Alagille syndrome

There is no cure for Alagille syndrome, but there are treatments to manage the disease. In rare cases, children may need a liver transplant. However, researchers at Children’s are exploring other surgical options that may help relieve symptoms.? ?In some cases, itching and xanthomas (cholesterol deposits) can be so severe that a child can’t maintain a normal quality of life. Surgeons at Children’s have performed a procedure called “ileal exclusion” or “internal biliary bypass” that may alleviate some of these symptoms. The word “ileal” refers to “ileum,” which is the last segment of the small intestine.

During this procedure:

• The end of the small intestine is bypassed, preventing the re-absorption of some bile acids from the intestine.

• Since bile acids are believed to be a primary contributor to itching, reduction in bile acid absorption can lead to improvements in symptoms.

• In addition, bile acids are the building blocks for cholesterol, so this procedure may also help reduce the severity of symptoms over time.

This procedure is reversible and additional procedures may still be necessary, including external biliary bypass or a liver transplant. Any child with Alagille syndrome-related itching is a candidate for ileal exclusion.