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Case Reports
. 2014 Nov 24:2014:bcr2014011414.
doi: 10.1136/bcr-2014-011414.

A novel approach to the treatment of a direct carotid-cavernous fistula in a patient with Ehlers-Danlos syndrome type IV

Ichiro Nakagawa  1 , Hun Soo Park  1 , Takeshi Wada  2 , Katsutoshi Takayama  2 , Hiroyuki Nakagawa  2 , Kimihiko Kichikawa  2 , Hiroyuki Nakase  1
Affiliations
Case Reports

A novel approach to the treatment of a direct carotid-cavernous fistula in a patient with Ehlers-Danlos syndrome type IV

Ichiro Nakagawa et al. BMJ Case Rep. .

Abstract

We report a case of a direct carotid-cavernous fistula (CCF) in a patient with Ehlers-Danlos syndrome type IV who presented with progressive chemosis and diplopia. To prevent potential lethal arterial wall injury due to the fragility of the arterial vessel wall, the ipsilateral carotid artery and internal jugular vein were surgically exposed for direct insertion of endovascular sheaths, and transvenous embolization was performed using triple microcatheters with detachable coils. The clinical course was uneventful, and chemosis and diplopia subsequently resolved. By the 6 month follow-up, MRI revealed no recurrence of the CCF. These techniques offer a unique access alternative for endovascular treatment, thereby reducing the risks associated with arterial dissection that often accompanies transfemoral access in this particular condition.

Keywords: Fistula; Intervention; Vessel Wall.

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Figures

Figure 1
Figure 1
MR angiography at admission showing an abnormally dilated right superficial middle cerebral vein (arrows) and bilateral superior ophthalmic veins (arrowheads) as an indication of a carotid–cavernous fistula (A, B). Susceptibility weighted venogram imaging reveals marked venous congestion in the right cerebral hemisphere (C).
Figure 2
Figure 2
Photograph showing direct neck dissection. A 4 F sheath was introduced into the common carotid artery, and a 5 F sheath was introduced into the internal jugular vein.
Figure 3
Figure 3
Right common carotid artery (CCA) angiogram (anteroposterior view) showing right carotid–cavernous fistula with multiple routes of venous drainage (A). Right CCA angiogram (lateral view) showing a guidewire balloon catheter introduced into the fistulous portion of the carotid siphon (B, C; white arrow) and triple microcatheters introduced into the cavernous sinus (B, C; black arrows). Right CCA angiogram (lateral view) showing transvenous coil embolization, superficial middle cerebral vein outflow occlusion (D; black arrow), uncal vein outflow occlusion (D; white arrow), right superior ophthalmic vein (SOV) outflow occlusion (E; white arrow), left SOV and intercavernous sinus outflow occlusion, and right cavernous sinus packing (E; black arrow).
Figure 4
Figure 4
Follow-up MR angiography at 6 months showing no new carotid artery dissection (A, arrows) and occlusion of the right carotid–cavernous fistula (B, arrowhead).
See this image and copyright information in PMC

References

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