Posterior microphthalmos with pigmentary retinopathy

We report a case of 19-year-old man with gradual diminution of vision in both eyes since childhood. His best-corrected visual acuity was 20/160, N16 in the right eye and 20/200, N16 in the left eye. Slit-lamp biomicroscopic examination revealed normal cornea, anterior segment, intraocular pressure and lens. Fundus of both eyes showed crowded optic disc with pigmentary changes. Ancillary tests were performed to aid in the diagnosis. A-scan ultrasound revealed short axial lengths with normal corneal diameter, anterior chamber depth and lens thickness. Optical coherence tomography of both eyes showed inner retinal layer folds. Electroretinogram of both eyes showed extinguished photopic as well as scoptopic responses. A diagnosis of posterior microphthalmos with pigmentary retinopathy was made. The patient was counselled regarding nature of the disease and the condition was managed with low vision aids.