Contralateral Ocular Manifestations of a Carotid Cavernous Fistula Associated With Primitive Persistent Trigeminal Artery

A 41-year-old woman developed binocular diplopia and redness of her right eye after a motorcycle accident. She was diagnosed with a left carotid cavernous fistula causing contralateral manifestations through intercavernous sinus venous connections related to ruptured persistent primitive trigeminal artery.

Trainees’ Corner Section Editors: Vivek R. Patel, MD Prem Subramanian, MD, PhD Contralateral Ocular Manifestations of a Carotid Cavernous Fistula Associated With Primitive Persistent Trigeminal Artery Tung Thanh Hoang, MD, Cuong Ngoc Nguyen, MD, Anh Quoc Nguyen, MD, Hieu Lan Nguyen, MD, PhD, Prem S. Subramanian, MD, PhD, Van Trong Pham, MD, PhD A 41-year-old woman developed binocular diplopia and redness of her right eye after a motorcycle accident. She was diagnosed with a left carotid cavernous fistula causing contralateral manifestations through intercavernous sinus venous connections related to ruptured persistent primitive trigeminal artery. She underwent a coil embolization procedure to fix the fistula. Her ophthalmic manifestations improved after the intervention. This case serves as a reminder of the complex anatomy of venous drainage pathways at the skull base and the potential for traumatic lesions to cause contralateral or even bilateral symptoms. CASE PRESENTATION A 41-year-old woman reported a 10-day history of binocular diplopia and redness in the right eye. Two months before, she was involved in a motorcycle collision. She initially presented with redness of her right eye and was diagnosed elsewhere with conjunctivitis. She then developed progressive horizontal double vision and mild pulsatile tinnitus in the right ear without any blurry vision, eye pain, headache, dizziness, or nausea. Ocular examination revealed that her visual acuity was 20/25 in the right eye and 20/20 in the left eye with elevated intraocular pressure in the right eye (34 mm Hg in the right eye and 19 mm Hg in the left eye). General ophthalmoparesis of the right eye was noted with manifest exotropia; the left eye moved normally (Fig. 1A). There was no ptosis. No relative afferent pupillary defect or anisocoria was present. Anterior segment examination of the right eye showed diffuse conjunctival injection with corkscrewing pattern of the blood vessels (Fig. 1A). This appearance and right ocular hypertension suggested increased episcleral venous pressure. Right funduscopy demonstrated no abnormality in cup/disc ratio or venous stasis pattern. No abnormality was found in the left eye. The patient had no neurological deficits, and her systemic review was stable. A dilated right superior ophthalmic vein without any other abnormality of the orbital structures was detected with B-scan echography and confirmed by MRI of brain and orbits. A cerebral angiogram thus was performed and demonstrated a left carotid cavernous fistula (CCF) inducing contralateral manifestations through intercavernous sinus venous connections (Fig. 2A) related to persistent primitive trigeminal artery (PPTA). The patient underwent a transarterial endovascular embolization of trigeminal artery with Guglielmi detachable coils (Axium 4/8 mm and 3/6 mm, EV3) in the left side (Fig. 2B, C). After the intervention, clinical signs and symptoms improved rapidly with decrease of intraocular pressure to 18 mm Hg in the right eye and 15 mm Hg in the left eye, decreased redness in the right eye, and improved motility (Fig. 1B). The patient was discharged 3 days after the procedure and remained stable in follow-up with resolution of diplopia. Ophthalmology Department (TTH, VTP), Hanoi Medical University, Vietnam; Ophthalmology Unit (TTH), Hanoi Medical University Hospital, Vietnam; Save Sight Institute (TTH), the University of Sydney School of Medicine, Australia; Radiology Department (CNN), Hanoi Medical University Hospital, Vietnam; Oculoplastic and Cosmesis Surgery Department (AQN), Vietnam National Eye Hospital; Cardiology Center (HLN), Hanoi Medical University Hospital, Vietnam; and Ophthalmology Department (PSS), University of Colorado School of Medicine. The authors report no conflicts of interest. T. T. Hoang and C. N. Nguyen share the cofirst authorship. Address correspondence to Tung Thanh Hoang, Hanoi Medical University, No 1, Ton That Tung Street, Dong Da distr, Hanoi 11518, Vietnam; E-mail: [e-mail] Hoang et al: J Neuro-Ophthalmol 2021; 41: e803-e805 FIG. 1. A. Before intervention and (B) 3 days after coil embolization. e803 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Trainees’ Corner FIG. 2. Findings in DSA. A. Manifestations of the right side induced by left CCF. B. No shunt in the right side after coil embolization of left CCF. C. Left PPTA after coil embolization. D. 3D DSA of left PPTA. DISCUSSION Our case demonstrates 2 unusual features in a patient with signs and symptoms suggesting a classic posttraumatic (direct) CCF. Direct CCF typically manifests with ophthalmologic symptoms on the ipsilateral side. However, in this case, signs and symptoms were solely contralateral to the fistula because of intercavernous channels that were involved preferentially by the fistula, and the ipsilateral eye, orbit, and cavernous sinus were normal. In addition, the fistula arose not from the internal carotid artery (ICA) but instead from a PPTA. PPTA is a persistent embryological carotid-basilar anastomosis in adults that normally regresses prenatally (1). The failure of this regression has been associated with serious complications such as cerebral artery aneurysm, dural arteriovenous fistula, trigeminal neuralgia, and ischemic stroke (2). PPTA also may be asymptomatic and detected only by digital subtraction angiography (DSA) in posttraumatic scenarios like our case in which PPTA was ruptured after a mild head injury. The incidence of PPTA has been reported to vary from 0.02% to 0.6% (1). Posttraumatic CCF associated with PPTA is quite uncommon, although its relative fragility may make the vessel subject to traumatic disruption insufficient to injure the ICA (3). Both contralateral (4) and bilateral (5) findings have been described in association with direct CCF from ICA injury. Similarly, there are prior case reports of traumatic CCF with PPTA involvement (6) and ipsilateral e804 orbital bruit (1) as well as spontaneous CCFs associated with PPTA inducing the manifestations of the ipsilateral side (7,8). However, this patient seems to be the first reported case to have a CCF fed by PPTA on one side causing ophthalmic manifestations solely on the contralateral side. Depending on the direction of fistula shunt, all or some oculomotor nerves could be affected, which might cause complete or incomplete ophthalmoplegia. Orbital congestion also may contribute to decreased motility. A CCF should always be highly suspected in any case with conjunctival injection with corkscrewing pattern of the blood vessels, ocular hypertension, ophthalmoplegia, a dilation of the superior ophthalmic vein, and a positive history of head trauma. Differentiating a PPTA-involving CCF from typical CCF due to ICA laceration is critical so that the appropriate treatment strategy is undertaken. Both types have been treated with detachable coils (3) and/or balloon embolization (6), but correct identification of the feeding arterial vessels allows for targeted embolization and maximal preservation of the remaining vascular tree. In our case, we consider coil embolization as the most appropriate treatment based on the anatomy. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: T. T. Hoang, C. N. Nguyen, A. Q. Nguyen, H. L. Nguyen, P. S. Subramanian, and V. T. Pham; b. Hoang et al: J Neuro-Ophthalmol 2021; 41: e803-e805 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Trainees’ Corner Acquisition of data: T. T. Hoang, C. N. Nguyen, A. Q. Nguyen, H. L. Nguyen, P. S. Subramanian, and V. T. Pham; c. Analysis and interpretation of data: T. T. Hoang, C. N. Nguyen, A. Q. Nguyen, H. L. Nguyen, P. S. Subramanian, and V. T. Pham. Category 2: a. Drafting the manuscript: T. T. Hoang, C. N. Nguyen, A. Q. Nguyen, H. L. Nguyen, P. S. Subramanian, and V. T. Pham; b. Revising it for intellectual content: T. T. Hoang, C. N. Nguyen, A. Q. Nguyen, H. L. Nguyen, P. S. Subramanian, and V. T. Pham. Category 3: a. Final approval of the completed manuscript: T. T. Hoang, C. N. Nguyen, A. Q. Nguyen, H. L. Nguyen, P. S. Subramanian, and V. T. Pham. 4. 5. 6. REFERENCES 1. Ohshima T, Kawaguchi R, Miyachi S, Matsuo N. Traumatic carotid-cavernous fistula associated with persistent primitive trigeminal artery successfully treated using in-stent coil embolization. World Neurosurg X. 2019;128:360–364. 2. Alcalá-Cerra G, Tubbs RS, Niño-Hernández LM. Anatomical features and clinical relevance of a persistent trigeminal artery. Surg Neurol Int. 2012;3:111. 3. Tokunaga K, Sugiu K, Kameda M, Sakai K, Terasaka K, Higashi T, Date I. 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