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Biliary atresia

Disease Information

Overview

If the diagnosis of biliary atresia is made within the first couple months of a child?s life, a portoenterostomy ("Kasai" procedure) is the treatment of choice. If done early enough and bile flow is reestablished, some children can do well for a long period of time.

Maureen Jonas, MD, Medical Director, Liver Transplant Program

Bile is an important fluid produced by your child’s liver and stored in her gallbladder. In a healthy system, it flows through a tube called the common bile duct to the small intestine, where it helps to digest food.

If your child has biliary atresia, her common bile duct is blocked or damaged, so that it’s impossible for bile to flow through it, just as it’s hard for water to pass through a clogged pipe. This is called “cholestasis,” or poor bile flow, and quickly leads to malnutrition and liver damage.

Biliary atresia is a chronic, progressive liver problem, and fatal if left untreated. This condition:

  • is the most common cause of chronic liver disease in newborns
  • occurs once in every 30,000 births
  • is the most common reason for  liver transplants in children
  • affects children of all races and ethnicities, but may be more common in Asians

The Children’s Hospital Boston approach

At Children’s, biliary atresia is treated by a small number of doctors with specific expertise within the large, multidisciplinary Divisions of  Gastroenterology and  Pediatric Surgery. We’re known for our individualized and science-driven approach. We’re home to the most extensive research enterprise located in a pediatric hospital in the world, and we partner with a number of top biotech and health care organizations—but our physicians never forget that your child is a child, and not just a patient.

Children’s is also home to New England’s largest pediatric Liver Transplant Program. If your child has biliary atresia, our team will work with you from the beginning to identify the best treatment options for each stage of her condition. Call 617-355-6058 for an appointment.

“Helping Your Child with Medical Experiences: A Practical Parent Guide”

Download a free booklet, “Helping Your Child with Medical Experiences: A Practical Parent Guide” (please note that Adobe Acrobat is required) and read about topics including:

  • talking to your child about his condition
  • preparing for surgery and hospitalization
  • supporting siblings
  • taking care of yourself during your child’s illness
  • adjusting to life after treatment

In-Depth  >>

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