Cerebrovascular Involvement in Ehlers-Danlos Syndrome.

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.