Biliary Tract Atresia

Biliary tract atresia is a rare disease (estimated incidence 1:5000 – 1:18000 born alive) of a still unknown etiology and with a newborn expression.  This disease causes the obstruction of the bile ducts.

It is a degenerative and progressive disease of unknown origin that leads to a progressive destruction of the biliary tract. The direct consequence is the accumulation of bile in the liver no longer discharged into the intestine, with a progressive toxic damage. Biliary atresia, however, is not a hereditary disease.

In the absence of treatment, severe cholestasis, which characterizes it, leads to biliary cirrhosis and end-stage hepatic failure within the first two years of life.

The diagnosis of biliary tract atresia is very complex and requires multidisciplinary clinical, radiological and histological skills. For this reason, it must be carried out in specialized centers identified on a regional basis

As a Regional reference center for the treatment of rare diseases of the liver and biliary tract of pediatric patients, ISMETT is the identified facility in Sicily also for the treatment of atresia.

In the case of atresia, a Kasai procedure (portoenteroanastomosis) named after the Japanese surgeon who first performed it, can be performed.  This surgical approach has radically changed the natural history of the disease, as it offers the possibility to restore, partially or completely, the biliary flow and slow down the liver damage. This way, the indication to a liver transplant can be postponed for many years. Biliary tract atresia is the most frequent indication for a pediatric transplant.

Timing is crucial with the Kasai procedure: the sooner it is performed, the higher its success rate. Sixty days of life is still the age limit before which it is acceptable to perform a Kasai procedure.