Ophthalmologic Finding and Long-Term Coure in Patient With Neurofibromatoi Type 2

Purpoe To evaluate ophthalmologic finding and long-term coure in patient with neurofibromatoi type 2 (NF2). Deign Retropective obervational cae erie. Method etting: ingle-center intitutional practice. tudy population: Thirty referred patient with NF2 were enrolled from 1991 to 2003 and underwent at leat one thorough neuroophthalmologic examination. Follow-up of 23 patient ranged from 4.3 to 12.5 year. main outcome meaure: Viual function, tructural ocular abnormalitie, onet and type of preenting NF2-related ymptom, and number of central nervou ytem tumor. Reult Initial ymptom for patient with early-onet NF2 motly compried ophthalmologic ymptom (n = 7) and lower motor neuron extremity weakne (n = 6), a oppoed to eighth nerve impairment (n = 11) in late dieae onet. NF2-pecific ocular finding were noted in 83% of all patient (94% childhood onet; 67% adult onet): 67% had cataract, 40% epiretinal membrane, 3% hamartoma, 13% dik glioma, and 27% optic nerve heath meningioma. Only 14% in the childhood-onet group—a oppoed to 78% in the adult-onet group—utained viual acuity of 1.0 in both eye at final examination. ignificantly more patient with early onet of ymptom developed multiple central nervou ytem tumor (P = .004) and howed a higher amount of NF2-pecific finding (P = .015). Concluion Initial manifetation of NF2 differ between children and adult. NF2-pecific ophthalmologic finding can help etablih the diagnoi. ymptom onet at a young age i clearly a rik factor for marked dieae progreion. Thee patient hould be carefully followed becaue urvival rate have increaed, and viion become increaingly important a the dieae progree.