Abstract - Cerebrovascular Involvement in Ehlers-Danlos Syndrome.

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Wouter I. Schievink

Cerebrovascular Involvement in Ehlers-Danlos Syndrome.

Curr Treat Options Cardiovasc Med, June 1, 2004; 6(3): 231-236.

The Maxine Dunitz Neurosurgical Institute, Cedars-Sinai Medical Center, 8631

West Third Street, Suite 800E, Los Angeles, CA 90048, USA.

schievinkw@...

Ehlers-Danlos syndrome type IV is one of the most prominent heritable

disorders of connective tissues associated with neurovascular disease. It is

an uncommon disorder characterized by thin translucent skin, distinctive

facial features, excessive bruising, and rupture of blood vessels or

viscera. The typical neurovascular complications of this syndrome are

carotid cavernous fistulas, intracranial aneurysms, and cervical artery

dissections. Because of the inordinate fragility of the blood vessels in

patients with this syndrome, conservative treatment is always indicated.

However, in select cases in which the person or family history indicates a

more benign form of the disease, treatment that includes surgical or

endovascular treatment of asymptomatic lesions may be indicated.