“Biliary atresia is a condition in which there is a disruption in the flow of bile. As a result, bile cannot reach the intestines and accumulates in the liver, causing liver damage (cirrhosis). This disorder is a rare disease and is typical in newborns.”
Understanding Biliary Atresia
Biliary atresia is a rare disease of the bile ducts that only affects infants. The bile ducts are the pathways that carry a digestive fluid called bile from the liver to the small intestine. Once there, bile breaks down fats and absorbs vitamins, and then filters waste from the body.
As a result of biliary atresia, these ducts become swollen and blocked. Bile becomes trapped in the liver, where it begins to destroy cells. Over time, the liver can become scarred (cirrhosis). Once that happens, it can no longer filter toxins as it should.
Some babies develop biliary atresia in the womb. However, most often symptoms appear between 2 and 4 weeks after the baby is born. There are two forms of biliary atresia, perinatal and postnatal.
Symptoms of Biliary Atresia
In the perinatal form, symptoms usually appear before 2 weeks of age, while in the postnatal type, complaints usually only appear at 2–8 weeks of age. Early symptoms of biliary atresia include:
1. Jaundice
Yellowing of the skin and eyes due to very high levels of bilirubin (bile pigment) in the bloodstream. Jaundice caused by an immature liver is common in newborns. It usually resolves within the first week to 10 days of life. Babies with biliary atresia usually appear normal at birth but develop discoloration two to three weeks after birth.
2. Dark-colored urine
Caused by the accumulation of bilirubin (a breakdown product of hemoglobin) in the blood. Bilirubin is then filtered by the kidneys and excreted in the urine.
3. Acholic stool (white or clay-colored stool)
This is because there is no bile dye or bilirubin being emptied into the intestine. Bile gives the stool a green or brown color, and without it the stool is colorless.
4. Weight Loss
Babies experience weight loss and become very fussy as the level of jaundice increases.
Causes of Biliary Atresia
This disease is not hereditary or contagious. The exact cause of biliary atresia is still uncertain, but it is likely related to:
- Genetic changes.
- Immune system disorders.
- Disorders of liver and bile duct development in the womb.
- Exposure to toxic substances while in the womb.
- Viral or bacterial infection.
Biliary Atresia Risk Factors
Risk factors for biliary atresia are as follows:
- Newborn baby.
- The baby was born prematurely.
- Of Asian or African-American origin.
- Female gender.
Diagnosis of Biliary Atresia
To establish a diagnosis, the doctor will ask several questions related to the history of pregnancy and birth, and the mother’s illness or complaints during pregnancy and the child shortly after birth. Furthermore, a physical examination will be performed on the baby to confirm the signs and symptoms experienced. Supporting examinations may be recommended to confirm the diagnosis, including:
Blood tests, such as:
- Direct and indirect serum bilirubin.
- Alkaline Phosphatase, serum aminotransferases, serum bile acids, and GGTP ( 5 nucleotidase, gamma-glutamyl transpeptidase ).
- Serum Alpha-antitrypsin with Pi typing. Serum alpha-antitrypsin deficiency is one of the signs of liver disease in biliary obstruction in newborns.
- Sweat Chloride (CI). This examination is to check for cystic fibrosis which can also cause disorders in the bile duct.
Imaging tests, such as:
1. Ultrasound
This examination is used to provide an overview of the condition of the gallbladder and bile ducts.
2. Liver biopsy
The doctor will take a small amount of liver tissue (sample) to look at under a microscope. This can help distinguish biliary atresia from other liver conditions, such as hepatitis. A liver sample can be taken with a percutaneous liver biopsy or intraoperative cholangiography.
Biliary Atresia Treatment and Side Effects
Therapy for biliary atresia is with surgery. The Kasai procedure is a method used to treat conditions of bile flow disorders that occur outside the liver. This procedure is also called Kasai Portoenterostomy.
In this procedure, the surgeon will connect the bile duct to the intestine so that the bile fluid can flow back properly. After the procedure is done, antibiotics can be given to prevent infection in the duct and gallbladder.
In the case of obstruction of the flow in the liver, then drugs and vitamins A, D, and E can be given. Drugs that can be given such as ursodiol, which aims to help the flow of bile in the condition of the bile duct outside the liver in normal conditions. In addition, liver transplantation can also be considered for biliary atresia with severe liver damage.
To ensure that infants and children with biliary atresia get enough nutrition and calories, work with your doctor to create a personalized meal plan and provide supportive dietary supplements.
Complications Caused by Biliary Atresia
Common postoperative complications include cholangitis, anastomotic leak, bowel obstruction, portal hypertension, and hepatopulmonary syndrome. Biliary atresia can also cause a number of other problems in children, such as:
1. Growth Problems
Babies have difficulty digesting fats from breast milk or regular formula because bile (needed for fat digestion) does not enter the intestines. This causes poor growth in children. Babies may need special foods that contain different types of fats, which are better digested. Some babies need additional food or supplements to help with growth.
2. Vitamin Deficiency
Vitamins A, D, E, and K are fat-soluble and therefore cannot be properly absorbed into the body in biliary atresia. The baby will need additional medication to get these vitamins.
3. Itching
Liver disease can cause babies to become very itchy. This problem does not affect all babies with jaundice, but it can be very uncomfortable for them.
4. Other Issues
If surgery is not successful and the liver begins to fail, other problems can occur. A team of specialist doctors will talk to the baby’s parents about the risks to the baby and what to look out for.
Prevention of Biliary Atresia
In children with biliary atresia, bile flow to the small intestine is reduced and liver damage occurs, which can interfere with the digestive process and cause malnutrition. Therefore, it is necessary to ensure calorie and nutrient intake, both through food and supplements.
When to See a Doctor?
If your child or close relative experiences symptoms or complaints that indicate biliary atresia, immediately consult a doctor nearby.