Billary Atresia

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Overview

Biliary atresia is an obstructive cholangiopathy of unknown etiology involving both the intrahepatic and extrahepatic bile ducts. It presents in the neonatal period with persistent jaundice, clay-colored stools, and hepatomegaly. It is fatal if left untreated with a reported survival of less than 10% at 3 years of age.

The earliest reported reference of the condition was in 1817 by Dr. John Burns, who described it as an incurable state of the biliary apparatus. Later on, the first surgical success was achieved by Dr. William Ladds in 1920, but biliary atresia continued to have a dismal outcome until the 1950s when Dr. Morio Kasai first described the Kasai porto-enterostomy by dissecting the proximal obstructed biliary tract and creating a Roux-en-Y loop. It is now considered the standard procedure and is offered to all children undergoing surgical correction. With the progress and advancement of transplantation surgery, liver transplantation is an option available to children who have either failed to restore the bile flow in the initial Kasai surgery or have developed advanced liver cirrhosis.


Symptoms


Inflammation (swelling) and scarring caused by problems with the immune system

Infection by a virus

Exposure to harmful chemicals

Mutations (changes) in the genes. (Biliary atresia is not inherited from the baby’s parents.)

Light beige stools (Normal stool color for infants is yellow, green or brown.)

Dark brown urine.

A swollen belly (as the liver and spleen grow)

Difficulty gaining weight.

Ascites (fluid in the belly)

Liver failure after several months if the biliary atresia is not treated.

Causes

The causes of biliary atresia are not known, but may include:


Inflammation (swelling) and scarring caused by problems with the immune system

Infection by a virus

Exposure to harmful chemicals

Mutations (changes) in the genes. (Biliary atresia is not inherited from the baby’s parents.)

Risk factors

Biliary atresia is a rare disorder. It occurs in one in 15,000 babies. It Biliary atresia affects girls more than boys and doesn't usually happen in the same family.

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Complications

Complications of biliary atresia include failure to thrive link and malnutrition, cirrhosis and related complications, and liver failure. Without treatment, infants with biliary atresia would develop cirrhosis within 6 months and liver failure within 1 year.

Prevention

Prevention of biliary atresia is not possible because of the reason that either it occurs in the womb or within 2 to 4 weeks after birth. However, the risk of developing biliary atresia can be reduced.

Liver transplantation is the only cure for biliary atresia.