Presumed bilateral acute idiopathic maculopathy in a pediatric patient

Purpose: To describe a rare case of presumed bilateral acute idiopathic maculopathy (AIM) in a pediatric patient.

Observation: An 11-year-old male was evaluated for a "fuzzy Dorito-shaped" spot in the central vision of his right eye (OD) that started 3 days before presenting to our clinic. On examination, best-corrected visual acuity (BCVA) was counting fingers at 5 feet OD, and 20/25 in the left eye (OS). Fundus examination demonstrated a central macular lesion with pigmentary changes OD and mild retinal pigment epithelium (RPE) changes OS. Optical coherence tomography (OCT) imaging showed elevated, irregular RPE with overlying subretinal fluid in the fovea OD and trace ellipsoid zone changes temporal to the fovea OS. Uveitis work-up was unremarkable for any infectious or inflammatory etiologies. Given the severe vision loss in the right eye and the negative infectious work-up, the decision was made to initiate oral corticosteroids in agreement with the patient's family. One week following the initial presentation, the patient developed visual symptoms OS without much improvement OD. The patient was admitted to the hospital, and further work-up revealed elevated coxsackie B3 antibody titers (1:20). Given the characteristic findings on multimodal imaging and elevated coxsackie B3 antibodies, the patient was presumptively diagnosed with bilateral AIM. Over the following 4 months, he would intermittently complain of nonspecific visual complaints such as blurry vision and floaters requiring an increase in corticosteroid dose and addition of immunomodulatory therapy, although the clinical examination and OCT findings remained unchanged. At the patient's follow-up visit 8 months after the initial visit, BCVA was stable at 20/40 OD and 20/30 OS, and OCT demonstrated stable foveal lesions in both eyes.

Conclusion: This case report describes a rare presentation of presumed bilateral AIM in a pediatric patient. This prompts further consideration of this condition in pediatric patients, especially after a prodromal flu-like illness.