The Figure demonstrates the evolution from relatively mild telangiectatic vascular changes over the superior optic nerve in a youth to a large, typical combined hamartoma of the optic nerve 5 years later.1,2 Over this period, the visual acuity declined from 20/40 OS to 7/200 OS. Shields et al3 reported their experience on 77 cases of combined hamartoma of retina and retinal pigment epithelium and noted that progressive vision loss occurs in only 13% of extramacular tumors, as this case was initially according to their definition. In contrast, if the macula is involved, progressive vision decline was noted in 60% of cases.
McCannel CA, Mohney BG. Evolution of a Peripapillary Combined Hamartoma of the Retina and Retinal Pigment Epithelium in a Child. JAMA Ophthalmol. 2020;138(6):e192670. doi:10.1001/jamaophthalmol.2019.2670
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