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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 458-460

Torpedo maculopathy with bilateral familial fleck retina: An unusual coexistence


1 Department of Ophthalmology, Netradhama Super Speciality Eye Hospital, Bengaluru, Karnataka, India
2 Department of Ophthalmology, Netradhama Super Speciality Eye Hospital, Bengaluru, Karnataka; Consultant, Cataract and VR Surgeon, Dr Agarwals Eye Hospital, Rajahmundry, Andhra Pradesh, India
3 Department of Ophthalmology, G. S. Medical College, Hapur, Uttar Pradesh, India

Date of Submission10-Jun-2021
Date of Acceptance15-Sep-2021
Date of Web Publication13-Apr-2022

Correspondence Address:
Rahul Bhargava
Department of Ophthalmology, G. S. Medical College, Hapur - 245 304, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1610_21

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  Abstract 


This report describes a patient with redness in the left eye and normal vision. Routine fundus examination revealed bilateral macular sparing multiple yellow white retinal flecks situated beneath the retinal vessels. In the right eye, a torpedo-shaped hypopigmented lesion with a hyperpigmented temporal edge was observed temporal to the macula. There was mild thickening of retinal pigment epithelium on spectral-domain optical coherence tomography and retinal thinning and atrophy. Fundus autofluorescence showed multiple hyperfluorescent lesions around the posterior pole with a normal signal and a torpedo lesion of variegated autofluorescence in the right eye. The unusual coexistence of unilateral torpedo maculopathy with bilateral retinal flecks is being reported here.

Keywords: Familial fleck retina, optical coherence tomography, torpedo maculopathy


How to cite this article:
Kumar M, Singh K, Ravichandra G, Patel A, Kapoor M, Bhargava R. Torpedo maculopathy with bilateral familial fleck retina: An unusual coexistence. Indian J Ophthalmol Case Rep 2022;2:458-60

How to cite this URL:
Kumar M, Singh K, Ravichandra G, Patel A, Kapoor M, Bhargava R. Torpedo maculopathy with bilateral familial fleck retina: An unusual coexistence. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 May 24];2:458-60. Available from: https://www.ijoreports.in/text.asp?2022/2/2/458/342892



Benign familial fleck retina (BFFR) is characterized by postequatorial multifocal yellowish infiltrates at level of retinal pigment epithelium (RPE). Torpedo maculopathy is a rare, congenital, nonprogressive, oval-shaped, hypopigmented lesion, usually located temporal to the macula. We report a rare case of the unusual occurrence of torpedo retinopathy in a patient with bilateral fleck retina. To the best of our knowledge, this unusual occurrence has not been previously reported in literature.


  Case Report Top


We report a case of 31-year-old male who presented with occasional redness in the left eye. There was no history of nyctalopia. The best-corrected visual acuity was 6/6 Snellen in both eyes. A dilated fundus examination revealed concentric, multiple, subretinal yellowish-white flecks distributed throughout the retina sparing disc and macula in right and left eyes [Figure 1]a and [Figure 1]b. In the right eye, there was a torpedo-shaped mildly hypopigmented lesion temporal to macula. This torpedo-shaped lesion was a mix of hypo and hyperpigmentation, the size of which was about 0.5-disc diameter (DD) vertically and around 2.5 DD horizontally.
Figure 1: Fundus picture showing bilateral yellowish flecks involving the postequatorial retina but sparing macula and torpedo-shaped lesion in right eye

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The flecks involved the equatorial, peripheral, and far peripheral areas of the retina. The flecks had a variable shape, but the centrally located ones had a round to oblong shape and were approximately of the diameter of the third-order arteriole. Peripherally located flecks were much larger and had a tendency toward confluence. The more peripheral flecks showed great variability in shape with linear, geographical or amoeboid outlines, and smooth sinuous margins. The lesions were flat.

Color vision tests with Ishihara plates and the Farnsworth dichotomous test was normal in each eye. Infrared fundal images showed visible lesion contour, transverse elliptical shape, and a tip pointing toward the fovea. Fundus autofluorescence revealed normal signal with slight hyper autofluorescence at the nasal lesion margin. Benign flecks appear hyperautofluorescent in their distribution zone [Figure 2]. The spectral-domain optical coherence tomography showed a normal inner retina, with mild thinner outer retina and RPE in the temporal macular area, and correspondingly increased choroidal reflectivity [Figure 3]a and [Figure 3]b. Other optical coherence tomography (OCT) findings included outer retinal loss/attenuation with significant atrophy of an intact ellipsoid zone. Multifocal ERG showing subnormal response in both eyes with slightly greater reduction in the right eye corresponding to the Torpedo lesion is shown in [Figure 4].
Figure 2: Fundus autoflorecence showing variegated pattern of hyper and hypoautofluorescence temporal to fovea in the right eye and symmetrical pattern of yellowish white fleck lesions in the left eye

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Figure 3: Optical coherence tomography (SD-OCT) in the right eye showing a normal inner retina, mild thinning of RPE, and outer retina with loss of IS-OS junction. There is an ellipsoid zone with correspondingly increased choroidal reflectivity with endogenously hyperreflective lesions at the level of retinal pigment epithelium corresponding to retinal flecks

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Figure 4: Multifocal ERG showing subnormal response in both eyes with slightly greater reduction in the right eye corresponding to the Torpedo lesion

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  Discussion Top


Krill and Klien introduced the term “flecked retina” to describe multiple yellowish-white fundal lesions of various size and configuration, without vascular lesion or optic nerve abnormality.[1] BFFR is an uncommon condition reported sporadically since the first case series reported by Aish and Dajani in 1980.[2],[3],[4] It is an autosomal recessive condition that presents with diffuse yellow-white fleck lesions extending into the far-periphery; however, these lesions spare the macular area. Patients usually do not complain of nyctalopia. Visual acuity and color vision are typically normal. Neriyanuri et al.[5] described multimodal imaging in four patients with benign fleck retina. The authors found that all patients were visually asymptomatic and flecks had a peripheral distribution and were hyperautofluorescence. In line with our case extent of lesions varied from RPE, inner segment to outer segment extending till external limiting membrane on OCT. A similar lesion was described by Garcia et al.[6] on multimodal imaging in a 27-year-old female.

Torpedo maculopathy refers to a congenital, unilateral, hypopigmented lesion of the RPE in the temporal macula; often presenting as a chance finding, visual acuity is usually preserved despite reports of this lesion causing scotoma and progression on OCT. The exact etiology of torpedo maculopathy remains speculative, but most reported cases and case series mention a consistent temporal location.[7],[8],[9] Among the various proposed causes, a persistent defect in the development of RPE in the fetal temporal bulge seems to be the most accepted of all.[10]

Spectral domain OCT has delineated two types of lesions: attenuation of the outer nuclear layer, interdigitation zone, and ellipsoid zones in the absence of any cavitation of the outer layers (Type 1) and an outer retinal layer attenuation along with cavitation (Type II).[11] Our case resembles Type I due to the absence of a cavity above the RPE [Figure 3]a and [Figure 3]b.

Venkatesh et al.[12] retrospectively reviewed database of nine patients with torpedo-shaped lesions and found that there was macular involvement in six patients; all had a nasally pointed tip directed toward the posterior pole and a temporal rounded margin. There were varying degrees of hyperpigmented RPE changes around the lesions. On OCT, inner retina was normal in all but one eye. All eyes had thinning of the outer nuclear layer, interdigitation zone, and ellipsoid zone. In contrast to our case, subretinal cavitation was observed in two cases.

In another retrospective observational case series in 13 patients with torpedo-shaped lesions, Golchet et al. found an abnormal RPE with thinning and a negative scotoma corresponding to the lesion.[7]


  Conclusion Top


BFFR spares the macula and region around optic nerve and is often detected in routine fundus examination. Torpedo maculopathy on the other hand may or may not be accompanied by subretinal cavitation. The simultaneous occurrence of the two lesions is rare and has not been reported in literature.

Acknowledgements

We are thankful to <www.indianmedicalstats.com> for technical help.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Krill AE, Klien BA. Flecked retina syndrome. Arch Ophthalmol 1965;74:496-508.  Back to cited text no. 1
    
2.
Aish SF, Dajani B. Benign familial fleck retina. Br J Ophthalmol 1980;64:652-59.  Back to cited text no. 2
    
3.
Galindo-Ferreiro A, Sanabria MR, Garcia EP, Coco-Martin RM, Galindo-Alonso J, Palencia-Ercilla J. Benign fleck retinal findings on multifocal ERG, microperimetry, and OCT. Ophthalmic Surg Lasers Imaging 2010;41:e1-5.  Back to cited text no. 3
    
4.
Isaacs TW, McAllister IL, Wade MS. Benign fleck retina. Br J Ophthalmol 1996;80:267-8.  Back to cited text no. 4
    
5.
Neriyanuri S, Rao C, Raman R. Structural and functional characterization of benign fleck retina using multimodal imaging. Retin Cases Brief Rep 2017;11:186-190.  Back to cited text no. 5
    
6.
Garcia JMBB, Isaac DLC, Sardeiro T, Aquino É, Avila M. Benign familial fleck retina: Multimodal imaging including optical coherence tomography angiography. Arq Bras Oftalmol 2017;80:321-3.  Back to cited text no. 6
    
7.
Golchet PR, Jampol LM, Mathura JR Jr, Daily MJ. Torpedo maculopathy. Br J Ophthalmol 2010;94:302-6.  Back to cited text no. 7
    
8.
Shields CL, Guzman JM, Shapiro MJ, Fogel LE, Shields JA. Torpedo maculopathy at the site of the fetal “bulge” Arch Ophthalmol 2010;128:499-501.  Back to cited text no. 8
    
9.
Hamm C, Shechtman D, Reynolds S. A deeper look at torpedo maculopathy. Clin Exp Optom 2017;100:563-8.  Back to cited text no. 9
    
10.
Shields CL, Guzman JM, Shapiro MJ, Fogel LE, Shields JA. Torpedo maculopathy at the site of the fetal “bulge”. Arch Ophthalmol 2010;128:499-501.  Back to cited text no. 10
    
11.
Wong EN, Fraser-Bell S, Hunyor AP, Chen FK. Novel optical coherence tomography classification of torpedo maculopathy. Clin Exp Ophthalmol 2015;43:342-8.  Back to cited text no. 11
    
12.
Venkatesh R, Jain K, Pereira A, Thirumalesh, Yadav NK. Torpedo retinopathy. J Ophthalmic Vis Res 2020;15:187-94.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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