Posterior Microphthalmos With RPE Dysfunction Misdiagnosed as Macular Edema

Posterior microphthalmos (PM) typically manifests as retinal thickening, edema, and the presence of papillomacular fold (PMF). We report a case of bilateral PM with macular atrophy and RPE dysfunction. Late-stage of RPE dysfunction in PM is poorly documented in the literature. We present a case that highlights this scenario, aiming to raise awareness of this condition among ophthalmologists.

The authors emphasize the atypical imaging findings associated with PM and RPE dysfunction. This case was initially misdiagnosed as macular edema, leading to the administration of anti-VEGF treatment, a common clinical pitfall in PM due to intraretinal cystoid changes. Nevertheless, no improvement was observed in retinal thickness, as evidenced by OCT examination, or inpatient visual acuity following anti-VEGF therapy.

Our case adds to recently reported cases linking PM and RPE dysfunction. The recognition of the short axial length and the utilization of imaging modalities such as OCT and FFA contribute to the diagnosis of this condition. It is important to note that anti-VEGF therapy is not applicable for this condition.