Pediatric biliary atresia

What is pediatric biliary atresia?

Missing or incomplete bile ducts is a serious condition known as biliary atresia. The bile is normally made by the liver to assist with the digestion of fatty foods. It drains through the small tube of the bile ducts, past the gallbladder and into the intestine.

Blockage of the common bile duct and resulting cholestasis in newborns is surgically managed, and surgery with the Kasai procedure (or Kasai portoenterostomy) can reestablish bile flow in most infants.

Risk factors

Biliary atresia is a rare condition, but the condition is one of the most frequent causes of liver transplantation.

What are the signs and symptoms of pediatric biliary atresia?

Some babies will start to gain weight, then lose weight and have other symptoms such as:

• Light-colored stools

• Dark-colored urine

• Irritability

• Yellow discoloration of skin can get worse

How is pediatric biliary atresia diagnosed?

• Ultrasound - Performing an abdominal ultrasound may help suggest biliary atresia, but a diagnosis is confirmed by an intraoperative cholangiogram to determine the structures of the bile duct and gallbladder, as well as microscopic evaluation of the liver.

• Blood work - Blood work look at the makeup of elements in the blood (bilirubin levels) and specialized scans (cholescintigraphy) further examine the bile ducts and gallbladder.

How is pediatric biliary atresia treated?

The Kasai procedure replaces the infant’s damaged atretic bile ducts with a section of the infant’s intestine. This restores bile flow from the liver to the intestine and prevents liver damage and failure. However, there are times when the scarring has become too advanced and the Kasai operation doesn’t work. For this reason, it’s important to make the diagnosis and perform a Kasai procedure early in the baby’s life.

After surgery, infants must be followed closely for several years for signs of inadequate biliary flow restoration or other complications. We work together with our patients’ families and primary care physicians to coordinate follow-up care.