Torpedo Maculopathy Associated with Choroidal Neovascularization

Abstract

Torpedo maculopathy is a very rare, congenital, usually unilateral hypopigmented lesion in the temporal macula. This retrospective case series describes three patients with torpedo maculopathy. The first two cases demonstrate typical clinical and imaging findings of torpedo maculopathy in asymptomatic patients. The third case relates to a symptomatic young patient with a torpedo lesion, a smaller satellite lesion, and evidence of choroidal neovascularization confirmed by fluorescence angiography. In the area of the clinically visible torpedo lesion, spectral domain optical coherence tomography showed atrophy of the outer retina with increased choroidal signalling and a hyperreflective lesion above the retinal pigment epithelium suggestive of choroidal neovascularization. Fundus autofluorescence imaging revealed a hyperautofluorescent rim along the margin of the hypoautofluorescent torpedo lesion. In the literature, torpedo lesions are usually regarded as benign lesions with no tendency for progression. The third case demonstrates that torpedo lesions may be associated with choroidal neovascularization, which has been successfully treated with anti-VEGF therapy.

Torpedo maculopathy: disease spectrum and associated choroidal neovascularisation in a paediatric population

“…All satellite lesions to date are located temporally and are small, round and flat as seen in patient 5. OCT imaging of these lesions has previously been described as normal however OCT of the satellite lesion in our patient reveals similar changes to the main TM lesion with normal inner retina but localised broadening and attenuation of the interdigitation zone resulting in elevation of the external limiting membrane and ellipsoid zone [6] (Fig. 3).…”

“…1). Reviewing previously documented cases in the literature we can identify 3 other clinical cases that have similar presentations [6,10]. All satellite lesions to date are located temporally and are small, round and flat as seen in patient 5.…”

“…As previously mentioned, TM is felt to be an asymptomatic, benign condition and many patients are discharged from routine follow-up. However, a recent case of a 34year-old patient with CNV related to TM was published by a group in Switzerland [6]. It was only described once prior to this in Gass' Atlas of macular diseases [5].…”

“…Generally, TM is felt to be a non-progressive lesion, although occasionally the size of the lesion has shown documented increase [4]. To the authors knowledge, there have only been two reports of choroidal neovascularization (CNV) associated with TM, described in 2011 by Gass and in 2017 by Jurjevic [5,6]. The aim of this case series is to demonstrate the spectrum of clinical appearance, the patient demographics, coinciding systemic conditions and variable optical coherence tomography (OCT) appearance in a paediatric population.…”

Torpedo Maculopathy

“…[1] Rarely, it might be associated with the formation of choroidal neovascular membrane. [6] In conclusion, in torpedo maculopathy, the characteristic OCT-A finding is vascular alterations in the choriocapillaris layer, which supports the hypothesis that the choroid is the first structure to be involved in this condition. Further studies might be needed to provide deeper insights into the pathophysiology of torpedo maculopathy.…”