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B. Barahimi, R. Sobel, J. R. Bilyk, R. C. Sergott, P. J. Savino; Optic Nerve Sheath Meningioma: The Differential Diagnosis. Invest. Ophthalmol. Vis. Sci. 2009;50(13):5744.
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To assess and compare the distinguishing clinical features in patients with optic nerve lesions and presumed optic nerve sheath meningioma (ONSM).
A retrospective chart review of patients with optic nerve lesions presenting to a neuro-ophthalmology and orbital practice at Wills Eye Institute from 1991 to present was performed.
Sixty-five patients (73 optic nerves) were identified. The most common diagnosis was presumed ONSM (48%), followed by anterior visual pathway sarcoidosis (AVPS) (33%), juvenile glioma (11%), and aggressive glioma of adulthood (malignant glioma) (4%). ONSM and AVPS shared similar characteristics with regards to age and gender distribution, resulting in confusion between AVPS and the tubular form of ONSM. Juvenile glioma and adult glioma were clinically distinct from ONSM. However, adult glioma was frequently confused with ischemic optic neuropathy during initial clinical evaluation. AVPS was also found to vary widely in clinical duration, and progression of visual loss, and response to systemic corticosteroid therapy.
Although ONSM and AVPS share similar characteristics, the combination of history, clinical exam, serologic testing, and imaging aid in distinguishing each entity. Angiotensin converting enzyme and either chest X-ray or CT should be performed in all patients presenting with presumed ONSM, especially if the tubular variant is noted on neuroimaging, in order to exclude AVPS.
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