Multifocal Torpedo Maculopathy Complicated by Choroidal Neovascularization

Abstract

Purpose: To present a pediatric patient with a unique configuration of torpedo maculopathy complicated by macular choroidal neovascularization (CNV). Methods: A single case was retrospectively reviewed. Results: An 8-year-old male child presented with decreased vision in the left eye and was found to have 2 distinct torpedo maculopathy lesions, 1 a smaller hypopigmented lesion in the temporal parafovea and the other a larger hyperpigmented comet-shaped lesion in the temporal periphery. Multimodal imaging showed active CNV. The patient received 2 intravitreal injections of ranibizumab with regression of CNV and recovery of visual acuity. Conclusions: CNV is a rare complication of torpedo maculopathy that can affect pediatric patients in the absence of choroidal excavation. The presence of a hyperpigmented peripheral lesion exhibiting symmetry across the horizontal raphe lends support to the hypothesis that an alteration in the development and migration of retinal pigment epithelium cells across the fetal bulge results in this disorder.

Keywords: anti-VEGF agents; choroidal neovascularization; pediatric retina; retinal pigment epithelium; torpedo maculopathy.

Figure 1.

(A) Pseudocolor widefield photograph of the left eye at presentation shows a subfoveal pigmented scar with surrounding subretinal fluid (SRF), a temporal parafoveal oval hypopigmented lesion, and a larger hyperpigmented comet-shaped lesion in the temporal periphery. (B) Late-phase fluorescein angiography shows leakage from the subfoveal lesion, staining of the temporal parafoveal lesion, and blockage from the temporal peripheral lesion. (C–D) Optical coherence tomography (OCT) of the macula shows subfoveal hyperreflective material with adjacent SRF as well as ellipsoid zone attenuation in the area of the hypopigmented lesion. (E) OCT of the temporal peripheral lesion shows outer retinal loss and choroidal hypertransmission.

Figure 2.

(A) Optical coherence tomography (OCT) of the left macula 1 month after presentation shows decreased subretinal hyperreflective material and subretinal fluid (SRF). (B) Pseudocolor widefield photograph and (C) OCT of the left eye 7 months after presentation shows regression of the choroidal neovascularization, resolution of the SRF, and a stable appearance of torpedo maculopathy lesions.