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Martina M. Bosch, MD; Werner W. Wichmann, MD; Eugen Boltshauser, MD; Klara Landau, MD

Arch Ophthalmol. 2006;124:379-385.

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.

Author Affiliations: Department of Ophthalmology, University Hospital Zurich (Drs Bosch and Landau); Institute of Neuroradiology and Radiology, Klinik im Park (Dr Wichmann); and Department of Neurology, University Children's Hospital Zurich (Dr Boltshauser), Zurich, Switzerland.