Kikuchi-Fujimoto disease associated optic neuropathy in an adolescent

We present a case of Kikuchi-Fujimoto Disease (KFD) associated with bilateral optic neuropathy progressing to vision loss.

A 17-year-old male was referred for bilateral optic nerve pallor. Eight years prior, he was diagnosed with KFD after workup for lymphadenopathy and treated with prednisolone acutely followed by long-term Plaquenil. He had reduced vision shortly after the diagnosis of KFD. Visual acuity was 20/50 OD and 20/250 OS, with pinhole to 20/40 OD and 20/70 OS. Comprehensive laboratory workup for infectious and autoimmune etiologies, including systemic lupus erythematosus (SLE), were negative. Magnetic resonance imaging (MRI) showed bilateral optic nerve atrophy and atrophy of the optic chiasm. The patient trialed B-complex vitamins with no improvement and continued to worsen with acute decrease in vision to no light perception OD at three-year follow-up. Treatment remained supportive.

This is a case of bilateral KFD-associated optic neuropathy in an adolescent. Unlike previously reported cases, the patient experienced acute vision loss rather than full recovery of visual acuity after treatment. Ophthalmologists should be aware of the rare but serious association of optic neuritis and KFD.