To determine baseline characteristics and outcomes in pediatric patients presenting with peri-optic neuritis (P-ON).

P-ON is a rare inflammatory disorder leading to inflammation of the nerve sheath. Most literature addresses adults, with little information on the pediatric population.

We conducted a single-center retrospective study of patients presenting to UPMC Children’s Hospital from 01/2017 – 03/2024. We used ICD-10 codes (optic neuritis (ON), optic papillitis, retrobulbar neuritis, other ON, unspecified ON) to identify potential patients with P-ON. For identified patients, we reviewed documented interpretations of MRI brain/orbit imaging by radiology. For patients with interpretations suspicious for P-ON, we reviewed their imaging with a neuroradiologist to confirm the diagnosis. For those patients, we collected baseline characteristics and outcomes.

We identified 20 cases of P-ON. The median age at diagnosis was 9 years, and 9 patients (45%) were female. Most patients presented with vision changes (75%), headaches (45%), and painful eye movements (40%). Initial ophthalmologic findings included disc edema (55%) and disc pallor (30%). The most common diagnoses (all with confirmed P-ON on imaging) were idiopathic ON (35%), MOG+ ON (35%), and inflammatory P-ON without ON (10%). No patients were diagnosed with MS or NMO. Of the 17 patients tested for MOGAD, 7 (41%) were positive. Eighteen (90%) patients initially received intravenous (IV) steroids, followed by oral steroids. Two (10%) patients had escalation of therapies to plasmapheresis or Rituximab. Four (20%) patients had documented relapses. Steroid therapy produced stable or improved visual acuity at follow-up (ranging from 3-10 months) in all patients (100%) with data present (n=16).

MOG+ status was common among patients with P-ON, along with ON cases. Regarding visual outcomes, all patients either had stable or improved visual acuity at follow-up, showing that the regimen of IV to oral steroids may be a good treatment option in the pediatric population.