Gaze evoked amaurosis is a rare symptom usually explained by vascular compromise of the retina or optic nerve in an eccentric gaze position caused by orbital and even intracranial disease (optic nerve sheath meningioma,1, 2 intraconal cavernous haemangioma,1, 2 orbital birdshot pellet,3 dysthyroid orbitopathy,4 pseudotumour cerebri,5 orbital fracture,6 nasopharyngeal angiofibroma,6 idiopathic intracranial hypertension,7 intracranial aneurysm).8
We report a patient with cavernous sinus meningioma who presented with symptoms of loss of vision of the right eye in right gaze.
A 37-year-old female patient was referred to our clinic by a neurologist for evaluation of right-sided visual loss occurring rapidly after initiating and sustaining right gaze. Vision recovered quickly on returning to primary gaze.
There was a 3 years' history of vertigo in right gaze, followed 1 year later by attacks of right-sided pain in the head, ear, and eye initiated on looking right, subsiding on returning to primary gaze. A neurological examination including MRI of head and neck revealed no explanation.
On a repeat neurological examination (3 years later) gaze-evoked amaurosis was reported and a nystagmus was noted in right gaze, which prompted an ophthalmologic referral.
The ophthalmologic examination revealed visual acuities of 6/6 OU. Visual acuity of the right eye dropped repeatedly to 6/18 within 5 s after assuming right gaze. Our patient reported decreasing vision moving from the temporal periphery to the centre of vision. After half a minute of sustained right gaze, a horizontal pendular nystagmus was noted in the fixating right eye. Vision recovered to 6/6 s after returning to the primary position. Eye movements were normal, there was no proptosis and no ptosis, pupillary light reactions were normal, the fundi showed no abnormalities (no papilloedema).
A visual field test showed no abnormalities in PP and a central scotoma in right gaze (Figures 1a, b). A fluorescein angiogram (in right gaze) showed no abnormalities. VEP responses decreased markedly over both hemispheres in right gaze (beyond 30°) (Figure 2). A CDI showed a higher resistance index of the right ophthalmic artery in right gaze (Figure 3). An MRI showed a meningioma of the right cavernous sinus displacing the intracavernous internal carotid artery (Figures 4, 5 and 6) and no abnormalities were seen in the right orbit and optic canal.
A conservative management was advised in view of the relatively benign symptoms and the possibility of surgery-related morbidity.
At 6 years after the onset of symptoms, our patient's condition has changed little apart from the recent onset of corneal hypesthesia.
To our knowledge, this is the first report of gaze-evoked amaurosis with an intracavernous sinus meningioma. The cause of visual loss is undoubtedly vascular of origin in view of its rapid onset and higher resistance index of the right ophthalmic artery as shown in the CDI with dextroversion of right eye.
Our patient refused to undergo an internal carotid artery angiography which might have yielded further interesting results to explain this phenomenon.
We assume that the perfusion of the right ophthalmic artery is compromised at its origin by compression, distortion and/or displacement of the internal carotid artery by the adjacent meningioma (Figure 4) without causing visual loss. On abduction of the right eye this haemodynamic compromise increases due to stretch or adjacent tissue compression on an artery8 leading to amaurosis. The location of this compromise could be anywhere—and possibly in multiple locations—from the origin of the ophthalmic artery up to the feeding vessels of the optic nerve in the meninges around the intracanalicular part of the optic nerve or even in the orbital apex.
In summary, we demonstrate a case that illustrates that gaze-evoked amaurosis may be caused, as previously reported,8 by intracranial pathology that compromises blood perfusion.
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We are grateful to the imaging departments of Delfzicht Ziekenhuis for providing CDI images, Electro physiological Ophthalmic Department, University Medical Center, Groningen for providing VEP images and Dr Mciners of Neuro radiology Department, University Medical Center, Groningen for the MRI images.
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Koch, M., Houtman, A. & de Keizer, R. Gaze-evoked amaurosis with cavernous sinus meningioma. Eye 20, 840–843 (2006). https://doi.org/10.1038/sj.eye.6701977
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