Reviewed By: Pramod Kerkar, MD, FFARCSI

Biliary atresia is a rare liver condition found in children that affects extrahepatic bile duct. It can be congenital (found at the time of birth) or acquired (found in a couple of weeks after birth). Bile duct is responsible for carrying bile from liver to small intestine, which helps in fat metabolism as well as absorption of fat-soluble vitamins (vitamin A, D, E and K).

Causes and Symptoms of Biliary Atresia

The etiology for biliary atresia still remains unclear; however, it has been postulated that it might develop due to malformations and disruptions in the formation of bile duct at the time of embryogenesis. It has also been linked with chromosomal abnormalities, but no specific genetic predisposition has been known. It has also been associated with imbalance in immune system and viral infections (cytomegalovirus, rotavirus and reovirus type 3, Epstein Barr virus, human papillomavirus) have also been associated with its etiology.

Jaundice is the most common symptom that is present in all cases of biliary atresia along with dark colored urine and acholic stools. The child also shows signs of weight loss and irritability with progression of jaundice. Along with the above symptoms, other symptoms may also be present including distension of stomach due to hepatomegaly or splenomegaly due to portal hypertension. If the disease process is not controlled or treated, then it may lead to liver fibrosis and scarring causing cirrhosis and ultimately liver failure, which leads to death of the infant within 2-3 years of the onset of disease.

Management of Biliary Atresia

The only effective management of biliary atresia is Kasai procedure that involves removal of the obliterated extrahepatic bile duct and re-establishing the connection between liver and small intestine by connecting a portion of small intestine directly with the liver. This procedure helps regain bile drainage in about 80% cases. There is normalization of bilirubin level within 3 months postoperatively, whereas the stool color also comes back to normal within first week postoperatively.

What Not to Eat when you have Biliary Atresias?

What foods to avoid when you have biliary atresia? Even with the management techniques such as Kasai procedure, the total amount of bile may not reach the small intestine and there may be health complications related to bile deficiency. This includes reduced metabolism of fat, reduced absorption of fat-soluble vitamins and hypermetabolism. The liver is also gradually damaged due to fibrosis, so liver function is also compromised leading to deficiency in proteins and coagulopathy.

The child becomes malnourished due to deficiency in nutrients and weight loss pursues. The patients of biliary atresia are usually given a well balanced diet with proper nutrients and supplements that combat protein deficiency, fat deficiency, and fat-soluble vitamins deficiency including vitamin A, D, E and K. Vitamin A deficiency leads to night blindness; vitamin D deficiency leads to calcium deficiency causing rickets and osteoporosis; vitamin E deficiency leads to decline in immunity, peripheral neuropathy and ataxia and vitamin K deficiency leads to hemorrhaging, particularly esophageal varices that is commonly seen in patients of biliary atresia.

There are certain foods that should be avoided when one has biliary atresia.

In biliary atresia cases, the child is usually deficient in fat due to incomplete metabolism of fat and hence, digestion leading to steatorrhea (excess fat excretion in feces). Since body does not digest and absorb most of the fats, medium chain triglycerides are given in place of other forms of fats. However, all fats should not be replaced by medium chain triglycerides in order to prevent essential fatty acid deficiencies. Therefore, neither medium chain triglycerides should be taken in excess nor should long chain triglycerides be completely eliminated from the diet. If there is consumption of excess fats then it will be excreted in feces.

Overfeeding should be avoided in patients of biliary atresia in order to decrease risk of obesity after liver transplantation. There have been more cases of obesity in biliary atresia children due to overfeeding in fear of malnutrition.

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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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