Abstract
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Background: Torpedo maculopathy is an uncommon benign congenital macular abnormality characterized by a characteristic torpedo-shaped temporal hypopigmented lesion with its tip directed toward the fovea. Persistent peripheral avascular retina (PAR), in contrast, represents incomplete retinal vascularization that may persist following retinopathy of prematurity (ROP). We report an unusual case of torpedo maculopathy with associated subretinal fluid coexisting with extensive bilateral persistent peripheral avascular retina in child with regressed ROP.
Case Presentation: A 7-year-old Chinese girl, born at 28 weeks of gestation, presented with reduced vision in the right eye of uncertain duration. She had previously been followed for ROP but was subsequently lost to ophthalmic follow-up during the COVID-19 movement restrictions. There was no history of laser photocoagulation or intravitreal treatment.
Best-corrected visual acuity was 6/18 in the right eye and 6/6 in the left eye with right exotropia. Fundus examination of the right eye revealed a solitary, flat, well-demarcated hypopigmented torpedo-shaped lesion with a hyperpigmented border in the temporal macula, with its pointed tip directed toward the fovea. Bilateral temporal peripheral avascular retina extending from anterior zone II was also present, without neovascularization.
Multimodal imaging revealed characteristic outer retinal abnormalities involving the interdigitation zone with associated subretinal fluid on optical coherence tomography. Fundus autofluorescence demonstrated corresponding abnormal autofluorescence. Optical coherence tomography angiography showed no evidence of choroidal neovascularization. Fundus fluorescein angiography demonstrated early hyper fluorescence of macular lesion without progressive leakage, consistent with a window defect, while bilateral extensive temporal peripheral capillary non-perfusion was demonstrated without leakage or neovascularization.
A diagnosis of right torpedo maculopathy associated with bilateral persistent peripheral avascular retina secondary to regressed ROP was made. Bilateral laser photocoagulation to the persistent avascular peripheral retina was subsequently performed. The patient remains under close ophthalmic surveillance.
Conclusion: This case highlights the value of multimodal retinal imaging in distinguishing torpedo maculopathy from other macular lesions and demonstrates the unusual coexistence of torpedo maculopathy and persistent avascular retina in a child with previous ROP. Continued long-term surveillance of children with a history of ROP, particularly those with interrupted follow-up, is important for identifying persistent retinal vascular abnormalities may persist and potential late complications.
Keywords: torpedo maculopathy; retinopathy of prematurity; persistent avascular retina; prematurity; subretinal fluid; optical coherence tomography; fluorescein angiography; pediatric retina
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