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Biliary Atresia

By

Jaime Belkind-Gerson

, MD, MSc, University of Colorado

Reviewed/Revised Aug 2023
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Topic Resources
  • This defect causes bile to collect in the liver and can lead to irreversible liver damage.

  • Typical symptoms include a yellowish discoloration of the skin (jaundice), dark urine, pale stools, and an enlarged liver.

  • The diagnosis is based on blood tests, radionuclide scanning, and surgical examination of the liver and bile ducts.

  • Surgery is needed to create a path for bile to drain from the liver.

Bile, a digestive fluid secreted by the liver, carries away the liver's waste products and helps digest fats in the small intestine. Bile ducts Gallbladder and Biliary Tract The gallbladder is a small, pear-shaped, muscular storage sac that holds bile and is interconnected to the liver by ducts known as the biliary tract. (See also Overview of the Liver and Gallbladder... read more Gallbladder and Biliary Tract carry the bile from the liver to the intestine.

In biliary atresia, starting several weeks to months after birth, the bile ducts progressively narrow and become blocked. Thus, bile cannot reach the intestine. It eventually accumulates in the liver and then escapes into the blood, causing a yellowish discoloration of the skin (jaundice Jaundice in the Newborn Jaundice is a yellow color to the skin and/or eyes caused by an increase in bilirubin in the bloodstream. Bilirubin is a yellow substance formed when hemoglobin (the part of red blood cells... read more Jaundice in the Newborn ). Progressive, irreversible scarring of the liver, called cirrhosis Cirrhosis of the Liver Cirrhosis is the widespread distortion of the liver's internal structure that occurs when a large amount of normal liver tissue is permanently replaced with nonfunctioning scar tissue. The scar... read more Cirrhosis of the Liver , starts by the age of 2 months and progresses if the defect is not treated.

Overview of Biliary Atresia
VIDEO

Doctors do not know why biliary atresia develops, but some infection-causing organisms and gene defects may be involved. About 15 to 25% of infants with biliary atresia have other birth defects.

Symptoms of Biliary Atresia

In infants with biliary atresia, the urine becomes dark, the stools become pale, and the skin becomes increasingly yellowed (jaundiced). These symptoms and an enlarged, firm liver are usually first noticed about 2 weeks after birth.

By the time infants are 2 to 3 months old, they may have stunted growth, be itchy and irritable, and have large veins visible on their abdomen, as well as a large spleen.

Diagnosis of Biliary Atresia

  • Blood tests

  • Radionuclide scanning

  • Ultrasonography

  • Surgery

To diagnose biliary atresia, a doctor does a series of blood tests and an imaging test using a radioactive tracer. The tracer is injected into the infant's arm, and a special scanner tracks the flow of the tracer from the liver into the gallbladder and small intestine (called hepatobiliary scanning—a type of radionuclide scanning Radionuclide (Radioisotope) Scanning Imaging tests of the liver, gallbladder, and biliary tract include ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), endoscopic retrograde cholangiopancreatography... read more ). Ultrasonography Ultrasonography Ultrasonography is a type of medical imaging that uses high-frequency sound (ultrasound) waves to produce images of internal organs and other tissues. During an ultrasound, a device called a... read more Ultrasonography of the abdomen may be helpful.

Treatment of Biliary Atresia

  • Surgery

  • Frequently liver transplantation

Surgery is needed to create a path for bile to drain from the liver. The path is made by sewing a loop of intestine to the liver where the bile duct comes out. This operation should be done in the first month of life, before the liver has become scarred. If the operation is unsuccessful, infants need liver transplantation. Even when the operation is successful, about half of infants continue to have worsening liver disease and ultimately need liver transplantation.

After the operation, infants are often given antibiotics for a year to prevent inflammation of the bile ducts. They may also be given a medication called ursodiol. Ursodiol increases the flow of bile, which helps keep the bile drainage path open. Because good nutrition is important, infants are also given supplemental fat-soluble vitamins Fat-soluble vitamins Vitamins are a vital part of a healthy diet. The recommended dietary allowance (RDA)—the amount most healthy people need each day to remain healthy—has been determined for most vitamins. A safe... read more .

Prognosis for Biliary Atresia

Biliary atresia gets progressively worse. If untreated, it causes irreversible scarring of the liver (cirrhosis Cirrhosis of the Liver Cirrhosis is the widespread distortion of the liver's internal structure that occurs when a large amount of normal liver tissue is permanently replaced with nonfunctioning scar tissue. The scar... read more Cirrhosis of the Liver ) by the time the infant is several months of age, then liver failure, and death by 1 year of age. The long-term prognosis varies and depends on many factors.

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