Reviewed By: Pramod Kerkar, MD, FFARCSI

Biliary atresia is a rare disease gastrointestinal condition in which there is anomaly of the extrahepatic bile duct. Bile duct is responsible for carrying bile from liver to small intestine, which is responsible for fat metabolism and also helps in absorption of fat and fat-soluble vitamins. The absence or gradual destruction of bile duct can lead to abnormal accumulation of bile inside the liver leading to liver damage or cirrhosis. Biliary atresia is classified into congenital or fetal (embryonic) form and acquired or perinatal form. Biliary atresia is seen in every one out of 15000 live births. It is the most common cause of liver transplantation in US children. It is slightly more common in females than in males and also more common in Asian and African-American populations.

Symptoms of Biliary Atresia

Biliary atresia presents with symptoms of persistent jaundice, dark yellow urine, acholic stools (pale or white colored bowels), enlarged liver and enlarged spleen along with abdominal distension, poor weight gain along with irritability and portal hypertension. It may even result in liver cirrhosis and hepatic failure if it is not treated promptly.

Treatment of Biliary Atresia

Biliary atresia is fatal, if it is not treated promptly. The best surgical outcomes are expected when biliary atresia is treated before 3 months of age to correct bile flow obstruction. Kasai procedure is used to establish the flow of bile from liver to small intestine by removing the obliterated extrahepatic bile duct and attaching a segment of small intestine directly to the liver. This procedure helps regain bile flow in about 80% cases, but in about 20-25% cases the bile flow is not established. In cases of unsuccessful Kasai procedure, liver transplantation is opted.

What to Eat when you have Biliary Atresia?

A child’s diet and nutrition should be managed properly because even after treatment with Kasai procedure, the child still has reduced bile flow to the small intestine that might create problem in digesting and absorbing fat-soluble vitamins and there is loss of appetite. The child also has a faster metabolism with an increased need for calories and due to liver damage; there might be deficiency in proteins, vitamins and certain minerals. The child may become malnourished and show stunted growth.

The child may be referred to a pediatric nutritionist for formulating the child’s diet plan. The diet plan should include well balanced diet with supplementation of vitamins and fats that are not properly absorbed or digested by the child. The child may be deficient in fat-soluble vitamins such as vitamin A, D, E and K. They should be supplemented as 5-15000 IU of vitamin A per day, 50 ng/kg/day of vitamin D, 50-200 mg/day of vitamin E and 2.5-5 mg/day of vitamin K.

Hypermetabolism, in which the energy expense is 30% more in biliary atresia cases, should be compensated by giving high calorie, high protein feed to the child by providing 110-160% of the recommended daily requirement. Steatorrhea is managed by providing 40-60% of fat in the form of medium chain triglycerides. Medium chain triglycerides add calorie to food and are easily digested without bile and other fats. The recommended energy intake in biliary atresia patients should be around 15-17 % from proteins, 20-25% from fats and the remaining 60-60% from carbohydrates. Doctors recommend a special eating plan that includes special formulas and supplements added to breast milk, formula or food. If a child is not receiving enough nutrients from food and supplements by mouth, then the child might be recommended nasogastric feeding tube for providing high calorie liquid directly into the stomach. In some cases of biliary atresia, a child might require total parenteral nutrition (nutrition through intravenous line).

After a liver transplant, in general, a child can eat a healthy and balanced diet that is normal for their age. In both, surgery as well as transplanted cases, the child should follow up regularly with their healthcare provider as advised.

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Pramod Kerkar

Written, Edited or Reviewed By:

, MD,FFARCSI

Pain Assist Inc.

Last Modified On: July 13, 2018

This article does not provide medical advice. See disclaimer

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