Selective Shunt Occlusion of Direct Carotid-Cavernous Fistula with Vascular Ehlers-Danlos Syndrome by Multidevice Technique: A Case Report and Technical Note.

BACKGROUND: Vascular Ehlers-Danlos syndrome (vEDS) is a vascular disease associated with a genetic collagen abnormality. It is characterized by vessel fragility, vessel rupture, and massive hemorrhage. Carotid-cavernous fistula (CCF) is the most frequent neurovascular complication of vEDS. However, CCF treatment using conventional diagnostic angiography and neuroendovascular therapy can result in a high rate of major complications.

CASE DESCRIPTION: We report a case of a right CCF in a 48-year-old man with vEDS. The carotid artery and jugular vein were exposed by direct neck dissection. To avoid systemic vascular complications, multiple catheters were inserted into the shunt segment via the carotid artery and jugular vein. This transarterial and transvenous multi-device technique enabled compact placement of coils in the shunt segment. The CCF was eliminated via selective shunt occlusion. Postoperative magnetic resonance imaging revealed occlusion of the right CCF.

CONCLUSION: Selective shunt occlusion via a transarterial and transvenous multidevice technique is a useful and safe approach for treating vEDS-associated CCF.