occur in 1 in 10,00 infants or 1 in 18,000 live births. It is also more common in Asian and African-Americans, as compared to Caucasian infants.

Typically, biliary atresia in children does not necessarily affect any other part of the body. But in rare cases, it can increase risks in the following:

• Abnormalities in the heart
• Blood vessels such as the inferior vena cava and preduodenal portal vein
• The spleen, which can rupture
• Intestinal malrotation, where the midgut rotates abnormally pushing the small bowel towards the right end

During birth, most infants with biliary atresia appear normal and healthy. But as mentioned before, they begin to show symptoms after two or three weeks. Let’s take a look at some of the common symptoms of this condition.

Symptoms of biliary atresia in children

You may notice the symptoms of this medical condition a few weeks post birth. Typically, there are four common symptoms of biliary atresia.

1. Jaundice

When the red blood cells in our body naturally destroy, they leave behind a by-product called bilirubin. If the body produces too much bilirubin, it can lead to yellow discolouration of the skin and a white area in the eye and mucous membranes. We call this condition jaundice.

Jaundice is the most common symptom of biliary atresia in children. If it goes away within a week or two of birth, your child isn’t suffering from biliary atresia. If however, jaundice appears after two or four weeks of birth, you will have to take your baby for an immediate checkup.

2. Dark yellow urine

When your infant’s body produces a large amount of bilirubin, it leads to dark coloured urine. It could be dark yellow bordering on orange, or it may even appear brown in some cases.

This is usually filtered by the kidneys and removed from the body as the baby passes urine. But if you notice dark coloured urine, it can be a symptom of biliary atresia.

3. Acholic stools

If your infant’s stool looks like clay and is soft but not watery, he could be suffering from this condition. Because there is additional buildup of bilirubin and bile is not thrown out, it can cause the stool to change its colour.

This can also sometimes lead to enlargement of your baby’s liver. His abdomen may also seem tough and hard.

4. Sudden weight loss

Because your baby is unable to clear his liver and there is waste still lying in his abdomen, he may not feel the urge to eat. This will eventually result in him losing a considerable amount of weight.

He might also become irritable and develop colic in the process. He might even continue to lose weight as jaundice progresses.

If you notice any of these symptoms, do not wait for them to pass because they will not. All of these symptoms need immediate attention by the doctor.

Only with a proper and thorough checkup, can they diagnose the level of biliary atresia and prescribe medications accordingly.

Diagnosis of biliary atresia in children

If your infant is suffering from this rare medical condition, the first thing he will be diagnosed for is jaundice. Now in order to properly diagnose jaundice, he will have to undergo a few tests, including blood screening.

Here are the tests you can expect if your baby has jaundice:

1. Blood test

A blood test can help identify any underlying issues with the liver. They can also uncover the causes of jaundice and any other organ problem that your child might be facing due to biliary atresia. Doctors will also check the complete blood count.

2. X-ray tests and ultrasound

An x-ray can show any enlargement of the liver or spleen. Therefore, most doctors will choose to perform this test on your infant to check their liver.

An ultrasound, on the other hand, will check the viability and functioning of the gallbladder.

3. Liver biopsy

In this test, a tiny piece of the liver is removed for testing. A liver biopsy is a reliable method to check biliary atresia in children. It can also help the doctor to decide whether or not to recommend surgery and prescribe further medication based on the condition of the liver.

4. Diagnostic surgery

An infant who is suffering from Biliary Atresia might also need diagnostic surgery. In this procedure, the doctors can inspect the injured parts of the bile duct and the liver, as well as the intestines.

Based on this analysis, they can begin the required treatment.

5. Operative cholangiogram

This is a another diagnostic surgery that can confirm biliary atresia in children. In these diagnostic surgeries, doctors inject a dye through the gallbladder. This dye travels to the bile ducts.

After the surgery, an x-ray is taken. If the dye flows normally, the infant is normal. But if the dye is unable to flow properly, it means the child is suffering from biliary atresia.

Once proper diagnosis is over, doctors may discuss a treatment plan with you. You should know that biliary atresia is not 100 percent curable. But with surgery, the child does have the chance to survive for a long period. But he will need extra care.

Treatment for biliary atresia in children

Unfortunately, biliary atresia is not treatable through medication. It requires surgery, namely the Kasai procedure or hepatoportoenterostomy.

This surgery is named after the Japanese surgeon Dr Morio Kasai, who first performed it.

What is the Kasai procedure?

In this procedure, surgeons try to re-establish a flow of bile between the ducts and the intestines. During the surgery, the doctors will identify and remove damaged bile ducts, including those outside the liver (extrahepatic ducts).

Simultaneously, they also zero in on smaller ducts that are functioning properly and are able to drain the bile into the intestines.

Once identified, they remove the damaged ducts.

Then a small part of the intestine is attached to that part of the liver (from where the damaged duct was removed). After this, bile flows directly from the liver to the intestines without obstruction.

Take a look at the video to understand how the Kasai procedure works.

After this procedure, the infant is kept under constant care for about 10 days and given antibiotics to keep infections at bay.

Ideally, the success of this procedure depends on the following:

• Age of the baby. The younger the infant, the more chances of the surgery’s success. If the child is four months or older, the chances of success decreases. By this time the liver should have developed quickly, but it is already half way destroyed.
• Liver damage. The extent of damage to the liver also determines the success of the surgery. If the liver is non-functional, it is tough to help the infant.
• The good ducts. The number of small ducts that can still pass bile into the intestine also determine the success. Fewer small ducts indicate a decreased rate of success.

Once the surgery is performed and it is a success, your child has a new lease on life. But it also means that you will have to give him extra care.

Care for infants suffering from biliary atresia

Usually, children who suffer from this rare medical condition have a fast metabolism. Even more so than healthy children. Therefore, they need more calories to burn.

A child with this condition and jaundice cannot digest fat. That’s because bile doesn’t reach the intestines. And because of damage to the liver, they also lose out on essential vitamins and proteins.

Nutrition for such kids will be special.

• You will have to give three square meals plus snacks throughout the day. Their food should also be low in fat and high in protein, minerals and fibre.
• You may have to give additional vitamin supplements, which the doctor will often recommend.
• In case of infants on baby formula, medium-chain triglyceride (MCT) oil is added. This adds extra calories into the formula. You may even have to supplement your breast milk with this oil, but only after the doctor’s recommendation.
• For babies younger than four months, feeds are given through the nasogastric tube. This is placed on the nose and brings the food to the stomach.

There is also a chance that this surgery won’t work. If it doesn’t, the infant can get an infection in his bile ducts. He may experience itching or a jaundice relapse. And in rare cases, bruising on the skin and even nosebleeds may occur. It can also lead to retention of body fluids.

Long-term effects of biliary atresia in children

It is crucial to mention here that the success of the surgery depends on the condition of the liver and development of high blood pressure.

Most kids who undergo the Kasai procedure may require a liver transplant, especially if they are under the age of five. Older kids may go through life without any liver or jaundice relapses.

Some kids might also develop hypertension, high blood pressure, gastrointestinal bleeding and even enlargement of spleen.

A few studies also suggest that 85 percent of children suffering from biliary atresia need a liver transplant before they turn 20. About 15 percent can go through their lives without a transplant.

A liver transplant will often be the final option. Your doctor will first try the Kasai procedure and then recommend a transplant. The important thing to note here that like with every illness, biliary atresia in children is also manageable.

It will, however, require you to be patient and to care for your child just as you would if he didn’t have this condition.

Sources: Cincinnati Children’s Hospital, Children’s Hospital of Pittsburgh, PAFMJ

Republished with permission from: theAsianParent Singapore