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.

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

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.