Optic nerve sheath meningiomas in patients with neurofibromatosis type 2.
OBJECTIVE
To determine the prevalence of optic nerve sheath meningiomas (ONSMs) in patients with neurofibromatosis type 2 (NF2).


METHODS
An observational retrospective case series of 30 consecutive patients with NF2 referred to an academic ophthalmology unit from November 1, 1991, through August 31, 2003. Twenty-six patients were followed up for a mean of 93 months (range, 3-150 months). One individual was lost to follow-up, and 3 had been referred recently. Diagnosis of ONSM was made based on typical neuroradiologic and clinical features in 7 patients and on histologic criteria in 1.


RESULTS
Eight of 30 patients harbored unilateral (n = 6) or bilateral (n = 2) ONSMs. Six ONSMs were diagnosed at initial examination, and 4 during follow-up.


CONCLUSIONS
There is a strong association between ONSMs and NF2 that parallels the well-known association of optic nerve gliomas with NF1. Physicians should be aware of the possibility that patients with ONSMs may also have NF2.
Department of Ophthalmology, University Hospital Zurich, Switzerland.
Martina M
MM
Bosch
Werner W
WW
Wichmann
Eugen
E
Boltshauser
Klara
K
Landau
D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008579:Meningioma / Q000175:diagnosis*; D008875:Middle Aged; D016518:Neurofibromatosis 2 / Q000175:diagnosis*; D019574:Optic Nerve Neoplasms / Q000175:diagnosis*; D015995:Prevalence; D012189:Retrospective Studies