J Korean Ophthalmol Soc.  2016 Jun;57(6):977-982. 10.3341/jkos.2016.57.6.977.

A Case of Cavernous Sinus Dural Arteriovenous Fistula Following Tolosa-Hunt Syndrome

• Park A
• Kim SW
• Chung SA

• KMID: 2290502
• DOI: http://doi.org/10.3341/jkos.2016.57.6.977

Abstract

PURPOSE
To report a case of cavernous sinus dural arteriovenous fistula following Tolosa-Hunt syndrome.
CASE SUMMARY
A 64-year-old female with a history of hypertension, presented with blepharoptosis and periorbital pain in the right eye and diplopia. Her right pupil was dilated. She had right exotropia and right hypertropia with inability to elevate, depress, and adduct the right eye. Magnetic resonance imaging including angiography, revealed hyperintensities in the right cavernous sinus consistent with inflammation and no vascular abnormalities. Three days after oral corticosteroid therapy, the pain disappeared. A presumptive diagnosis was Tolosa-Hunt syndrome presenting as a form of complete oculomotor nerve palsy. Two months later, she experienced severe pain in the right periocular area, even though blepharoptosis was resolved and ocular motility was improved. A rapid response to high-dose intravenous corticosteroids was consistent with recurrence of Tolosa-Hunt syndrome. Three months later, she had normal ocular motility, but developed a conjunctival injection, pulsatile orbital bruits, and exophthalmos in the right eye. Cerebral angiography showed a cavernous sinus dural arteriovenous fistula. She received a transvenous coil embolization and her symptoms markedly improved over 2 months.
CONCLUSIONS
Tolosa-Hunt syndrome, a granulomatous inflammation in the cavernous sinus may be followed by cavernous sinus dural arteriovenous fistula and should be considered during follow-up.

Keyword

Cavernous sinus; Dural arteriovenous fistula; Tolosa-Hunt syndrome

MeSH Terms

Adrenal Cortex Hormones
Angiography
Blepharoptosis
Cavernous Sinus*
Central Nervous System Vascular Malformations*
Cerebral Angiography
Diagnosis
Diplopia
Embolization, Therapeutic
Exophthalmos
Exotropia
Female
Follow-Up Studies
Humans
Hypertension
Inflammation
Magnetic Resonance Imaging
Middle Aged
Oculomotor Nerve Diseases
Orbit
Pupil
Recurrence
Strabismus
Tolosa-Hunt Syndrome*
Adrenal Cortex Hormones

Figure

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  Figure 1. Clinical findings and imaging at initial presentation. (A) Gaze photographs showing severe limitation on supraduction, adduction, and infraduction in the right eye. (B) Photographs showing blepharoptosis, and dilated pupil in the right eye. (C) Brain magnetic resonance angiography (MRA) not showing any abnormality such as aneurysmal dilatation or compressed lesion. (D) Gadolinium enhanced T1-weighted brain magnetic resonance image (MRI) showing increased signal intensity in the right cavernous sinus, indicated by red circle. P = posterior aspect; A = anterior aspect.

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  Figure 2. Gaze photographs 2 months after initial presentation when she suffered from severe periocular pain and headache, showing resolution of blepharoptosis and improvement in ocular motility in the right eye.

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  Figure 3. Clinical findings and imaging 5 months after initial presentation. (A) Gaze photographs showing resolution of the right oculomotor nerve palsy, but severe redness in the right eye. Slit lamp photographs showing engorged vessels on the conjunctiva of right eye. (B) Cerebral angiography showing arteriovenous shunt from the right middle meningeal artery and ascending pharyngeal artery, supplied from the right external carotid artery to the cavernous sinus (type C in Barrow classification,10 red arrow, left column), and no more shunt after coil embolization (right column).

Reference

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