Biliary Atresia in children: Causes, symptoms, diagnosis and treatment

Biliary atresia is a rare genetic/congenital disease in which the bile ducts inside and outside the liver and gall bladder do not develop well from birth. Although several theories have been proposed, the cause of biliary atresia is not well known.

Causes:

In an interview with HT Lifestyle, Dr Ravi Mohanka, Director – Liver Transplant and HPB at HN Reliance Foundation Hospital, explained, “Normally the bile (a liquid containing digestive enzymes and some by-products of detoxification) produced by the liver is secreted into the intestine through the bile duct and temporarily stored in the gall bladder. A poorly formed bile duct prevents bile flow out of the liver and its accumulation in the liver. Toxic substances in the accumulated bile damage the liver and over a long time may progress to liver cirrhosis (scarring in the liver) causing liver failure.”

Symptoms:

Dr Ravi Mohanka revealed, “Children with biliary atresia may be normal at birth. They may develop jaundice after 2 – 3 weeks of age or their physiological/neonatal jaundice (harmless jaundice at birth that resolves in about 1 week) fails to resolve. Their family may notice yellowness of eyes or dark urine and pale or clay-colored stools. Such children should be seen by a liver specialist immediately.”

Diagnosis and treatment:

According to Dr Ravi Mohanka, the disease may be diagnosed by blood tests, sonography of the abdomen and a special scan (HIDA scan). He said, “In some cases, a liver biopsy or an abdominal operation to look for bile ducts may be required for diagnosis. If the diagnosis is confirmed at an early stage, an operation may be performed to connect a part of the intestine directly to the liver, bypassing the absent bile duct (Kasai portoenterostomy). This operation is more likely to be successful if done within 1 to 2 months of age, therefore it should not be delayed after the diagnosis.”

He added, “Children in whom the diagnosis is missed at an early stage or if the Kasai portoenterostomy is not successful develop progressive liver disease with worsening jaundice, excessive crying and irritability, growth failure, muscle loss, fat and vitamin malabsorption, water accumulation in feet and belly, an enlarged and stiff liver and/or spleen and other symptoms. Medicines are given to control these symptoms. A high calorie low-fat diet with MCT (a type of fat) and vitamins is used. Some children also have abnormalities of heart, intestines, blood vessels in the abdomen or develop secondary damage in the kidneys or lungs. Children developing liver cirrhosis, liver failure or growth failure have a high risk because of biliary atresia and can best be treated with a liver transplant. It is the most common reason for liver transplants in children.”