Presentation
Questionable light perception in the left eye
Patient Data
- left retinal detachment with subretinal fluid that is T1 hyperintense, T2 hypointense, and non-suppressing on FLAIR compared to normal vitreous
- smaller left eye size compared to the right
- intraretinal macrocyst at the temporal aspect (best seen on high resolution T2 SPACE)
- T2 hypointense, nonenhancing nodule in the superotemporal aspect
- no lens deformity, ciliary body deformity, central stalk under the retinal detachment, or optic nerve atrophy
Case Discussion
When faced with a retinal detachment in a young child, the differential diagnosis includes retinoblastoma and its two most common mimics: persistent fetal vasculature/retinal dysplasia and Coats disease. In this case, findings that weigh against retinoblastoma are the smaller size of the affected eye and the lack of significant enhancement in the solid lesion. Negative findings that weigh against persistent fetal vasculature include the lack of deformation of the lens and ciliary body, central stalk, or optic nerve atrophy. Finally, the intraretinal macrocyst has been described as a specific finding in Coats disease 1.
This patient subsequently underwent dilated fundoscopy under anesthesia. There was total retinal detachment. The blood vessels showed telangiectatic changes with aneurysms, as well as pigmentary and frost-like changes of peripheral branches.
Ocular ultrasound (B-scan) showed the total retinal detachment as well. The subretinal fluid had tiny echoes that may reflect exudation. The dome-shaped lesion in the superotemporal quadrant was found but did not appear calcified.
Fluorescein angiogram showed the telangiectatic and aneurysmal vessels in the peripheral retina, as well as evidence of far peripheral ischemia.
The retinal vascular appearance was considered characteristic of Coats disease.
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