Alagille syndrome surgery

Overview
Although Alagille syndrome may be treated through pharmacological therapies, surgical therapy may also be necessary. Surgical procedures include angioplasty, liver transplants, and biliary diversion.

Surgery
Corrective surgery is sometimes needed to repair heart defects associated with Allagile Syndrome. Also, because the pulmonary arteries are often narrow in Alagille patients, a catheterization process similar to angioplasty may be used to widen the arteries to reduce pressure on the heart's pumping valves. In moderate to severe cases, stents may be placed in the arteries to increase their diameter. Transplantation of the liver has been a successful alternative to medication in severe cases. However, liver transplantation from donor parents should always be preceded by genetic testing because cases have been known where donor mother was also found to have alagille syndrome post transplant owing to the autosomal dominant inheritance pattern of the syndrome. Death from graft failure, neurological, and cardiac complications is significantly higher in patients with Alagille Syndrome than patients with Biliary Atresia.

Recently, a procedure called partial biliary diversion has been used to significantly reduce pruritus, jaundice, and xanthomas caused by poor bile flow. A portion of the bile produced by the liver is directed through a surgically created stoma into a plastic pouch on the patient's lower right abdomen. The pouch is periodically drained as it fills with bile.

The Kasai procedure, although appropriate for children with biliary atresia, does not benefit children with Alagille Syndrome and actually appears to worsen outcome.