Biliary atresia: All about the congenital liver disease of babies that could turn fatal

Being aware of congenital disease in your infant is extremely important for their survival and growth. Some of them could lead to disabilities of intelligence or development. One of such deadly congenital conditions is biliary atresia, a defect or blockage in the tubes or ducts that carry bile from the liver to the gallbladder. If left untreated, it could cause liver damage in babies and can turn deadly. (Also read: World Heart Day: All you want to know about congenital heart disease in children)

“Biliary atresia is developmental anomaly of biliary ductal system seen in perinatal period or in children. In this there is bile which is synthesised in liver cannot reach the gall bladder or the the intestine as the biliary system from the liver to the gall bladder and intestine is not developed,” says Dr. Dipankar Sankar Mitra Associate Director Minimal Access Gastrointestinal and Bariatric Surgery, Marengo QRG Hospital, Faridabad.

SYMPTOMS OF BILIARY ATRESIA

Dr Mitra says perinatal biliary atresia is the most common type in which the extra hepatic bile duct is not developed and bile can not drain into the intestine and gall bladder. Bile gets stagnant in the liver and causes hepatocytes (liver cell) injury. This will cause patient present with jaundice and pale colored stool. The other symptoms are growth retardation and failure to thrive.

DIAGNOSIS OF BILIARY ATRESIA

– The ultrasound of abdomen is the first investigation to see the part of biliary ductal system which is underdeveloped

– HIDA hepatobiliary scan will give us information about the full biliary ductal system from intrahepatic area to the common bile duct draining to the intestine.

TREATMENT FOR BILIARY ATRESIA

– Dr Mitra says Kasai procedure can help where we make an artificial way from base of liver to small intestine.

– Surgery: We make a small intestinal limb from 10cm distal to duodenojejunal flexure and bring that to the base of liver.

– We anastomose the small intestine opening to the hilar plate where the biliary ductal system opens into the extrahepatic bile duct system. By this we make artificial road to bile flow from liver to intestine.