Pigmented Macular Lesion in a Child

A 5-year-old boy with a history of an Escherichia coli infection complicated by hemolytic uremic syndrome presented with a visual acuity of 20/125 OD and 20/20 OS (HOTV letters). He had no history of hearing loss or pigmented skin lesions. He had no past ocular history or prior ophthalmic examinations. Color vision was severely diminished in the right eye, as determined by Ishihara color plates, and he had no detectable stereoacuity, as determined by Titmus testing. Intraocular pressure and extraocular movements were normal. He had a mild relative afferent pupillary defect in the right eye but no strabismus or nystagmus. The cycloplegic refraction was +2.50 + 0.50 × 100 OD and +2.00 sphere OS. Results of an anterior segment slitlamp biomicroscopy examination showed no abnormalities. Results of a dilated retinal examination showed a large, elevated, homogenous, gray mass in the macula of the right eye with temporal pallor of the optic disc (Figure, A). Optical coherence tomography demonstrated gliosis with a tractional epiretinal membrane and modest disorganization of the retinal layers, producing a sawtooth configuration within the outer plexiform layer (Figure, B).