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Case Reports
. 2025;19(1):2024-0121.
doi: 10.5797/jnet.cr.2024-0121. Epub 2025 May 8.

Management of Direct Internal Carotid-Cavernous Sinus Fistula in a Patient with Ehlers-Danlos Syndrome: A Case Study on Selective Transvenous Embolization Using Coils and N-Butyl-2-Cyanoacrylate

Akina Hirohashi  1 , Shunsaku Goto  1   2 , Eiki Imaoka  2 , Masahiro Nishihori  2 , Takashi Izumi  2 , Shinsuke Muraoka  2 , Nobuhiko Mizutani  1 , Satoshi Ito  1 , Ryuta Saito  2
Affiliations
Case Reports

Management of Direct Internal Carotid-Cavernous Sinus Fistula in a Patient with Ehlers-Danlos Syndrome: A Case Study on Selective Transvenous Embolization Using Coils and N-Butyl-2-Cyanoacrylate

Akina Hirohashi et al. J Neuroendovasc Ther. 2025.

Abstract

Objective: Direct carotid-cavernous fistula (CCF) is a common neurovascular complication associated with Ehlers-Danlos syndrome (EDS). Nevertheless, reports indicate a significant incidence of treatment-related complications.

Case presentation: We present a case of right CCF in a 28-year-old female with EDS. Femoral artery and vein punctures were performed under ultrasound guidance. We executed transvenous embolization (TVE) of the draining veins and the shunted fistula using a combination of coils and n-butyl-2-cyanoacrylate (NBCA), facilitated by an assisted transarterial balloon. The CCF resolved without any procedural complications.

Conclusion: Utilizing a combination of coils and NBCA in TVE is seen as a safe and efficient method for addressing CCF in patients with EDS. It enables preserving better visualization of the cavernous sinus and adjacent structures, making this approach particularly effective. By keeping a close watch, monitoring for potential high-risk complications, and strategically placing devices between the arterial and venous sides, the arterial puncture profile is reduced, enabling safer endovascular treatment.

Keywords: direct carotid-cavernous fistula; transvenous embolization; vascular Ehlers–Danlos syndrome.

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Figures

Fig. 1
Fig. 1. Imaging findings in right ICA aneurysm and associated fistula. (A) Sagittal view of the 3D rotational angiography of the right ICA, revealing an aneurysm-like structure in the anterior wall of the cavernous segment. (B) Preoperative DSA (anterior view and lateral view) showing right CCF draining anteriorly into the right SOV, IOV, and pterygoid plexus via FOEV, as well as posteriorly into the UV, IPS, prepontine BV, and ICS. BV, bridging vein; CCF, carotid-cavernous fistula; FOEV, foramen ovale emissary vein; ICA, internal carotid artery; ICS, intercavernous sinus; IOV, inferior ophthalmic vein; IPS, inferior petrosal sinus; SOV, superior ophthalmic vein; UV, uncal vein
Fig. 2
Fig. 2. DSA results and schematic overview during TVE. (A) Selective embolization of the FOEV was performed using coils and NBCA. (Notable vessels include FOEV, arrow; UV, double arrow; and SOV and IOV, arrowhead). (B) Final angiographic view after transvenous embolization, highlighting an aneurysm-like structure (double arrowhead). (C) Schematic representation of the CS and ICA. CS, cavernous sinus; FOEV, foramen ovale emissary vein; ICA, internal carotid artery; IOV, inferior ophthalmic vein; NBCA, n-butyl-2-cyanoacrylate; SOV, superior ophthalmic vein; UV, uncal vein; TVE, transvenous embolization
Fig. 3
Fig. 3. Comparative DSA imaging of TVE procedures. (A) Postoperative DSA following the first TVE shows complete disappearance of intracranial venous reflux, with observed slow flow in the right CS and IPS. (B) Preoperative DSA for the second TVE reveals an increased flow in the shunt and reflux extending to the transverse pontine vein and the contralateral SPS. CS, cavernous sinus; IPS, inferior petrosal sinus; SPS, superior petrosal sinus; TVE, transvenous embolization
Fig. 4
Fig. 4. Intraoperative imaging and intervention during the second TVE. (A) DSA of the right CCF showing antegrade flow in the IPS, retrograde flow in the BV (arrow), including transverse pontine vein, and the contralateral SPS. The angiographic images were acquired at right anterior oblique 42° (RAO 33°) and caudal 33° (CAU 40°) projections. (B) Micro-DSA of the prepontine BV indicating retrograde flow in the transverse pontine vein and the contralateral SPS (RAO 33°/CAU 40°). (C) Embolization of the prepontine bridging vein using coils and 20% NBCA (RAO 33°/CAU 40°). (D) Additional coil embolization of the aneurysm via a venous approach and balloon assistance through an arterial approach. (E) Complete resolution of shunt flow was observed following the second TVE. BV, bridging vein; CCF, carotid-cavernous fistula; IPS, inferior petrosal sinus; NBCA, n-butyl-2-cyanoacrylate; SPS, superior petrosal sinus; TVE, transvenous embolization
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References

    1. Freeman RK, Swegle J, Sise MJ. The surgical complications of Ehlers-Danlos syndrome. Am Surg 1996; 62: 869–873. - PubMed
    1. Schievink WI, Piepgras DG, Earnest F 4th, et al. Spontaneous carotid-cavernous fistulae in Ehlers-Danlos syndrome Type IV. J Neurosurg 1991; 74: 991–998. - PubMed
    1. Barrow DL, Spector RH, Braun IF, et al. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg 1985; 62: 248–256. - PubMed
    1. Srienc AI, Huguenard AL, Gupta VP, et al. Carotid Cavernous Fistulas and Vein of Galen Malformations. In: Mascitelli JR, Binning MJ. ed. Introduction to Vascular Neurosurgery. Springer. Cham, 2022, 421–437. 10.1007/978-3-030-88196-2_22. - DOI
    1. Hollands JK, Santarius T, Kirkpatrick PJ, et al. Treatment of a direct carotid-cavernous fistula in a patient with type IV Ehlers-Danlos syndrome: a novel approach. Neuroradiology 2006; 48: 491–494. - PubMed

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