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Figure 1. Ultra-widefield fundus photograph showing temporal hypopigmented retinal lesion in the left eye.
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Figure 2. Spectral domain optical coherence tomography of the left eye demonstrating temporal outer retinal and choroidal atrophy.
A 10-year-old male patient was referred for retinal evaluation after failing vision screening at school. The patient endorsed blurry vision and headaches. Past medical and family history were unremarkable.
On examination, his Snellen visual acuity was 20/30 in both eyes. His anterior segment examination was normal. Dilated fundus exam of the right eye was unremarkable. Left fundus exam showed a temporal hypopigmented retinal lesion.
Optical coherence tomography (OCT) of the left eye revealed temporal outer retinal and choroidal atrophy.
First described by Roseman and Gass in 1992, torpedo maculopathy, also known as solitary hypopigmented nevus of the retinal pigment epithelium (RPE), is a rare retinal condition of unknown etiology. Several theories propose the pathogenesis of torpedo maculopathy, including a...
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First described by Roseman and Gass in 1992, torpedo maculopathy, also known as solitary hypopigmented nevus of the retinal pigment epithelium (RPE), is a rare retinal condition of unknown etiology. Several theories propose the pathogenesis of torpedo maculopathy, including a developmental deficiency in the nerve fiber layer, abnormal choroidal circulation, persistent developmental defects in the RPE, and intrauterine chorioretinitis.1-4
Clinically, torpedo maculopathy is typically characterized by a solitary, unilateral, hypopigmented, torpedo-shaped lesion located temporal to the fovea.3 Fundus autofluorescence shows hyperfluorescent border surrounding the hypofluorescent area of atrophic RPE.5 Optical coherence tomography (OCT) is the primary diagnostic tool revealing attenuation of outer retinal structures and increased signal transmission along the choroid.5 OCT angiography is characterized by decreased vessel density in the deep retinal capillary plexus with diffuse attenuation of the choriocapillaris. This corresponds to decreased retinal sensitivity as measured by microperimetry.6
Torpedo maculopathy is a benign nonprogressive retinal condition that typically remains stable over time. Therefore, management includes observation and possible treatment with intravitreal injection of anti-vascular endothelial growth factor in the rare case of development of choroidal neovascular membrane.7
Dr Hoyek is a postdoctoral research fellow in Ophthalmology at Massachusetts Eye and Ear and Harvard Medical School, Boston
Dr Patel is an Assistant Professor of Ophthalmology at Massachusetts Eye and Ear and Harvard Medical School and Director of Pediatric Retina at Boston Children’s Hospital, Boston
References
1. Pian D, Ferrucci S, Anderson SF, Wu C. Paramacular coloboma. Optom Vis Sci. 2003;80(8):556-563. doi:10.1097/00006324-200308000-00008
2. Teitelbaum BA, Hachey DL, Messner LV. Torpedo maculopathy. J Am Optom Assoc. 1997;68(6):373-376.
3. Shields CL, Guzman JM, Shapiro MJ, Fogel LE, Shields JA. Torpedo maculopathy at the site of the fetal “bulge.” Arch Ophthalmol. 2010;128(4):499-501. doi:10.1001/archophthalmol.2010.29
4. de Manuel-Triantafilo S, Gili P, Bañuelos Bañuelos J. Torpedo maculopathy: Two case reports and a literature review. Arch Soc Esp Oftalmol. 2016;91(8):400-403. doi:10.1016/j.oftal.2016.01.028
5. Thomas AS, Flaxel CJ, Pennesi ME. Spectral-domain optical coherence tomography and fundus autofluorescence evaluation of torpedo maculopathy. J Pediatr Ophthalmol Strabismus. 2015;52 Online:e8-10. doi:10.3928/01913913-20150303-01
6. Grimaldi G, Scupola A, Sammarco MG, Marullo M, Blasi MA. Morpho-functional evaluation of torpedo maculopathy with optical coherence tomography angiography and microperimetry. Am J Ophthalmol Case Rep. 2018;10:165-168. doi:10.1016/j.ajoc.2018.02.019
7. Shirley K, O’Neill M, Gamble R, Ramsey A, McLoone E. Torpedo maculopathy: disease spectrum and associated choroidal neovascularisation in a paediatric population. Eye (Lond). 2018;32(8):1315-1320. doi:10.1038/s41433-018-0074-7
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