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Definition

Biliary atresia is a condition in which the normal extrahepaticbiliary system is disrupted. Progressive damage of extrahepatic and

intrahepatic bile ducts secondary to inflammation, leading to fibrosis,biliary cirrhosis, and eventual liver failure . 

IncidenceBiliary atresia affects approximately 1 in 10,000-15,000 births and

occurs in 2 distinct forms: fetal-embryonic and postnatal. 

Types

• The fetal-embryonic form appears in the first 2 weeks of life and10-20% of affected neonates have associated congenital defects.

• The postnatal form of biliary atresia is typically found in neonatesand infants aged 2-8 weeks. Progressive inflammation andobliteration of the extrahepatic bile ducts occur after birth. This formis not associated with congenital anomalies, and infants may have ashort jaundice-free interval.

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Classification:

Three main types of biliary atresia are surgicaly defined:

1 -In type I, the common bile duct is obliterated while the proximal bileducts are patent.

2 -In type II, atresia of the hepatic duct is seen. In type IIa, the cysticand common bile ducts are patent, whereas in type IIb, the cystic,common bile duct and hepatic ducts are all obliterated.

3-Type III atresia refers to discontinuity of both right and left hepaticducts to the level of the porta hepatis. Unfortunately, type III biliaryatresia is common, accounting for >90% of cases.

Pathophysiology• Infection with cytomegalovirus, group C rotavirus, and reovirus type

3 have been implicated in certain cases. Cholestasis almostcertainly contributes to ongoing hepatocellular and biliary damage.

• Histologic findings on liver biopsy typically include acute or chronicinflammatory change with obstruction, fibrosis, and the proliferationof ductal and glandular element. 

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What are the symptoms of biliary atresia?

Babies with biliary atresia usually appear healthy when they are

born. Symptoms of the disease typically appear within the first twoweeks to two months of life. Those symptoms include: 

-A baby with biliary atresia usually develops jaundice at two orthree weeks after birth.

-Dark urine - The bilirubin is filtered by the kidney and removedin the urine.

-Acholic stools (clay-colored stools) -- because no bile orbilirubin coloring is being emptied into the intestine.

-Also, the abdomen may become swollen from a firm, enlargedliver.

-Weight loss and irritability -- develop when the level of jaundice increases.

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How is biliary atresia diagnosed?

• Blood tests for liver function abnormalities.

• X-rays of the abdomen look for an enlarged liver and spleen.• An abdominal ultrasound can tell if there is a small gall bladder

or none at all.

• A nuclear test, called an HIDA scan, determines the flow of bile.

In this scan, a radioactive dye is injected into the infant's vein.The dye acts like bilirubin.

If the baby has biliary atresia, the liver will take up the dyebut it will not be able to flow through the damaged biliarysystem into the small intestine.

• A liver biopsy tells if an infant is likely to have biliary atresia.If the biopsy shows that the infant probably has biliary atresia,

further surgery will confirm the diagnosis.

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Investigations continue----Cholangiography •   Exploratory laparotomy with surgical cholangiography is recommended.

This is done by injecting contrast material through the gallbladder. To fined the

communication between the biliary tree and the gastrointestinal tract,

• Sonography-guided percutaneous cholecystocholangiography; radiographiccontrast material is injected into the gallbladder under sonographic guidanceand the extrahepatic biliary system is viewed by using fluoroscopy.

• Endoscopic retrograde cholangiopancreatography allows direct visualization of

the extrahepatic biliary tree with the injection of radiologic contrast agent intothe extrahepatic biliary system through the papilla of Vater.

Duodenal intubation To perform this study, a nasogastric tube is placed in the distal duodenum.

The absence of bilirubin in aspirated fluid suggests obstruction.

Liver biopsy •

Percutaneous liver biopsy is useful in evaluating neonatal cholestasis.•  Histologic findings, including bile-duct proliferation and obstruction, may not

be definitive in neonates younger than 2 weeks.

•  Results of repeat biopsy at 2-week intervals confirm the diagnosis in as manyas 95% of patients.

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Who is at risk for biliary atresia?• Biliary atresia is a rare disorder. About one in 15,000 to

20,000 babies do not have complete bile ducts.

•

Biliary atresia seems to affect girls more than boys.• There does not appear to be any link to medications

taken during pregnancy. 

Do children with biliary atresia have other

associated abnormalities?Ten to 15 percent of infants with biliary atresia may be born

with other problems: 

• Heart defects.

• Spleen (polysplenia).

• Blood vessels (inferior vena caval anomalies, pre-duodenal portal vein).

• Intestine (situs-inversus or malrotation).

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How is biliary atresia treated?

-The Kasai procedure is an op erat ion to create an o pen du ct sobi le can drain from the l iver. It is named after the surgeon who

develop ed it.-The su rgeon remo ves the damaged duc ts ou tside of the l iver

(extrahepatic duc ts) and replaces them w ith a piece of thebaby's own intest ine. This new du ct al lows bi le to pass fromthe liver into th e intes tine.

-The Kasai procedu re is no t a cu re for bi l iary atresia, bu t i t does

al low babies to grow and h ave fair ly go od h ealth for severalyears.

-When this procedure does not work, l iver transplantat ion cancorrect th is problem.

Success with th is proc edure is related to :  

• Age. Surgery is most successful in infants younger than two to

three months of age.• Extent of liver damage (cirrhosis) at the time of surgery.

• The number and size of microscopic ducts in the scarred tissuethat can drain bile.

• The experience of the surgical and medical team.

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Guidelines from your doctor for your child'snutrition may include: 

• Children with liver disease have a faster metabolism than healthy

children. This means that children with biliary atresia may requiremore calories.

•  A child with biliary atresia cannot properly digest fats. This is

because not enough bile gets to the intestine. Due to liver damage,

there may also be a loss of vitamins and protein. 

• A well-balanced diet, consisting of three meals a day plus smallsnacks in between meals.

• Vitamin supplements.

• Adding medium-chain triglyceride (MCT) oil to foods andliquids or infant formulas. MCT adds extra calories that willhelp your child grow.

• High-calorie liquid feedings may be recommended if your childis too ill to eat normally. Feedings are given through naso-gastric tube.

•  Although digestion may return to normal after surgery, extra vitaminsor MCT oil may be needed.

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What are the complications of biliary atresiaand what can be done for them?

• After the Kasai procedure, it is common to get an infection inthe bile ducts. This is usually treated using intravenousantibiotics.

• Jaundice or itching may occur. These can often be treatedsuccessfully with phenobarbital (for jaundice), cholestyramineand ursodeoxycholic acid (for itching).

• Many patients with cirrhosis have changes in blood flowthrough the liver and intestines. These changes may produceproblems such as easy bruising of the skin, nasal bleeding,retention of body fluid and varices in the stomach andesophagus.

• If retention of body fluid occurs, it can be treated with diureticsand potassium replacement.

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Liver Transplant

• If there is st i l l not enough bi le f low with the Kasaipro cedure, l iver transplantat ion is a final option . Al iver transp lant operat ion removes the damaged l iverand replaces i t wi th a new l iver from a donor.

• After transp lant su rgery, the chi ld 's health mayimp rove qu i te quick ly . However, the chi ld 's bodym igh t reject the new o rgan. To p revent reject ion, astr ic t schedule of ant i -reject ion medicat ions m ust betaken.

• After a transp lant, ongo ing l i felong care is required.Frequent contact wi th ph ysic ians and othermembers of the transp lant team  is also necessary.

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