|Year : 2022 | Volume
| Issue : 3 | Page : 725-727
Double torpedo maculopathy in an adolescent patient
Farhan Hussain, Venkatkrish M Kasetty, Abdualrahman Hamad
Department of Ophthalmology, Henry Ford Health System, Detroit, Michigan, USA
|Date of Submission||08-Dec-2021|
|Date of Acceptance||15-Apr-2022|
|Date of Web Publication||16-Jul-2022|
Dr. Venkatkrish M Kasetty
2799 W Grand Blvd, Detroit - 48202, Michigan
Source of Support: None, Conflict of Interest: None
Torpedo maculopathy is a rare, typically singular, asymptomatic, unilateral torpedo-shaped lesion located in the temporal macula. The pathophysiology of these lesions is currently unknown but is thought to occur during embryogenesis. Even rarer are cases of torpedo maculopathy with two lesions in the affected eye, with only one prior case identified in the literature. The authors present a case of double torpedo maculopathy with accompanying optical coherence tomography, fundus photos, and fundus auto-fluorescence imaging over a 1.5-year follow-up period. Although our case further establishes the non-progressive nature of this phenotype, the exact pathophysiology warrants further investigation.
Keywords: Embryogenesis, macula, torpedo maculopathy
|How to cite this article:|
Hussain F, Kasetty VM, Hamad A. Double torpedo maculopathy in an adolescent patient. Indian J Ophthalmol Case Rep 2022;2:725-7
Torpedo maculopathy is a rare condition characterized by congenital hypo-pigmented lesions located in the temporal macula first described by Roseman and Gass in 1992., These lesions are typically unilateral, horizontal torpedo-shaped lesions with the leading edge pointing toward the fovea. The etiology of these lesions is not fully understood, but because of their stability and non-progressive nature, they have been theorized to occur during fetal retinal pigment epithelium (RPE) development. Although the majority of these lesions are solitary, Raju et al. describe a pediatric patient who presented with two lesions consistent with torpedo maculopathy in the same eye without follow-up data. Herein, the authors describe a case of double torpedo maculopathy observed in an asymptomatic adolescent eye with a stable 1.5 year follow-up. The collection of patient health information was compliant with the Health Insurance Portability and Accountability Act, and this report adheres to the tenets of the Declaration of Helsinki.
| Case Report|| |
A 15-year-old female with past medical history significant for migraine headaches presented to an ophthalmology clinic through optometry referral for evaluation of retinal lesions in her right eye. The patient had no prior ophthalmic surgical history or no known family history of glaucoma, macular degeneration, or gastro-intestinal malignancies. A complete ophthalmologic exam revealed a visual acuity of 20/20 and an intra-ocular pressure (IOP) of 18 mmHg in both the eyes. Confrontational visual fields were full, and no afferent pupillary defect was observed. Examination of the left eye was within normal limits. Fundus examination of the right eye showed two flat, hypo-pigmented, torpedo-shaped lesions. The first lesion was located in the infero-temporal macula adjacent to the fovea, and the second irregularly shaped lesion was located about 0.5 disc diameters infero-temporal to the first lesion [Figure 1]a. Otherwise, examination of right eye was within normal limits. Optical coherence tomography (OCT) revealed similar mild retinal pigment epithelium (RPE) atrophy and outer retinal disruption of both lesions [Figure 2]a. Auto-fluorescence did reveal that the second more peripheral lesion exhibited a more stippled appearance with the hyper auto-fluorescent ring not seen in the first lesion. A diagnosis of double torpedo maculopathy was made, and the patient was to continue with routine follow-up. At follow-up examination 1.5 years later, the patient had no changes to her vision. The visual acuity was 20/20 in the right eye and 20/25 in the left eye with normal IOP. Complete ophthalmic examination and OCT macula remained unchanged from the initial examination [Figure 1]b and [Figure 2]b. Fundus auto-fluorescence (FAF) showed hypo-auto-fluorescence in the lesions with a hyper-auto-fluorescent rim as described above [Figure 3].
|Figure 1: (a) Fundus photography of the right eye demonstrating double torpedo maculopathy at the initial presentation. (b) Fundus photography of the right eye demonstrating double torpedo maculopathy at 1.5-year follow-up|
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|Figure 2: (a) OCT macula demonstrating mild RPE atrophy and outer retinal disruption (black arrows) at the initial presentation. (b) Stable OCT macula at 1.5-year follow-up|
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|Figure 3: FAF demonstrating hypo-auto-fluorescence in the lesions with a hyper-auto-fluorescent rim|
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| Discussion|| |
We present a rare case of torpedo maculopathy with two classic torpedo-shaped lesions in the same eye. Torpedo maculopathy, which has also been referred to as para-macular coloboma, is commonly described as a unilateral, congenital, solitary, hypo-pigmented torpedo-shaped lesion found in the temporal macula. It was initially described as a hypo-pigmented macular nevus and has a similar appearance to a congenital hypertrophy of RPE lesion. However, it is distinct because of its characteristic location and unique shape. It is generally considered a benign, non-progressive entity with most patients diagnosed incidentally on routine examination. The classic lesions of this entity, typically “torpedo” shaped or horizontally oval, are found in the temporal macula with the tail pointing to the fovea., Most patients are asymptomatic, and as a result, predictions on the prevalence of this condition are likely under-estimated. Although a mostly benign condition, complications such as choroidal neo-vascularization, neuro-sensory retinal detachments, or a focal non-central scotoma have been previously described.,
The pathogenesis for torpedo maculopathy is currently unknown; however, several theories have been proposed to explain the pathognomonic location in the temporal macula and similarly shaped lesions. Pian et al. hypothesized that torpedo lesions result from congenital incomplete differentiation of the arcuate bundles of the nerve fiber layer along the horizontal raphe. Shields et al. proposed that a persistent RPE defect in the temporal fetal bulge during embryogenesis results in torpedo maculopathy. Teitelbaum et al. suspects that abnormal development or disruption of choroidal vasculature development leads to the characteristic lesions. More recently, development of OCT imaging has aided in gaining a better understanding of torpedo maculopathy. Wong et al. describe two types of torpedo lesions based on differences in OCT findings. Type 1 lesions are described as a mild attenuation of outer retinal structures. Type II lesions demonstrate similar outer retinal attenuation with additional outer retinal cavitation. On FAF, the torpedo lesions typically show hypo-auto-fluorescence that corresponds to the area of RPE atrophy. Commonly, there is a rim of surrounding hyper-auto-fluorescence that is hypothesized to occur because of accumulated lipofuscin. OCT angiography may show diffuse attenuation of the choriocapillaris adjacent to the lesion. Because of the benign nature of this condition, most cases of torpedo maculopathy are managed with observation. However, some cases that demonstrate complications such as choroidal neo-vascularization have responded well to anti-vascular endothelial growth factor injections.
In the present case, this patient demonstrates two type I torpedo lesions in the same eye. Initial imaging including FAF and OCT was consistent with torpedo maculopathy. There was no evidence of progressive outer retinal atrophy associated with torpedo lesions over a 1.5-year follow-up period. This finding is consistent with the non-progressive nature of this condition. However, the second, more peripheral torpedo lesion has an irregular appearance and varied hyper-fluorescence and does not align with the horizontal raphe as would be expected if this condition was associated with nerve fiber layer abnormalities or persistent RPE defects at the site of the fetal bulge.
| Conclusion|| |
There have been previous reported cases of double torpedo maculopathy with similar orientation of the lesions; however, no follow-up examination has been published. Considering the appearance of double torpedo maculopathy, further studies are needed to describe the pathogenesis of this condition. In conclusion, torpedo maculopathy is a rare, congenital condition. Even rarer, double torpedo maculopathy has been described, to our knowledge, only once in the literature thus far without follow-up data. Our case further demonstrates the non-progressive nature of this condition. While located in the temporal macula, these lesions are unlikely to affect vision. Complications such as choroidal neovascularization have been reported, highlighting the importance of routine examinations of these typically benign lesions. Considering the appearance of double torpedo maculopathy, further long-term studies with multimodal imaging are warranted to further understand this entity.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]