[Unusual presentation of a carotid-cavernous fistula in Ehlers-Danlos type IV]

Carotid-cavernous fistulae may present clinically in different ways, depending on the diameter and flow of the artery involved. In a direct fistula between the internal carotid artery and the cavernous sinus (high - flow fistula) the clinical presentation is usualLy dramatic and the signs and symptoms are severe. Indirect fistulae, in contrast, result from the connection of branches of the internal carotid, external carotid, or both to the cavernous sinus (low - flow fistula) and often present in a moderate and/or indolent manner. Patients with Ehlers-Danlos type IV suffer from vascular wall fragility resulting in a high frequency of aneurysms, dissections and fistulae. We describe a patient with Ehlers-Danlos vascular type IV who presented with mild signs and symptoms, suggesting the existence of a low-flow fistula. After two weeks, the patient's condition rapidly deteriorated. Endovascular catheterization demonstrated direct carotid cavernous fistula which was successfully treated. This case demonstrates the importance of early consultation with an endo-vascular surgeon in every patient with Ehlers-Danlos suspected of harboring a carotid cavernous fistula.