Biliary Atresia

National Digestive Diseases Information Clearinghouse

What is biliary atresia? Ordinarily, bilirubin is taken up by the liver

and released into the bile. However, block­
Biliary atresia is a serious but rare disease age of the bile ducts causes bilirubin and
of the liver that affects newborn infants. It other elements of bile to build up in the
National occurs in about one in 10,000 children and blood.
Institute of
Diabetes and
is more common in girls than in boys and
Digestive in Asian and African-American newborns Jaundice may be difficult for parents and
and Kidney
Diseases
than in Caucasian newborns. The cause of even doctors to detect. Many healthy new­
biliary atresia is not known, and treatments borns have mild jaundice during the first 1
NATIONAL are only partially successful. Biliary atresia
INSTITUTES
to 2 weeks of life due to immaturity of the
OF HEALTH is the most common reason for liver trans­ liver. This normal type of jaundice disap­
plantation in children in the United States pears by the second or third week of life,
and most of the Western world. whereas the jaundice of biliary atresia
deepens. Newborns with jaundice after 2
The liver damage incurred from biliary weeks of life should be taken to the doctor
atresia is caused by injury and loss (atresia) to check for a possible liver problem.
of the bile ducts that are responsible for
draining bile from the liver. Bile is made by Other signs of jaundice are a darkening of
the liver and passes through the bile ducts the urine and a lightening in the color of
and into the intestines where it helps digest bowel movements. The urine darkens from
food, fats, and cholesterol. The loss of bile the high levels of bilirubin in the blood
ducts causes bile to remain in the liver. spilling over into the urine, while stool
When bile builds up it can damage the lightens from a lack of bilirubin reaching
liver, causing scarring and loss of liver the intestines. Pale, grey, or white bowel
tissue. Eventually the liver will not be able movements after 2 weeks of age are proba­
to work properly and cirrhosis will occur. bly the most reliable sign of a liver problem
Once the liver fails, a liver transplant and should prompt a visit to the doctor.
becomes necessary. Biliary atresia can lead
to liver failure and the need for liver trans­
plant within the first 1 to 2 years of life. What causes biliary atresia?
The cause of biliary atresia is not known.
The two types of biliary atresia appear to
What are the symptoms of be a “fetal” form, which arises during fetal
biliary atresia? life and is present at the time of birth, and a
The first sign of biliary atresia is jaundice, “perinatal” form, which is more typical and
which causes a yellow color to the skin does not become evident until the second
and to the whites of the eyes. Jaundice to fourth week of life. Some children, par­
is caused by the liver not removing biliru­ ticularly those with the fetal form of biliary
U.S. Department
of Health and bin, a yellow pigment from the blood. atresia, often have other birth defects in the
Human Services heart, spleen, or intestines.
An important fact is that biliary atresia is Initial tests. The doctor will press on the
not an inherited disease. Cases of biliary baby’s abdomen to check for an enlarged
atresia do not run in families; identical liver or spleen and order blood, urine, and
twins have been born with only one child stool tests to check for liver problems. The
having the disease. Biliary atresia is most level of bilirubin in the blood will be meas­
likely caused by an event occurring during ured and special tests for other causes of
fetal life or around the time of birth. Pos­ liver problems will be done.
sibilities for the “triggering” event may
include one or a combination of the Ultrasound of the abdomen and liver.
following factors: Ultrasound tests produce an image on a
computer screen using sound waves. Ultra­
• infection with a virus or bacterium sound tests can show whether the liver or
bile ducts are enlarged and whether tumors
• a problem with the immune system or cysts are blocking the flow of bile. An
• an abnormal bile component ultrasound cannot be used to make a diag­
nosis of biliary atresia, but it does help rule
• an error in development of the liver out other common causes of jaundice.
and bile ducts
Liver scans. Liver scans are special types
Research on the cause of biliary atresia is of x rays that use substances that can be
of great importance. Progress in the man­ detected by cameras to create an image of
agement and prevention of biliary atresia the liver and bile ducts. One such test is
can only come from a better understanding called hepatobiliary iminodiacetic acid
of its cause or causes. (HIDA) scanning. HIDA scans trace the
path of bile in the body and can show
whether bile flow is blocked.
How is it diagnosed?
Worsening jaundice during the first month Liver biopsy. If another medical problem
of life means a liver problem is present. is not found to be the cause of jaundice, a
The specific diagnosis of biliary atresia liver biopsy may be recommended. For a
requires blood and x-ray tests, and some­ liver biopsy, the infant is sedated and a
times a liver biopsy. If biliary atresia is needle is passed through the skin and then
suspected, the newborn is usually referred quickly in and out of the liver. A small
to a specialist such as piece of liver, about the size of a pencil
lead, is obtained for examination using a
• a pediatric gastroenterologist who microscope. A liver biopsy will usually
is an expert in digestive diseases of show whether biliary atresia is likely. A
children biopsy can also help rule out other liver
problems, such as hepatitis.
• 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

2 Biliary Atresia
How is it treated? The Kasai Procedure
Surgery. If biliary atresia appears to be the The Kasai procedure can restore bile flow
cause of the jaundice in the newborn, the and correct many of the problems of biliary
next step is surgery. At the time of surgery atresia. This operation is usually not a cure
the bile ducts can be examined and the for the condition, although it can have an
diagnosis confirmed. For this procedure, excellent outcome. Without this surgery, a
the infant is sedated. While the infant is child with biliary atresia is unlikely to live
asleep, the surgeon makes an incision in beyond the age of 2. The operation works
the abdomen to directly examine the liver best if done before the infant is 90 days old
and bile ducts. If the surgeon confirms and results are usually better in younger
that biliary atresia is the problem, a Kasai children.
procedure will usually be performed on
the spot. The improved results of the surgery make
the early diagnosis of biliary atresia very
Kasai procedure (hepato-porto­ important, preferably before the infant is
enterostomy). If biliary atresia is the several months old and has suffered per­
diagnosis, the surgeon generally goes manent liver damage. Some infants with
ahead and performs an operation called biliary atresia who undergo a successful
the “Kasai procedure,” named after the Kasai operation are restored to good health
Japanese surgeon who developed this and can lead a normal life without jaundice
operation. In the Kasai procedure, the or major liver problems.
bile ducts are removed and a loop of intes­
tine 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

3 Biliary Atresia
Unfortunately, the Kasai procedure is donor’s liver to use for transplantation.
not always successful. If bile flow is not Thus, parents or relatives of children with
restored, the child will likely develop biliary atresia can donate a part of their
worsening liver disease and cirrhosis and liver for transplantation. Because healthy
require liver transplantation within the first liver tissue grows quickly, if a child receives
1 to 2 years of life. In addition, the Kasai part of a liver from a living donor, both
operation, even when initially successful, the donor and the child can grow complete
may not totally restore normal liver devel­ livers over time.
opment and function. A child with biliary
atresia may slowly develop cirrhosis and Use of reduced size livers from deceased
related complications and require a liver donors and left lobe livers, which are the
transplant later in childhood. smaller part of the liver, from living donors
have greatly increased the availability of
While the Kasai procedure has been a transplantation for children with liver dis­
great advance in the management of biliary ease. At present, almost all children with
atresia, improvements in the operation biliary atresia requiring a liver transplant
and clinical management of children who will be able to receive “the gift of life,” in
undergo it are needed to improve the out­ the form of a liver from a deceased or
comes of children with this disease. living donor.

Liver Transplantation What happens after

Liver transplantation is a highly successful surgery?
treatment for biliary atresia and the Both before and after the Kasai procedure,
survival rate after surgery has increased infants will receive a specific diet with the
dramatically in recent years. Children right mix of nutrients and vitamins in a
with biliary atresia are now living into form that does not require bile to be
adulthood, some even having children of absorbed. Poor nutrition can lead to
their own. Because biliary atresia is not an problems with development, so doctors
inherited disease, the children of survivors will monitor an infant’s nutritional intake
of biliary atresia do not have an increased closely.
risk of having it themselves.
Some infants develop fluid in the abdomen
Improvements in transplant surgery have after the Kasai procedure, which makes
also led to a greater availability of livers the baby’s belly swell. This condition is
for transplantation in children with biliary called ascites and usually only lasts for a
atresia. In the past, only livers from small few weeks. If ascites lasts for more than
children could be used for a child with 6 weeks, cirrhosis is likely present and
biliary atresia because the size of the liver the infant will probably require a liver
had to match. Recently, advanced methods transplant.
have been developed to use part of an
adult’s liver, called “reduced size” or “split Also common after the Kasai procedure is
liver” transplants, for transplant in a child infection in the remaining bile ducts inside
with biliary atresia. the liver, called cholangitis. Doctors may
prescribe antibiotics to prevent cholangitis
In addition, surgery has been developed or prescribe them once the infection
that allows taking part of a living adult occurs.

4 Biliary Atresia
Children with biliary atresia may continue
to have liver problems after the Kasai pro­ Hope Through Research
cedure. Even with success of the operation
and return of bile flow, some children will Researchers are studying the possible
develop injury and loss of the small bile causes of biliary atresia and new ways
ducts inside the liver, which can cause to diagnose and treat it. One of the
scarring and cirrhosis. largest research initiatives is the Biliary
Atresia Research Consortium (BARC),
The liver affected by cirrhosis does not a network of centers funded by the
work well and is more rigid and stiff than National Institute of Diabetes and
a normal liver. As a result, the blood flow Digestive and Kidney Diseases.
through the liver is slowed and under
higher pressure. This condition is called The network comprises 10 liver disease
portal hypertension. Portal hypertension and transplant centers and one data-
can also cause flow of blood around, rather coordinating center. The centers work
than through, the liver. This complication together to coordinate research and
can cause intestinal bleeding that may share ideas and resources. The network
require surgery and may eventually lead to will enroll infants with biliary atresia in
a recommendation for liver transplantation. a large study to evaluate the best ways
of managing the disease and to carry
Cirrhosis of the liver can also lead to prob­ out clinical trials of new and promising
lems with nutrition, bruising and bleeding, treatments or approaches for diagnosis
and itching skin. Itching, called pruritus, is and monitoring the disease. Because
caused by the build up of bile in the blood biliary atresia is a rare disease, only a
and irritation of nerve endings in the skin. network of centers can identify enough
Doctors may prescribe medications for infants with this disease to carry out
itching including resins that bind bile in the studies of new therapies.
intestines or antihistamines that decrease
the skin’s sensation of itching. Centers will collect blood, tissue, and
other samples from infants with biliary
After liver transplantation, an important atresia so researchers can learn more
regimen of medicines is used to prevent about biliary atresia and find better
the immune system from rejecting the treatments. An important goal of
new liver. Doctors may also continue to BARC is to help find the causes of
prescribe special diets, vitamins, blood biliary atresia and recommend ways
pressure medications, and antibiotics. for its early detection and proper
management.

5 Biliary Atresia
For More Information National Digestive Diseases
The American Liver Foundation Information Clearinghouse
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Internet: www.liverfoundation.org Internet: www.digestive.niddk.nih.gov

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2235 Sheppard Avenue East, Suite 1500 Clearinghouse (NDDIC) is a service of the
Toronto, Ontario M2J 5B5 National Institute of Diabetes and Digestive and
Kidney Diseases (NIDDK). The NIDDK is
Canada
part of the National Institutes of Health under
Phone: 1–800–563–5483 the U.S. Department of Health and Human
Fax: 416–491–4952 Services. Established in 1980, the Clearinghouse
Email: [email protected] provides information about digestive diseases to
Internet: www.liver.ca people with digestive disorders and to their fami­
lies, health care professionals, and the public.
Children’s Liver Association for The NDDIC answers inquiries, develops and
Support Services distributes publications, and works closely with
25379 Wayne Mills Place, Suite 143 professional and patient organizations and
Valencia, CA 91355 Government agencies to coordinate resources
Phone: 1–877–679–8256 about digestive diseases.
Fax: 661–263–9099 Publications produced by the clearinghouse are
Email: [email protected] carefully reviewed by both NIDDK scientists and
Internet: www.classkids.org outside experts. This fact sheet was reviewed
by BARC investigators: Ronald Sokol, M.D.,
The Children’s Organ Transplant University of Colorado/The Children’s Hospital
Association of Denver; Jorge Bezerra, M.D., Cincinnati
2501 West COTA Drive Children’s Hospital and Medical Center; and
Bloomington, IN 47403 Benjamin Shneider, M.D., Mount Sinai Hospital
Phone: 1–800–366–2682 of New York. The original illustration of the
Fax: 812–336–8885 Kasai procedure was provided by Julie Porter.
Internet: www.cota.org

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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.

U.S. DEPARTMENT OF HEALTH

AND HUMAN SERVICES
National Institutes of Health

NIH Publication No. 06–5289

July 2006