Endovascular repair of direct carotid–cavernous fistula in Ehlers–Danlos type IV

Abstract

Ehlers-Danlos syndrome (EDS) type IV is a collagen vascular disease with an autosomal dominant inheritance caused by COL3A1 mutation. Patients with EDS type IV can present with organ rupture, spontaneous arterial dissections and ruptured aneurysms. Because of their propensity to form arterial dissections, aneurysms and rupture, they can develop carotid-cavernous fistula (CCF) after minor trauma or spontaneously. In EDS, it has been reported that even conventional catheter diagnostic angiography may result in large artery dissections and vessel rupture. In addition, the treatment of CCF in EDS type IV can result in up to 59% mortality after initial treatment, of which 23% is attributed to direct complications of treatment. We present the case of a patient with EDS type IV who previously had spontaneous dissection and multiple pseudoaneurysms of both the iliac and femoral arteries and the distal abdominal aorta. Several years later the patient developed a direct type A CCF which was successfully treated with endovascular embolization using a transvenous approach with detachable coils. The literature pertaining to CCF in EDS type IV and its treatment is reviewed.

Diagnosis, natural history, and management in vascular Ehlers–Danlos syndrome

“…This may be recognized by the affected individual because of sudden onset of a swishing sound in the temporal region of the head followed by injection of the veins of the eye, pain around the eye, and protrusion with slow loss of visual acuity. The only treatment is closure of the fistula, usually by an arterial catheter and coiling or occlusion of the carotid proximal to the fistula [Halbach et al, ; Linfante et al, ].…”

Vascular Ehlers-Danlos Syndrome Presenting as a Pulsatile Neck Mass: a Case Report and Review of Literature

“…Endovascular procedures carry a significant risk of arterial dissection and aneurysm formation and are not typically recommended 17 . However, there are case reports of successful endovascular procedures in affected patients 18,19 .…”

Successful treatment of direct carotid–cavernous fistula in a patient with Ehlers–Danlos syndrome type IV without arterial puncture: the transvenous triple-overlay embolization (TAILOREd) technique

“…Although a transvenous approach to cavernous sinus embolization may be less effective than transarterial embolization in the treatment of direct CCF, there is substantial risk associated with arterial access in patients with vEDS using this approach with reported mortality rates of up to 58% 3. Several authors have recommended a transvenous treatment approach for CCF in patients with vEDS; however, arterial puncture is often required in order to delineate the vascular anatomy 4. Other case reports have described direct distal arterial access within the neck requiring surgical cut-down 9.…”

“…To minimize arterial vascular complications, several authors have suggested a transvenous route of cavernous sinus embolization in vEDS CCF 4 5. Despite this treatment strategy, arterial access is still commonly required in order to identify the precise anatomy of the cavernous sinus and to evaluate the fistulous venous drainage pattern 4…”