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Biliary Atresia - Causes, Symptoms, and Treatment

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Biliary Atresia - Causes, Symptoms, and Treatment

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Biliary atresia is a disease known for the absence of all or a portion of the bile duct. Its causes, symptoms, and management are explained in this article.

Medically reviewed by

Dr. Jagdish Singh

Published At July 26, 2022
Reviewed AtJanuary 5, 2024


The biliary system involves bile production, storage, and secretion in the liver and bile ducts. The biliary system usually helps with the digestive system. Biliary atresia is a rare gastrointestinal disease characterized by either the absence or destruction of a portion of the entire bile duct located outside the liver (extrahepatic). Due to this, there is an abnormal accumulation of bile in the liver, causing symptoms like jaundice in infants, fibrosis of the liver, heart defects, kidney malformations, and intestinal disorders. The cure for the disease is only surgery after a timely diagnosis. Special monitoring of nutritional needs and diets should be done for children suffering from biliary atresia. It is one of the common causes of end-stage liver disease and liver transplantation.

What Is a Bile Duct?

It is a tube-like structure that carries bile from the liver to the gallbladder, where it is stored. Further, this bile reaches the duodenum (the first part of the small intestine) for use in the digestion of food. The bile duct can be intrahepatic (inside the liver) or extrahepatic (outside).

How Common Is Biliary Atresia Condition?

Studies say this condition is seen in 1 in 12,000 babies in the United States. This condition is very common in regions of Asia. It occurs in 1 in 6,000 newborns in Taiwan.

This is a rare condition. Girl newborns are at greater risk compared to boy babies, and it usually does not occur within the same family.

What Are the Causes of Biliary Atresia?

The exact cause of the disease is not established, but several possibilities can lead to biliary atresia. Immunological, genetic, infectious, or toxic factors can contribute to the disorder's development and progression.

Some of the phenomena that can lead to disease are:

  • Several viruses are studied, which can be possible causative agents. These viruses include reovirus type 3, rotavirus, and cytomegalovirus.

  • In the majority of cases, bile ducts may be normal at the time of birth. Still, contributing factors such as genetic factors, immunological factors, or toxic factors can initiate epithelial degeneration, leading to rapid production of fibrous tissue, which causes sclerosis and, finally, obstruction of bile ducts.

  • A few cases can be seen due to developmental defects of the liver and biliary tree. These defects can occur during pregnancy.

What Signs and Symptoms Do Biliary Atresia Show?

Infants at two to six weeks of age develop symptoms such as


  • Yellowish discoloration of the mucous membrane, sclera of the eyes, and skin.

  • Swollen stomach or hepatomegaly (abnormal enlargement of the liver).

  • Dark urine and abnormally pale stool.

  • Poor weight gain.

Portal Hypertension:

  • Blood pressure increases in the veins that carry blood from the intestine to the liver.

  • Intrahepatic bile ducts (bile ducts inside the liver) are also involved.

  • If left untreated, it may result in permanent fibrosis of the liver (cirrhosis) and eventually hepatic failure.

Some children suffering from biliary atresia may develop congenital abnormalities like:

  • Situs Inversus: Rare condition with abnormal positioning of the heart.

  • Ventricular Septal Defects: Abnormal holes form in the ventricles of the heart.

  • Polysplenia: More than one spleen is present.

  • Asplenia: Absence of spleen from the body.

How Does Biliary Atresia Affect Diet?

Since children affected by biliary atresia have low bile flow, the following digestive effects take place:

  • Loss of appetite - The absence of a feeling of hunger.

  • A very fast metabolism leads to the need for more calories and energy.

  • Difficulty digesting fat-rich foods.

  • Fat-soluble vitamins are not absorbed properly.

  • Decreased concentration of protein, vitamins, and minerals in the body.

Usually, stool color depends on what the person eats and also depends on the amount of bile used for the digestion process. This amount of bile is seen in poop. When there is bile in poop, the stool color will be reflected as yellow. Another condition is alcoholic stools, which are caused by biliary atresia. In the case of alcoholic stool, the stool is pale or clay-colored.

Which Disorders Are Similar to Biliary Atresia?

  • Primary Sclerosing Cholangitis: A rare progressive disease in which inflammation, abnormal formation, and thickening of fibrous tissue within the bile duct and liver occur.

  • Neonatal Cholestasis: A group of disorders along with jaundice as one. There is a reduced flow of bile from the liver.

What Is Meant by Contracted Gallbladder?

Contracted gallbladder is a condition where the gallbladder is reduced in size and will not be visible in any imaging tests. A contracted gallbladder condition can impact the functioning of the gallbladder.

How to Diagnose Biliary Atresia?

Direct examination of bile ducts and tissue is necessary.

The procedures performed for this are:

  • Laparotomy: A small incision made in the abdomen to examine internal organs.

  • Intraoperative Cholangiogram: A special dye is injected into the gallbladder, and the filling of bile ducts is outlined using multiple X-rays. The movement or obstruction of dye through bile ducts and into the small intestine is traced.

  • Liver Biopsy: Tissue from the liver is excised surgically to investigate any malignancies.

  • Blood Tests: The presence of viral agents, the concentration of bilirubin, and elevated levels of liver enzymes can be monitored.

  • Ultrasound: It can help to check for the absence of organs like the gallbladder and spleen.

What Are Treatment Options for Biliary Atresia?

Surgical intervention is the only treatment option for biliary atresia. Additionally, attention to nutritional needs and diet is also essential for children.

  • Kasai Hepatoportoenterostomy: Surgical removal of obstruction from bile ducts to allow bile to flow to the intestine. In the procedure, the extrahepatic bile ducts are cut, removed, and replaced with a portion of the small intestine of the same infant. In most cases, bile drainage is established by this surgery. However, in some children, there can be liver dysfunction of a small degree still observed. The Kasai surgery can also be used as an early intermediate support procedure for a child’s growth. In some cases, despite the Kasai procedure, there are chances of liver transplantation. Liver transplantation is the removal of an infected or injured liver and replacement with a healthy liver from a different person called a donor.

How to Regulate Diet in Biliary Atresia?

Depending on the symptoms and treatment performed, a proper diet should be planned for the affected child.

  • Different food supplements like fat-soluble vitamins and medium-chain triglyceride (MCT) oil should be advised. MCT is easy to digest and adds calories to food.

  • If the child is not getting sufficient nutrition from the food, a nasogastric feeding tube is inserted to provide more caloric liquid directly into the stomach.

  • In some cases, total parenteral nutrition (intravenously) is also administered.

What Are the Complications Related to Biliary Atresia?

When this condition is not treated, it leads to many complications, such as

  • Cirrhosis, which leads to permanent scarring of the liver.

  • Portal hypertension is the vein that transports the blood from the intestine to the liver.

  • The buildup of fluid in the newborn’s baby is called ascites.

  • Hepatomegaly, which means liver enlargement.

  • The veins that line the esophagus get enlarged.

Overall, all these complications can result in liver failure.


Biliary disease, the absence or destruction of the bile duct, is a serious digestive issue in children. Proper diagnosis and cure of the symptoms and surgical treatment of the disease are very important. Maintenance of a diet and regulation of nutritional balance is important for the adequate growth and health of the affected children.

Frequently Asked Questions


How Long Can a Baby with Biliary Atresia Be Expected to Live?

Surgery for biliary atresia is successful when done at the earliest possible time. The success rate decreases as the child's age increases. Without surgery, a child might not be able to survive more than 2 years of age.


Is Biliary Atresia Treatable?

Biliary atresia is treatable with surgery. The Kasai procedure is one of the surgeries used to treat biliary atresia; ultimately, a liver transplant is usually required. Due to recent advancements in surgery, patients with biliary atresia manage to survive as adults.


What Is the Age at Which Biliary Atresia Is Diagnosed?

Biliary atresia is diagnosed in infancy. The first signs and symptoms usually appear when the infant is around two to six weeks of age. The initial symptoms include jaundice or yellowish discoloration of the eyes (white portion) and the skin, unusually pale stools, and dark urine.


Does Jaundice Cause Biliary Atresia?

Biliary atresia is an uncommon condition primarily affecting newborns; however, it is not genetic. It is not caused by jaundice but is one of the many symptoms of biliary atresia.


Is the Surgery for Biliary Atresia Successful?

Due to recent advancements, surgery for biliary atresia is becoming more successful. The damaged ducts present outside the liver are removed. Then, a little section of the intestine is removed and attached to the smaller ducts that still drain bile. As a result, the bitterness is then emptied directly into the intestine via the new, healthy ducts.


What Is the Level of Bilirubin in Biliary Atresia?

The directly bound (conjugated) fraction makes up 50–60% of the total serum bilirubin in infants with biliary atresia, which only exhibits a mild increase in total bilirubin, which is 6–12 mg/dL (milligram/ per deciliter).


What Is the Color of the Stool in Babies Suffering From Biliary Atresia?

Babies suffering from biliary atresia might have light yellow, grey, or white stools. Stools change color as they do not reach the intestine; they are excreted through the stool.


Which Test confirms Biliary Atresia?

A liver biopsy can confirm biliary atresia. A needle is used to take a microscopic sample of the liver. After that, a microscope is used to analyze the sample. Surgery is suggestive if biliary atresia is confirmed. It also aids in excluding or identifying additional liver issues. 


Who Is More Susceptible to Biliary Atresia?

Biliary atresia is a rare condition in which boys are less likely to develop biliary atresia than females, and it rarely occurs in the same family.


What Are the Causes of Biliary Atresia?

Though the exact cause is unknown, biliary atresia can be caused by exposure to certain toxic chemicals, a problem with the immune system, incomplete formation or malformation of bile ducts during pregnancy, or some sort of genetic mutation.


Do Infants Who Have Biliary Atresia Put On Weight?

Biliary atresia is a rare condition present since birth. The infants put on weight normally for the next few weeks after birth but show poor growth later due to malnutrition.


Is It Possible to Avoid Biliary Atresia?

Biliary atresia is not preventable as it is not genetically transferred or inherited, or transmissible. There is also no association between the mother's actions during pregnancy and biliary atresia, which is not transferred from the mother.


What Is the Standard Level of Bilirubin?

The normal bilirubin level is less than 1 mg/dL. Bilirubin levels in adults with jaundice are typically above 2.5 mg/dL.


Is Biliary Atresia a Serious Condition?

A baby's bile ducts, which are the tubes that transport bile from the liver, become blocked, causing biliary atresia, a serious liver disorder, to develop before or soon after birth. Bile is required by the body to support digestion and move liver waste outside the body.
Dr. Jagdish Singh
Dr. Jagdish Singh

Medical Gastroenterology


bile duct diseasebiliary atresia
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