Alagille Syndrome Clinical Care Program

Vascular Problems and Alagille Syndrome

Intracranial bleeding (bleeding within the skull) is the most dangerous central nervous system complication of Alagille syndrome and is a major cause of morbidity and mortality. It occurs in approximately 15 percent of people with AGS, and in 30 to 50 percent of these events the bleed is fatal. The majority of these events are spontaneous, although some have occurred after minor head trauma. It is not possible to predict who is at risk for developing a bleed. In addition to bleeding within the head, blood vessel problems such as aneurysms (dilatations) and stenoses (narrowings) can also be seen elsewhere in the body, such as kidney vessels and the aorta. At CHOP we recommend a baseline MRI of the brain in all young children with Alagille syndrome.

In the event of a head injury (for instance, a fall), a medical evaluation is recommended and imaging of the brain (by CT or MRI) may be warranted if there are any suspicious signs or symptoms.

Reviewed by: Binita M Kamath, MD
Date: January 2009

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