October 13, 2021
Authored by Burton Wisotsky, MD
A 6 year old boy was referred for reduced vision, an eye turn, and a retinal finding. Medical history is unremarkable. Ocular history is significant for right esotropia first noted about a year ago. It was initially intermittent and then became permanent. He was noted to have a retinal abnormality and was referred for evaluation. There is no history of patching. On examination, VA was OD: 20/150 and OS: 20/20. The anterior segments were normal. A constant RET was present. Retinoscopy was +3.00 sphere OU. Refraction did not improve the vision OD. DFE is noted below:
The left eye is normal. There is a prominent ILM, which is normal for a young child. In the right eye there is wrinkling of the posterior retina and retinal vessels with macular hyperpigmentation and optic nerve atrophy. Our patient was diagnosed with a combined hamartoma of the retina and the RPE. This is a rare congenital lesion. It usually affects the vision to a moderate degree although it does not significantly progress as the child ages. It is untreatable. Vitrectomy with membrane peeling usually does not improve the retinal status. We sent the patient to Dr. Napolitano for strabismus evaluation. He prescribed the full hyperopic correction with 8 hours per day patching of the left eye. Within a month the mother noted an improvement in the alignment. Visual acuity OD improved to 20/40! It will likely not improve beyond that level, but this case illustrates the importance of accurate retinal diagnosis and prompt amblyopia treatment in children.