Biliary Atresia in Children

Biliary atresia in children is a condition resulting from the partial or complete destruction of the bile ducts, which prevents the bile from reaching the small intestine.

  • Biliary atresia causes the accumulation of bile in the liver leading to permanent liver damage.
  • Jaundice, characterized by the yellowing of the skin, dark colored urine and pale stools are the usual symptom. The liver is enlarged.
  • Ultrasound scan and blood tests help in diagnosing the condition. The bile ducts as well as the liver can be surgically examined for accurate assessment.
  • Bile ducts are created surgically to facilitate the flow of bile into the intestine.

The functions of the bile prodiced by the liver include digestion of fats and the removal of the waste products formed by the chemical processes taking place in the liver. Bile is collected by the bile ducts in the liver and then carried to the small intestine. Biliary atresia causes the accumulation of the bile within the liver, and some of it gets into the blood, resulting in the characteristic yellowing of the skin. Eventually, the liver becomes permanently damaged, as a condition called biliary cirrhosis develops in the infant, usually within two months of birth.

When infants have biliary atresia, they pass dark colored urine and very pale stools. The discoloration of the skin also progresses. In about two weeks of birth, a physical examination of the infant may help the doctor feel the enlarged liver which feels firm to touch. Within two or three months, infants with this condition may develop severe growth retardation. Itching and irritability are usually present. Large, visible veins on the abdomen and enlargement of the spleen are also common symptoms.

If biliary atresia is diagnosed within the first two months of life, biliary cirrhosis can be prevented. Several blood tests and ultrasound scans may be required to make an accurate diagnosis. If biliary atresia cannot be confirmed by these tests, the bile ducts as well as the liver is surgically examined to reach a conclusive diagnosis. A biopsy of the liver may be done too.

A permanent drainage for the bile needs to be surgically created. In about 50% of cases, bile ducts connecting to the small intestine can be constructed surgically and these replacement bile ducts facilitate the normal functioning. Infants, in whom surgical construction of the replacement bile ducts could not be done at birth, often need liver transplantation in about two years.

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Yasser Elnahas

MD, PHD, Professor Of CardioVascular Surgery
Dr. Yasser Elnahas, Is an associate Professor of Cardiovascular Surgery. Dr. Elnahas was trained as a fellow At Texas Heart Institute And Mayo Clinic Foundation.Dr. Elnahas is dedicated to educating the general public about different disease conditions and simplifying the medical knowledge in an easy to understand terminology.

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