Journal of Neuro-Ophthalmology:
Monocular Nasal Hemianopia From Atypical Sphenoid Wing Meningioma
Stacy, Rebecca C MD, PhD; Jakobiec, Frederick A MD, DSc; Lessell, Simmons MD; Cestari, Dean M MD
Department of Neuro-Ophthalmology (RCS, SL, DMC); and David G. Cogan Laboratory of Ocular Pathology (RCS, FAJ), Massachusetts Eye and Ear Infirmary, Boston, Massachusetts
None of the authors has any financial conflicts of interest.
R. C. Stacy is supported in part by the Heed Foundation.
Address correspondence to Rebecca C. Stacy, MD, PhD, Suite 321, 243 Charles Street, Boston, MA 02114; E-mail: Rebecca_Stacy@meei.harvard.edu.
Neurogenic monocular nasal field defects respecting the vertical midline are quite uncommon. We report a case of a unilateral nasal hemianopia that was caused by compression of the left optic nerve by a sphenoid wing meningioma. Histological examination revealed that the pathology of the meningioma was consistent with that of an atypical meningioma, which carries a guarded prognosis with increased chance of recurrence. The tumor was debulked surgically, and the patient's visual field defect improved.
A 55-year-old woman presented with sudden painless blurred vision of the left eye. The patient had a history of hypertension, diabetes, and hypercholesterolemia, but her ophthalmic and neurologic histories were negative. She was evaluated elsewhere within 2 days of the onset of her symptoms, and nonarteritic anterior ischemic optic neuropathy (NAION) was diagnosed. We evaluated her 1 week after the onset of symptoms. The patient's visual acuity was 20/15 in the right eye and 20/25 in the left eye, with dyschromatopsia and a relative afferent pupillary defect in the left eye. No strabismus or motility disturbances were detected, and the left eye had 2 mm of proptosis. Automated perimetry showed a full field in the right eye and a nasal defect in the left eye (Fig. 1). There was temporal pallor of the left optic nerve with no disc edema.
Brain MRI with gadolinium revealed a tumor involving the left sphenoid wing measuring 3.3 × 3.6 × 4 cm with extension into the left cavernous sinus and orbital apex, compressing the left optic chiasm and left side of the optic nerve (Fig. 2). The tumor was debulked via a left frontotemporal craniotomy. Postoperative neuro-ophthalmologic examination showed that the vision was 20/20 in the left eye with a persistent relative afferent pupillary defect. Repeat visual fields showed improvement of the left nasal field defect (Fig. 3).
Histopathologic evaluation of the lesion revealed a meningioma proliferating in a hypercellular sheet-like fashion with interspersed hemorrhage (Fig. 4A) and foci of fresh necrosis, which lay adjacent to hyalinized fibrosis (Fig. 4B). Some regions manifested the whorl-like morphology of a meningoepithelial meningioma (Fig. 4C), while others contained spindle cells typical of a fibrous meningioma. The nuclei within some of the tumor cells were atypical manifesting prominent nucleoli (Fig. 4D). The findings of sheeting, hypercellularity, atypical nuclei with prominent nucleoli, and areas of necrosis were consistent with a diagnosis of atypical meningioma (1,2). A postoperative MRI demonstrated residual tumor abutting the intracranial portion of the left optic nerve. The patient is currently scheduled for treatment with radiation oncology.
Unilateral nasal field defects are extremely rare, and when organic, are often associated with optical phenomena such as subcapsular or traumatic cataract (3,4). Nasal field defects have rarely resulted from aneurysms and mass lesions compressing the temporal side of the optic nerve (5-8). In one case series, nasal field defects due to a dolichoectatic carotid artery, optochiasmatic arachnoiditis, meningioma, or pituitary tumor were associated with optic nerve compression and compromised visual acuity (9). In contrast to our patient's defect, in these cases, the superior portions of the nasal fields were often retained. When the meningioma compressing the optic nerve was debulked, the patient's field defect and acuity improved. This is consistent with previous reports.
Histological evidence shows that retinal ganglion cell axons that do not cross at the chiasm acquire their temporal arrangement in the optic nerve before they reach the optic foramen (10). Therefore, a sphenoid wing meningioma or other lesion that compresses the temporal side of the optic nerve would affect the nondecussating fibers resulting in a nasal field defect. With long-standing compression, temporal nerve pallor, as was apparent in our patient, may result. NAION was a diagnostic consideration in this patient with a history of sudden vision loss, hypertension, and diabetes. However, the absence of optic disc swelling at the time of presentation made the diagnosis of NAION unlikely (11). The onset of visual symptoms, which the patient interpreted as sudden, was almost certainly pseudosudden since optic atrophy was already present.
Histopathologic review of the patient's lesion revealed an atypical meningioma, which accounts for approximately 15% of meningiomas and are classified as World Health Organization Grade II lesions (12). The sheeting architecture, macronucleoli, and hypercellularity that may reflect a dedifferentiated state can be interspersed with more benign pathologic characteristics (2). Because atypical meningiomas have a higher chance for recurrence than the more common benign lesions, approaching 40% as compared with 12% for Grade I meningiomas (1), all areas of the biopsied meningioma must be sampled and carefully examined to avoid overlooking an atypical meningioma. Total resection of the lesion can reduce the rate of recurrence and adjuvant fractionated radiotherapy or stereotactic radiosurgery may help to control regrowth (13).
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