Vascular Ehlers-Danlos Syndrome: Literature review and surgical management of intracranial vascular complications

Abstract

Vascular Ehlers-Danlos (vEDS) is a rare form of the Ehlers-Danlos Syndrome (EDS) where arterial fragility results from mutations in the gene that encodes type III collagen. The disease can lead to major neurological complications including carotico-cavernous fistulae (CCF), aneurysms of the Circle of Willis and endovascular procedures have an increased risk profile due to the delicate vasculature. Management of intracranial disease in vEDS requires an intricate understanding of the syndrome but is still associated with significant complications that lead to morbidity and mortality. As well as providing an approach to the management of neurovascular complications in vEDS, the relevant literature regarding nosology, aetiology and genetics of the condition is summarised here. Particular emphasis is placed on the two most common intracranial complications, namely carotico-cavernous fistulas and and cerebral aneurysms. Pros and cons of surgical and endovascular interventions are discussed and a technical discussion is concentrated on the surgical aspects of management.

Keywords: Carotico-cavernous fistula (CCF); Cerebral aneurysm; Vascular Ehlers-Danlos Syndrome (vEDS).