Biliary Atresia
 

National Digestive Diseases Information Clearinghouse 

National 

Institute of 

Diabetes and 

Digestive 

and Kidney 

Diseases 

NATIONAL 

INSTITUTES 

OF HEALTH 

U.S. Department 

of Health and 

Human Services 

What is biliary atresia? 

Biliary atresia is a serious but rare disease 

of the liver that affects newborn infants. It 

occurs in about one in 10,000 children and 

is more common in girls than in boys and 

in Asian and African-American newborns 

than in Caucasian newborns. The cause of 

biliary atresia is not known, and treatments 

are only partially successful. Biliary atresia 

is the most common reason for liver transplantation in children in the United States 

and most of the Western world. 

The liver damage incurred from biliary 

atresia is caused by injury and loss (atresia) 

of the bile ducts that are responsible for 

draining bile from the liver. Bile is made by 

the liver and passes through the bile ducts 

and into the intestines where it helps digest 

food, fats, and cholesterol. The loss of bile 

ducts causes bile to remain in the liver. 

When bile builds up it can damage the 

liver, causing scarring and loss of liver 

tissue. Eventually the liver will not be able 

to work properly and cirrhosis will occur. 

Once the liver fails, a liver transplant 

becomes necessary. Biliary atresia can lead 

to liver failure and the need for liver transplant within the first 1 to 2 years of life. 

What are the symptoms of 

biliary atresia? 

The first sign of biliary atresia is jaundice, 

which causes a yellow color to the skin 

and to the whites of the eyes. Jaundice 

is caused by the liver not removing bilirubin, a yellow pigment from the blood. 

Ordinarily, bilirubin is taken up by the liver 

and released into the bile. However, blockage of the bile ducts causes bilirubin and 

other elements of bile to build up in the 

blood. 

Jaundice may be difficult for parents and 

even doctors to detect. Many healthy newborns have mild jaundice during the first 1 

to 2 weeks of life due to immaturity of the 

liver. This normal type of jaundice disappears by the second or third week of life, 

whereas the jaundice of biliary atresia 

deepens. Newborns with jaundice after 2 

weeks of life should be taken to the doctor 

to check for a possible liver problem. 

Other signs of jaundice are a darkening of 

the urine and a lightening in the color of 

bowel movements. The urine darkens from 

the high levels of bilirubin in the blood 

spilling over into the urine, while stool 

lightens from a lack of bilirubin reaching 

the intestines. Pale, grey, or white bowel 

movements after 2 weeks of age are probably the most reliable sign of a liver problem 

and should prompt a visit to the doctor. 

What causes biliary atresia? 

The cause of biliary atresia is not known. 

The two types of biliary atresia appear to 

be a “fetal” form, which arises during fetal 

life and is present at the time of birth, and a 

“perinatal” form, which is more typical and 

does not become evident until the second 

to fourth week of life. Some children, particularly those with the fetal form of biliary 

atresia, often have other birth defects in the 

heart, spleen, or intestines. 
An important fact is that biliary atresia is 

not an inherited disease. Cases of biliary 

atresia do not run in families; identical 

twins have been born with only one child 

having the disease. Biliary atresia is most 

likely caused by an event occurring during 

fetal life or around the time of birth. Possibilities for the “triggering” event may 

include one or a combination of the 

following factors: 

•	 infection with a virus or bacterium 

•	 a problem with the immune system 

•	 an abnormal bile component 

•	 an error in development of the liver 

and bile ducts 

Research on the cause of biliary atresia is 

of great importance. Progress in the management and prevention of biliary atresia 

can only come from a better understanding 

of its cause or causes. 

How is it diagnosed? 

Worsening jaundice during the first month 

of life means a liver problem is present. 

The specific diagnosis of biliary atresia 

requires blood and x-ray tests, and sometimes a liver biopsy. If biliary atresia is 

suspected, the newborn is usually referred 

to a specialist such as 

•	 a pediatric gastroenterologist who 

is an expert in digestive diseases of 

children 

•	 a pediatric hepatologist who is an
 

expert in liver disease of children
 

•	 a pediatric surgeon who specializes in 

surgery of the liver and bile ducts 

Initial tests. The doctor will press on the 

baby’s abdomen to check for an enlarged 

liver or spleen and order blood, urine, and 

stool tests to check for liver problems. The 

level of bilirubin in the blood will be measured and special tests for other causes of 

liver problems will be done. 

Ultrasound of the abdomen and liver. 

Ultrasound tests produce an image on a 

computer screen using sound waves. Ultrasound tests can show whether the liver or 

bile ducts are enlarged and whether tumors 

or cysts are blocking the flow of bile. An 

ultrasound cannot be used to make a diagnosis of biliary atresia, but it does help rule 

out other common causes of jaundice. 

Liver scans. Liver scans are special types 

of x rays that use substances that can be 

detected by cameras to create an image of 

the liver and bile ducts. One such test is 

called hepatobiliary iminodiacetic acid 

(HIDA) scanning. HIDA scans trace the 

path of bile in the body and can show 

whether bile flow is blocked. 

Liver biopsy. If another medical problem 

is not found to be the cause of jaundice, a 

liver biopsy may be recommended. For a 

liver biopsy, the infant is sedated and a 

needle is passed through the skin and then 

quickly in and out of the liver. A small 

piece of liver, about the size of a pencil 

lead, is obtained for examination using a 

microscope. A liver biopsy will usually 

show whether biliary atresia is likely. A 

biopsy can also help rule out other liver 

problems, such as hepatitis. 

2	 Biliary Atresia 
How is it treated? 

Surgery. If biliary atresia appears to be the 

cause of the jaundice in the newborn, the 

next step is surgery. At the time of surgery 

the bile ducts can be examined and the 

diagnosis confirmed. For this procedure, 

the infant is sedated. While the infant is 

asleep, the surgeon makes an incision in 

the abdomen to directly examine the liver 

and bile ducts. If the surgeon confirms 

that biliary atresia is the problem, a Kasai 

procedure will usually be performed on 

the spot. 

Kasai procedure (hepato-portoenterostomy). If biliary atresia is the 

diagnosis, the surgeon generally goes 

ahead and performs an operation called 

the “Kasai procedure,” named after the 

Japanese surgeon who developed this 

operation. In the Kasai procedure, the 

bile ducts are removed and a loop of intestine is brought up to replace the bile ducts 

and drain the liver. As a result, bile flows 

from the small bile ducts straight into the 

intestine, bypassing the need for the larger 

bile ducts completely. (More about the 

Kasai procedure follows.) 

Liver transplant. If the Kasai procedure is 

not successful, the infant usually will need a 

liver transplant within the first 1 to 2 years 

of life. Children with the fetal form of 

biliary atresia are more likely to need liver 

transplants—and usually sooner—than 

infants with the typical perinatal form. The 

pattern of the bile ducts affected and the 

extent of damage can also influence how 

soon a child will need a liver transplant. 

(More about liver transplantation follows.) 

The Kasai Procedure 

The Kasai procedure can restore bile flow 

and correct many of the problems of biliary 

atresia. This operation is usually not a cure 

for the condition, although it can have an 

excellent outcome. Without this surgery, a 

child with biliary atresia is unlikely to live 

beyond the age of 2. The operation works 

best if done before the infant is 90 days old 

and results are usually better in younger 

children. 

The improved results of the surgery make 

the early diagnosis of biliary atresia very 

important, preferably before the infant is 

several months old and has suffered permanent liver damage. Some infants with 

biliary atresia who undergo a successful 

Kasai operation are restored to good health 

and can lead a normal life without jaundice 

or major liver problems. 

The Kasai Procedure 

3 Biliary Atresia 
Unfortunately, the Kasai procedure is 

not always successful. If bile flow is not 

restored, the child will likely develop 

worsening liver disease and cirrhosis and 

require liver transplantation within the first 

1 to 2 years of life. In addition, the Kasai 

operation, even when initially successful, 

may not totally restore normal liver development and function. A child with biliary 

atresia may slowly develop cirrhosis and 

related complications and require a liver 

transplant later in childhood. 

While the Kasai procedure has been a 

great advance in the management of biliary 

atresia, improvements in the operation 

and clinical management of children who 

undergo it are needed to improve the outcomes of children with this disease. 

Liver Transplantation 

Liver transplantation is a highly successful 

treatment for biliary atresia and the 

survival rate after surgery has increased 

dramatically in recent years. Children 

with biliary atresia are now living into 

adulthood, some even having children of 

their own. Because biliary atresia is not an 

inherited disease, the children of survivors 

of biliary atresia do not have an increased 

risk of having it themselves. 

Improvements in transplant surgery have 

also led to a greater availability of livers 

for transplantation in children with biliary 

atresia. In the past, only livers from small 

children could be used for a child with 

biliary atresia because the size of the liver 

had to match. Recently, advanced methods 

have been developed to use part of an 

adult’s liver, called “reduced size” or “split 

liver” transplants, for transplant in a child 

with biliary atresia. 

In addition, surgery has been developed 

that allows taking part of a living adult 

donor’s liver to use for transplantation. 

Thus, parents or relatives of children with 

biliary atresia can donate a part of their 

liver for transplantation. Because healthy 

liver tissue grows quickly, if a child receives 

part of a liver from a living donor, both 

the donor and the child can grow complete 

livers over time. 

Use of reduced size livers from deceased 

donors and left lobe livers, which are the 

smaller part of the liver, from living donors 

have greatly increased the availability of 

transplantation for children with liver disease. At present, almost all children with 

biliary atresia requiring a liver transplant 

will be able to receive “the gift of life,” in 

the form of a liver from a deceased or 

living donor. 

What happens after 

surgery? 

Both before and after the Kasai procedure, 

infants will receive a specific diet with the 

right mix of nutrients and vitamins in a 

form that does not require bile to be 

absorbed. Poor nutrition can lead to 

problems with development, so doctors 

will monitor an infant’s nutritional intake 

closely. 

Some infants develop fluid in the abdomen 

after the Kasai procedure, which makes 

the baby’s belly swell. This condition is 

called ascites and usually only lasts for a 

few weeks. If ascites lasts for more than 

6 weeks, cirrhosis is likely present and 

the infant will probably require a liver 

transplant. 

Also common after the Kasai procedure is 

infection in the remaining bile ducts inside 

the liver, called cholangitis. Doctors may 

prescribe antibiotics to prevent cholangitis 

or prescribe them once the infection 

occurs. 

4 Biliary Atresia 
Children with biliary atresia may continue 

to have liver problems after the Kasai procedure. Even with success of the operation 

and return of bile flow, some children will 

develop injury and loss of the small bile 

ducts inside the liver, which can cause 

scarring and cirrhosis. 

The liver affected by cirrhosis does not 

work well and is more rigid and stiff than 

a normal liver. As a result, the blood flow 

through the liver is slowed and under 

higher pressure. This condition is called 

portal hypertension. Portal hypertension 

can also cause flow of blood around, rather 

than through, the liver. This complication 

can cause intestinal bleeding that may 

require surgery and may eventually lead to 

a recommendation for liver transplantation. 

Cirrhosis of the liver can also lead to problems with nutrition, bruising and bleeding, 

and itching skin. Itching, called pruritus, is 

caused by the build up of bile in the blood 

and irritation of nerve endings in the skin. 

Doctors may prescribe medications for 

itching including resins that bind bile in the 

intestines or antihistamines that decrease 

the skin’s sensation of itching. 

After liver transplantation, an important 

regimen of medicines is used to prevent 

the immune system from rejecting the 

new liver. Doctors may also continue to 

prescribe special diets, vitamins, blood 

pressure medications, and antibiotics. 

Hope Through Research 

Researchers are studying the possible 

causes of biliary atresia and new ways 

to diagnose and treat it. One of the 

largest research initiatives is the Biliary 

Atresia Research Consortium (BARC), 

a network of centers funded by the 

National Institute of Diabetes and 

Digestive and Kidney Diseases. 

The network comprises 10 liver disease 

and transplant centers and one datacoordinating center. The centers work 

together to coordinate research and 

share ideas and resources. The network 

will enroll infants with biliary atresia in 

a large study to evaluate the best ways 

of managing the disease and to carry 

out clinical trials of new and promising 

treatments or approaches for diagnosis 

and monitoring the disease. Because 

biliary atresia is a rare disease, only a 

network of centers can identify enough 

infants with this disease to carry out 

studies of new therapies. 

Centers will collect blood, tissue, and 

other samples from infants with biliary 

atresia so researchers can learn more 

about biliary atresia and find better 

treatments. An important goal of 

BARC is to help find the causes of 

biliary atresia and recommend ways 

for its early detection and proper 

management. 

5 Biliary Atresia 
For More Information 

The American Liver Foundation 

75 Maiden Lane, Suite 603 

New York, NY 10038 

Phone: 1–800–GO–LIVER (465–4837) 

Fax: 212–483–8179 

Email: info@liverfoundation.org 

Internet: www.liverfoundation.org 

Canadian Liver Foundation 

2235 Sheppard Avenue East, Suite 1500 

Toronto, Ontario M2J 5B5 

Canada 

Phone: 1–800–563–5483 

Fax: 416–491–4952 

Email: clf@liver.ca 

Internet: www.liver.ca 

Children’s Liver Association for 

Support Services 

25379 Wayne Mills Place, Suite 143 

Valencia, CA 91355 

Phone: 1–877–679–8256 

Fax: 661–263–9099 

Email: info@classkids.org 

Internet: www.classkids.org 

The Children’s Organ Transplant 

Association 

2501 West COTA Drive 

Bloomington, IN 47403 

Phone: 1–800–366–2682 

Fax: 812–336–8885 

Internet: www.cota.org 

U.S. DEPARTMENT OF HEALTH 

AND HUMAN SERVICES 

National Institutes of Health 

NIH Publication No. 06–5289 

July 2006 

National Digestive Diseases 

Information Clearinghouse 

2 Information Way 

Bethesda, MD 20892–3570 

Phone: 1–800–891–5389 

Fax: 703–738–4929 

Email: nddic@info.niddk.nih.gov 

Internet: www.digestive.niddk.nih.gov 

The National Digestive Diseases Information 

Clearinghouse (NDDIC) is a service of the 

National Institute of Diabetes and Digestive and 

Kidney Diseases (NIDDK). The NIDDK is 

part of the National Institutes of Health under 

the U.S. Department of Health and Human 

Services. Established in 1980, the Clearinghouse 

provides information about digestive diseases to 

people with digestive disorders and to their families, health care professionals, and the public. 

The NDDIC answers inquiries, develops and 

distributes publications, and works closely with 

professional and patient organizations and 

Government agencies to coordinate resources 

about digestive diseases. 

Publications produced by the clearinghouse are 

carefully reviewed by both NIDDK scientists and 

outside experts. This fact sheet was reviewed 

by BARC investigators: Ronald Sokol, M.D., 

University of Colorado/The Children’s Hospital 

of Denver; Jorge Bezerra, M.D., Cincinnati 

Children’s Hospital and Medical Center; and 

Benjamin Shneider, M.D., Mount Sinai Hospital 

of New York. The original illustration of the 

Kasai procedure was provided by Julie Porter. 

This publication is not copyrighted. The 

Clearinghouse encourages users of this fact 

sheet to duplicate and distribute as many 

copies as desired. 

This fact sheet is also available at 

www.digestive.niddk.nih.gov.