A 12-year-old girl presented with esotropia and defective vision in the left eye. Her right eye was normal. Her visual acuity was 20/200 OS, and the anterior segment was normal. A fundus examination showed corkscrewing of vessels, dragging of arcades with a subretinal yellowish lesion in the posterior pole, an adherent epiretinal membrane, and preretinal gliosis overlying the lesion (Figure). Optical coherence tomography showed obscured, disorganized inner retinal layers, with high reflectivity and shadowing of the underlying layers. Fundus fluorescein angiography (Figure) ruled out the possibility of an intraocular malignant condition or posterior uveitis. A systemic examination had normal results; it did not reveal any features of phacomatoses. A diagnosis of combined hamartoma of retina and retinal pigment epithelium was made based on these findings.1-3 Since the child had already developed suppression and there was gross distortion of macular anatomy and the surrounding retina, surgery was deferred,4 and the poor prognosis for visual acuity in the left eye was clearly explained to the parents.
M A, Mohan R, Gondhale HP. Tangled Vasculature in the Eye of a Young Girl. JAMA Ophthalmol. 2020;138(11):e202045. doi:10.1001/jamaophthalmol.2020.2045
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