Endovascular treatment of bilateral multiple carotid-cavernous fistulas in a patient with Ehlers-Danlos syndrome

Abstract

Introduction: In this article, we report the first patient with Ehlers-Danlos syndrome type IV (EDS IV) who developed multiple spontaneous contralateral direct carotid-cavernous fistulas (CCFs) after permanent occlusion of left internal carotid artery CCF 2 years before. We occluded the multiple fistulas and preserved the internal carotid artery (ICA) by using transarterial balloon-assisted embolization with coils and Onyx.

Case report: A 39-year-old woman presented with severe sudden-onset periorbital pain, increasing swelling, and proptosis of her left eye. Digital subtraction angiography (DSA) demonstrated direct left CCF. This patient underwent successful occlusion of left ICA and CCF by using detachable balloons. Two years later, she complained of a pulsatile intracranial bruit and then swelling and proptosis of her right eye.

Intervention: The right carotid artery angiogram revealed direct high-flow CCF with two fistulas in the cavernous segment of right ICA. Taking a transarterial approach, we occluded the two fistulas with GDC coils and Onyx-18 by an assisted balloon.

Conclusion: In the management of multiple CCFs in EDS IV patients, we are able to occlude the fistulas and leave the ICA intact by using transarterial balloon-assisted embolization. Intensive follow-up of treated arteries is mandatory, because EDS IV is widespread and can occur on contralateral vessels as well.