1. Use Exploratory Surgery to Definitely Diagnose
The usual first step in treating biliary atresia is definitively diagnosing the condition through exploratory surgery. During this surgery, physicians are able to observe the biliary system for damaged and or missing bile ducts. The procedure is best performed early in the infant's life to assure the best possible outcome. Exploration with surgery allows physicians to plan out the next step in treatment for biliary atresia--which is the Kasai procedure.
2. Treat With the Kasai Procedure
The Kasai procedure, also called a hepatoportoenterostomy, works to form a new duct from which bile can drain. This surgery consists of removing damaged outer ducts from the liver and then replacing them with a piece of the infant's intestine. This piece will serve as a conduit from which bile from the liver can pass to the intestine. Generally, a child receiving this procedure will be hospitalized for a week or 10 days after which she must be monitored closely. While this surgery is not a cure, it can often grant afflicted children moderately good health for several years. About half the children who undergo the Kasai procedure must have a liver transplant by the age of five.
3. Perform a Liver Transplant
A liver transplant is usually only performed after a Kasai procedure has either failed or years following a successful procedure when the child's condition worsens. The transplant may be of a whole or part of a liver when a family donates the organ of a child who has died. In this case, the child with biliary atresia needs to be placed on a waiting list for the first available matching organ. Another option is to transplant part of a liver donated by a live donor, generally a relative whose tissue types match that of the child. Following the operation, the child's health usually improves dramatically. It is necessary to guard against the body rejecting the new liver. Anti-rejection drugs are prescribed and must be administered according to the physician's directives. The child will need continued monitoring by his physicians and surgeons.
