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J.M. Facciani, P.A. Nixon, L.B. Kline, R.E. Turbin; Early Onset Pediatric Cavernous Sinus Meningioma and Neurofibromatosis Type 2 (NF2) . Invest. Ophthalmol. Vis. Sci. 2003;44(13):638.
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Purpose: To report the largest known series of children with cavernous sinus meningioma, comitant strabismus, and multiple intracranial and extracranial tumors. Methods: Retrospective chart review of 2 patients. Results: A 4 month old boy and 4 year old girl developed comitant esotropia and exotropia, respectively. Abnormal visual acuity was first recognized at ages four and five years (Hand Motion with afferent pupillary dysfunction [APD]; and 20/40 with APD). The former child developed third nerve dysfunction by age 6, two years after the onset of visual dysfunction, with ptosis, hypotropia and an ipsilateral elevation deficit. The latter child presented with third nerve dysfunction at the time of the visual loss with evident ptosis and marked deficits of elevation, depression, and adduction. Additional investigations ultimately revealed bilateral cavernous sinus meningioma, biopsy proven optic nerve sheath meningioma (psammomatous), an optic nerve head abnormality, a cervical and facial neurofibroma, and bilateral acoustic neuromas in the first child by age 7. The second child had biopsy proven cavernous sinus meningioma (psammomatous with reparative neuroma), a green pinpoint refractile crystalline lens abnormality, bilateral early acoustic neuroma, and spinal ependymoma at the time of initial presentation. Conclusion: Cavernous sinus meningioma in children are extremely rare, and should prompt an evaluation for NF2. These tumors may initially present as comitant strabismus, which may progress to an incomitant deviation more consistent with cranial nerve dysfunction. The presence of optic nerve sheath involvement and ipsilateral cavernous sinus involvement by meningioma may result from tumor multifocality, but the possibility of indirect extension between the nerve sheath, orbital apex, superior orbital fissure and cavernous sinus may occur and is difficult to distinguish on imaging studies.
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