Optic perineuritis is a rare orbital inflammatory disease that typically presents with unilateral symptoms, but may be bilateral and is rare in children. Bilateral uveitis and scleritis are uncommon in children. Rarely, perineuritis, sceritis and uveitis have been reported following influenza vaccination. To our best knowledge, optic perineuritis, scleritis and uveitis occurring simultaneously following vaccination has not been reported.
A seven-year-old female patient presented with two months of bilateral eye pain, vision loss, extreme photophobia and sleepiness that started two weeks following influenza vaccination. At presentation she had decreased visual acuity (VA) bilaterally (right, 20/100; left, 20/80), bilateral scleral erythema, and bilateral optic disc edema. Magnetic resonance imaging (MRI) of the brain showed bilateral scleritis and optic perineuritis (left greater than right), with classic tram-track sign on axial view and donut sign on coronal view of the optic nerves. No other brain or cervical/thoracic lesions were seen. Serum antibodies to aquaporin 4, myelin oligodendrocyte glycoprotein, rheumatological work up and infectious studies were unremarkable. C-reactive protein and erythrocyte sedimentation rate were elevated. Routine cerebrospinal fluids findings were normal. The patient was treated with 2 g/kg of intravenous immunoglobulins (IVIG) followed by 30 mg/kg methylprednisolone intravenously (given insufficient response to IVIG), and oral steroid taper that is still ongoing (current dose: 12.5 mg/day). Methotrexate was started due to uveitis (25 mg once a week). Follow-up MRI of the brain one month after treatment initiation showed a significantly improved bilateral optic per neural enhancement and scleritis. Current VA is 20/30 bilaterally.
Bilateral optic perineuritis, scleritis and uveitis can occur in a single pediatric patient and can potentially represent a post-vaccinal phenomenon. Timely recognition and treatment is important for visual outcomes.