Internal carotid artery aneurysm: a vascular manifestation of type IV Ehlers-Danlos syndrome.

Aneurysms of the distal internal carotid artery (ICA) can be difficult to manage. When seen in a patient with Ehlers-Danlos Syndrome, type IV (EDS-Type IV), the complexity of the problem is greatly increased. Proximal ICA ligation in the presence of an intact circle of Willis and adequate collateral circulation is a reasonable and safe approach. We recommend the liberal use of duplex scanning and intravenous digital angiography in patients with EDS, and plan to use these techniques to follow the small, asymptomatic, right ICA aneurysm in our patient. If the aneurysm rapidly grows or becomes symptomatic, treatment may require proximal ligation with extracranial-intracranial bypass. Understanding of the basic defects associated with EDS-Type IV will make the management of patients with vascular complications more safe.