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B. I. Rubin, S. Shroff, N. Plass, R. Goldbach-Mansky; Optic Nerve Elevation Evaluation in Patients With Nomid (neonatal Onset Multisystem Inflammatory Disease). Invest. Ophthalmol. Vis. Sci. 2007;48(13):944.
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20 patients between the ages of 14 months and 34 years with CIAS1 mutation positive and negative NOMID were systematically evaluated by a single ophthalmologist (BR). Evaluation included a slit lamp exam, dilated exam of the posterior retina. When ever possible Visual acuity. Visual field, photography and OCT of the optic nerve were performed.
Papilledema was the most common feature, occurring in 14 patients (70%), optical pallor, indicating optic nerve atrophy, in 8 patients (40%) and vascular sheathing in 4 patients (20%). Anterior segment manifestations included corneal opacities in 10 patients (50%) and cataract in 5 patients (25%). Iritis while not observed in previous studies was noted in 7 patients (35%) and 2 patients (10%) presented with posterior uveitis. The lowest visual acuity at baseline visit was 20/800 and highest was 20/16. Visual field reductions were seen in a large proportion of patients. Patients with elevated ICP and optic nerve elevation were treated with a combination of Anakinra, steroids and Diamox. Optic nerve elevation and change were documented by serial examinations and OCT.
Ocular manifestations of all levels of the eye and with potentially sight-threatening complications occur commonly in the NOMID syndrome. Familiarity with the unique profile of ocular complications will assist the ophthalmologist in diagnosing this rare disorder and help in distinguishing it from JRA. Once diagnosed NOMID patients should remain under the care of an ophthalmologist, since vision-threatening complications may develop and a team approach is needed in addressing elevated ICP
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