Ophthalmic manifestations of neonatal onset multisystem inflammatory disease

PURPOSE: To report the ophthalmic manifestations of neonatal onset multisys tem inflammatory disease, a recently recognized, rare systemic disorder cha racterized by the triad of arthropathy, rash, and abnormal central nervous system development. METHOD: Case report. RESULTS: A 2-year-old female with neonatal onset multisystem inflammatory d isease presented with visual acuity of fix and follow with each eye, bilate ral optic nerve head pallor and gliosis, as well as marked sheathing of the peripapillary vessels. No vitreous inflammation or macular edema was found , Visual acuity was stable from the neonatal period through the 3 months of follow-up after the changes involving the optic nerve heads and peripapill ary vessels were observed (a total of 33 months). CONCLUSIONS: This report provides a description of the ocular manifestation s of neonatal onset multisystem inflammatory disease and supports earlier s uggestions that swelling of the optic nerve heads can occur, In this case, optic nerve head pallor may have been a sequela of such swelling. The patho genesis of neonatal onset multisystem inflammatory disease is unknown. (C) 2000 by Elsevier Science Inc. All rights reserved.