Severe retinal vasculopathy associated with myelin oligodendrocyte glycoprotein antibody optic neuritis in a toddler

Abstract

We report the case of a 22-month-old boy with retinal vascular disease caused by optic neuritis due to myelin oligodendrocyte glycoprotein antibody disease. He presented with acute disseminated encephalomyelitis without optic nerve involvement. Despite treatment, he developed optic neuritis and vision loss within days. In addition to the usual findings of optic neuritis, he had evidence of severe retinal venous stasis including intraretinal, subretinal, preretinal, and choroidal hemorrhages that were rapidly progressive. The retinal perfusion did not recover, leading to neovascularization. This is a rare case of MOG antibody-associated disease with retinal venous stasis in which long-term follow-up revealed permanent vision loss from widespread retinal ischemia and optic atrophy. We propose that hemorrhages beyond the peripapillary region and macula can be a sign of retinal venous insufficiency related to vascular obstruction at the optic nerve head. Patients exhibiting this sign should undergo prompt antibody testing when it is associated with optic neuritis. Careful monitoring and treatment of retinal ischemia and neovascularization in severe cases is warranted to prevent late complications.