Surgical management of a combined hamartoma of the retina and retinal pigment epithelium

A 4-year-old boy was referred with visual acuity of 20/400 in his right eye. Examination revealed tortuous vasculature, retinal thickening with macular fibrosis, and underlying retinal pigment epithelial changes. Over time, the patient developed dense vitreal foveal traction causing the development of significant tractional subretinal fluid (Figs. A and B). A 3-port 23-gauge pars plana vitrectomy with removal of the posterior hyaloid, peeling of the internal limiting membrane toward the intraretinal fibrosis, and insertion of 17% perfluoropropane gas was performed. Following surgery, vision improved to 20/150, and the subretinal fluid had completely resolved (Figs. C and D). Fig. 1

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