scholarly journals Multimodal imaging of torpedo maculopathy in a Chinese woman: a case report

2019 ◽  
Author(s):  
Yuhua Ding ◽  
Bangtao Yao ◽  
Hui Ye ◽  
Yan Yu

Abstract Background: Torpedo maculopathy is a rare, benign, and congenital macular lesion that typically appears in ‘torpedo-shape’ and located at the temporal macula region. The purpose of this article is to describe in detailed regarding the torpedo maculopathy in a Chinese woman using multimodal imaging. Case presentation: A 30-year-old Chinese woman occasionally had an yellowish-white macular lesion in her right eye during a routine examination. She had no symptoms, and the best-corrected visual acuity of both eyes was 6/6. Funduscopic examination revealed a torpedo-shaped and mild hyperpigmentation lesion in the temporal macular area of her right eye. Infrared fundal (IR) photograph showed that the lesion contour was visible, transverse elliptical, a tip pointed towards the central fovea of the macula. Microperimetry visual field appeared normal. The spectral-domain optical coherence tomography (SD-OCT) showed a normal inner, mildly thinned retinal pigment epithelium (RPE), and increased choroidal signal. OCT angiography (OCTA) choroid capillary segment revealed increased density of the choroidal vasculature. With fundus autofluorescence (FAF), the lesion showed slight hypoautofluorescence, and marginal mild hypoautofluorescence. Fundus fluorescence angiography (FFA) of the lesion showed variegated fluorescence and no leakage and change in the morphology during the whole imaging process. Conclusions: This is the first report to put forward a thorough and detailed description of torpedo maculopathy simultaneously using fundal photograph, IR, microperimetry visual field, OCT, OCTA, FAF, and FFA. Multimodal imaging provides precious and detailed information to further clarify the characteristics and development of this rare disease.

2019 ◽  
Author(s):  
Yuhua Ding ◽  
Bangtao Yao ◽  
Hui Ye ◽  
Yan Yu

Abstract Background: Torpedo maculopathy is a rare, benign, and congenital macular lesion that typically appears in a ‘torpedo-shape’ and is located at the temporal macula region. This study aimed to describe in detail regarding torpedo maculopathy in a Chinese woman using multimodal imaging. Case presentation: A 30-year-old Chinese woman with occasional yellowish-white macular lesions in her right eye during a routine examination was presented to our hospital. She had no other symptoms, and the best-corrected visual acuity of both eyes was 6/6. Funduscopic examination revealed a torpedo-shaped and mild hypopigmented lesion in the temporal macular area of her right eye. Infrared fundal (IR) images showed visible lesion contour, transverse elliptical, and with a tip pointing towards the central fovea of the macula. Microperimetry visual field appeared normal. The spectral-domain optical coherence tomography (SD-OCT) showed a normal inner retina, with mild thinner outer retina and retinal pigment epithelium in the temporal macular area, and correspondingly increased choroidal reflectivity. Other OCT findings included outer retinal loss/attenuation with significant atrophy of an intact ellipsoid zone. OCT angiography (OCTA) of choroid capillary layer revealed increased density of choroidal vasculature in corresponding to the area of the lesion, while the superficial and deep layers revealed normal vasculature. Fundus autofluorescence (FAF) revealed normal signal with slight hyperautofluorescence at the nasal lesion margin. Fundus fluorescence angiography (FFA) of the lesion showed variegated fluorescence and no leakage and change in the morphology during the whole imaging process. Conclusions: This is the first report to include a thorough and detailed description of torpedo maculopathy by using fundal photograph, IR, microperimetry visual field, OCT, OCTA, FAF, and FFA. Multimodal imaging provides precious and detailed information to further clarify the characteristics and development of this rare disease.


2019 ◽  
Author(s):  
Yuhua Ding ◽  
Bangtao Yao ◽  
Hui Ye ◽  
Yan Yu

Abstract Background: Torpedo maculopathy is a rare, benign, and congenital macular lesion that typically appears in ‘torpedo-shape’ and is located at the temporal macula region. This article aimed to describe in detail regarding the torpedo maculopathy in a Chinese woman using multimodal imaging. Case presentation: A 30-year-old Chinese woman with occasional yellowish-white macular lesions in her right eye during a routine examination was presented to our hospital. She had no symptoms, and the best-corrected visual acuity of both eyes was 6/6. Funduscopic examination revealed a torpedo-shaped and mild hypopigmentation lesion in the temporal macular area of her right eye. Infrared fundal (IR) photograph showed that the lesion contour was visible, transverse elliptical, with a tip pointing towards the central fovea of the macula. Microperimetry visual field appeared normal. The spectral-domain optical coherence tomography (SD-OCT) showed a normal inner retina, with mild thinner outer retina and RPE in the temporal macular area, and correspondingly increased choroidal reflectivity. Other OCT findings included outer retinal loss/attenuation with significant atrophy of an intact ellipsoid zone. OCT angiography (OCTA) of choroid capillary layer revealed increased density of choroidal vasculature, corresponding to the area of the lesion, while the superficial and deep layers revealed normal vasculature. Fundus autofluorescence (FAF) revealed normal signal with slight hyperautofluorescence at the nasal lesion margin. Fundus fluorescence angiography (FFA) of the lesion showed variegated fluorescence and no leakage and change in the morphology during the whole imaging process. Conclusions: This is the first report that put forwarded a thorough and detailed description of torpedo maculopathy simultaneously by using fundal photograph, IR, microperimetry visual field, OCT, OCTA, FAF, and FFA. Multimodal imaging provides precious and detailed information to further clarify the characteristics and development of this rare disease.


2020 ◽  
pp. 112067212095758
Author(s):  
Ahmet Kaan Gündüz ◽  
Carol L Shields ◽  
Şükran Bekdemir ◽  
Jerry A Shields

Purpose: To investigate the alterations in the retinal pigment epithelium (RPE) in the crest and trough portions of chorioretinal folds (CRFs) induced by an orbital vascular tumor. Methods: Review of multimodal imaging in two eyes of two patients with globe compression and CRFs from an orbital vascular tumor. Results: Fundus photography demonstrated obliquely extending CRFs with alternating hyperpigmented and hypopigmented linear alterations in both eyes. Fundus autofluorescence (AF) imaging showed obliquely oriented hypoAF lines, incompletely alternating with hyperAF lines. In Case 1, the hyperAF lines had interspersed hypoAF segments and Case 2 had peripapillary mottling of AF. Fluorescein angiography (FA) showed alternating hyper and hypofluorescent lines in the late phase in Case 1. Optical coherence tomography (OCT) documented relative thinning of RPE at the folded crests in Case 1 and preservation of RPE in Case 2. Swept-source OCT angiography (SS-OCTA) demonstrated oblique hyporeflective lines in the outer retina and choriocapillaris layers in Case 2. These findings suggest that the crest of a CRF represents thinned or rarified RPE with hypoAF, transmission hyperfluorescence (FA), partially attenuated RPE layer (OCT), and isoreflectivity (SS-OCTA) while the trough represents compressed RPE with irregular hyperAF, transmission hypofluorescence (FA), thickened RPE layer (OCT), and hyporeflectivity (SS-OCTA). Conclusion: The anatomic and functional status of the RPE in CRFs based on multimodal imaging reveals normal to attenuated RPE with hypofunctionality at the fold crest and compacted, thickened RPE at the trough with segmental functional impairment on AF imaging. Anatomic information regarding CRFs is evident on OCT, FA, and SS-OCTA while the functional status is depicted on AF.


2016 ◽  
Vol 2016 ◽  
pp. 1-12 ◽  
Author(s):  
Yao Wang ◽  
Zhi-Qing Chen ◽  
Wei Wang ◽  
Xiao-Yun Fang

Purpose. To investigate the prevalence and characteristics of focal choroidal excavation (FCE) concurrent with central serous chorioretinopathy (CSC) using multimodal imaging.Methods. This was a retrospective single-institution study. Clinical features and multimodal imaging findings were analyzed in eyes with CSC and FCEs, using imaging methods including optical coherence tomography (OCT), OCT angiography (OCTA), fluorescein angiography (FA), indocyanine green angiography (ICGA), fundus autofluorescence (FAF), and multispectral imaging.Results. Seventeen patients (4.8%) with 21 FCEs (19 eyes) were found among 351 consecutive Chinese patients with CSC. Chronic CSC represented 47.1% of those cases. Window defects in 12 lesions identified through FA and hypoautofluorescence in 13 lesions identified through FAF revealed retinal pigment epithelial attenuation. Choroidal hemodynamic disturbances characterized by localized filling defects at the excavation and circumferential hyperperfusion were validated by both ICGA and OCTA, which were similar to the angiographic features of normal chronic CSC. The hyperreflective tissue beneath FCE, observed on B-scan OCT, presented as intensive choroidal flow signals on OCTA.Conclusions. FCE is not uncommon in patients with CSC. Multimodal imaging suggested that the aberrant choroidal circulation might be a contribution factor for leakage from the dysfunctional retinal pigment epithelium at the area of excavation.


2017 ◽  
Vol 1 (6) ◽  
pp. 424-427 ◽  
Author(s):  
Douglas S. M. Iaboni ◽  
Mark E. Seamone ◽  
Netan Choudhry ◽  
R. Rishi Gupta

Purpose: To report a case of torpedo maculopathy presenting with fovea plana in a 21-year-old female patient. Methods: Multimodal imaging including fluorescein angiography, fundus autofluorescence, and spectral domain optical coherence tomography (OCT) was used to characterize pathology. Results: A well-circumscribed ellipsoidal hypopigmented lesion was observed inferotemporal to the fovea OS. Fluorescein angiography and fundus autofluorescence revealed hyperfluorescence and focal hypoautofluorescence, respectively, corresponding to the hypopigmented lesion. Spectral domain optical coherence tomography imaging of the lesion revealed attenuation of the ellipsoid zone and retinal pigment epithelium with a hyporeflective subretinal cleft. En face OCT imaging demonstrated an area of subretinal hyporeflectivity at the subretinal cleft. Spectral domain optical coherence tomography revealed the absence of a foveal pit. Conclusion: We have described a novel case presentation of fovea plana alongside torpedo maculopathy. The significance of this association remains unclear. Further study into these conditions is necessary to help better determine factors responsible for visual symptoms or lack thereof and circumstances that promote their development.


2021 ◽  
Vol 8 ◽  
Author(s):  
Kari V. Vienola ◽  
Kunal K. Dansingani ◽  
Andrew W. Eller ◽  
Joseph N. Martel ◽  
Valerie C. Snyder ◽  
...  

Torpedo maculopathy (TM) is a rare congenital defect of the retinal pigment epithelium (RPE). The RPE is often evaluated clinically using fundus autofluorescence (AF), a technique that visualizes RPE structure at the tissue level from the intrinsic AF of RPE fluorophores. TM lesions typically emit little or no AF, but this macroscopic assessment is unable to resolve the RPE cells, leaving the organization of the RPE cell mosaic in TM unknown. We used fluorescence adaptive optics scanning laser ophthalmoscopy (AOSLO) to show here for the first time the microscopic cellular-level structural alterations to the RPE cell mosaic in TM that underlie the tissue-level changes seen in conventional clinical imaging. We evaluated two patients with TM using conventional clinical imaging techniques and adaptive optics (AO) infrared autofluorescence (IRAF) in AOSLO. Confocal AOSLO revealed relatively normal cones outside the TM lesion but altered cone appearance within it and along its margins in both patients. We quantified cone topography and RPE cell morphometry from the fovea to the margin of the lesion in case 1 and found cone density to be within the normal range across the locations imaged. However, RPE morphometric analysis revealed disrupted RPE cells outside the margin of the lesion; the mean RPE cell area was greater than two standard deviations above the normative range up to approximately 1.5 mm from the lesion margin. Similar morphometric changes were seen to individual RPE cells in case 2. Multi-modal imaging with AOSLO reveals that RPE cells are abnormal in TM well beyond the margins of the characteristic TM lesion boundary defined with conventional clinical imaging. Since the TM fovea appears to be fully formed, with normal cone packing, it is possible that the congenital RPE defect in TM occurs relatively late in retinal development. This work demonstrates how cellular level imaging of the RPE can provide new insight into RPE pathologies, particularly for rare conditions such as TM.


Diagnostics ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 1184
Author(s):  
Carl P. Herbort ◽  
Ilir Arapi ◽  
Ioannis Papasavvas ◽  
Alessandro Mantovani ◽  
Bruno Jeannin

Background and aim: AZOOR is a rare disease characterized by loss of zones of outer retinal function, first described by J Donald Gass in 1993. Symptoms include acute onset photopsias and subjective visual field losses. The syndrome is characterized by a normal fundus appearance, scotomas and electroretinographic changes pointing towards outer retinal dysfunction. Evolution, response to immunosuppressive treatment and outcome are difficult to predict. The aim of this small case series was to identify the morphological changes and sequence of events in AZOOR thanks to multimodal imaging. Methods: Charts of AZOOR patients seen in the Centre for Ophthalmic Specialized care (COS, Lausanne, Switzerland) were analyzed by multimodal imaging including fundus photography, fluorescein angiography (FA), indocyanine green angiography (ICGA), blue light fundus autofluorescence (BL-FAF) and spectral domain optical coherence tomography (SD-OCT) in addition to a complete ophthalmological examination including visual field testing and microperimetry, as well as OCT angiography (OCT-A) and ganglion-cell complex analysis when available. Cases and Results: Three AZOOR patients with a mean follow-up of 47 ± 25.5 months were included following the clinical definitions laid down by J Donald Gass. The primary damage was identified at the level of the photoreceptor outer segments with an intact choriocapillaris and retinal pigment epithelium (RPE) layer, these structures being only secondarily involved with progression of the disease. Conclusion: Although AZOOR has often been included within white dot syndromes, some of which are now known to be choriocapillaris diseases (choriocapillaritis entities), our findings clearly commend to differentiate AZOOR from entities such as MEWDS (Multiple evanescent white dot syndrome), APMPPE (Acute Posterior Multifocal Placoid Pigment Epitheliopathy), MFC (Multifocal Choroiditis) and others, as the damage to photoreceptors is primary in AZOOR (a retinopathy) and secondary in choriocapillaritis (a choriocapillaropathy).


2022 ◽  
Vol 22 (1) ◽  
Author(s):  
Zhe Zhu ◽  
Jun Xiao ◽  
Lifu Luo ◽  
Bo Yang ◽  
He Zou ◽  
...  

Abstract Background Herein, we report two cases of unilateral retinal pigment epithelium dysgenesis (URPED) in Chinese patients and explore the relationship between URPED and combined hamartoma of the retina and retinal pigment epithelium (CHRRPE). Case presentation The lesion margins in the two cases showed pathognomonic clinical features of URPED, namely, a scalloped reticular margin in hyperplastic retinal pigment epithelium and mild fibrosis. The hypoautofluorescence observed by fundus autofluorescence was inverted compared with that observed by fundus fluorescence angiography. A large amount of fibroglial proliferation and disorganization of the retina involving the whole layer, which are also found in peripapillary CHRRPE, were found in the lesions. Conclusions URPED appears to share some clinical features with CHRRPE, and the relationship between URPED and CHRRPE needs further study.


2019 ◽  
Vol 30 (2) ◽  
pp. NP27-NP31 ◽  
Author(s):  
Juliette Hugo ◽  
Marie Beylerian ◽  
Eric Denion ◽  
Aurore Aziz ◽  
Pierre Gascon ◽  
...  

Purpose: The etiology of torpedo maculopathy remains unknown, but it has been recently suggested that it could represent a persistent defect in the development of the retinal pigment epithelium. As retinal pigment epithelium and photoreceptors form a functional unit, an alteration of photoreceptor distribution or function is predictable. The aim of this study is to describe multimodal imaging, including adaptive optics, in three cases of torpedo maculopathy, and discuss its pathogenesis. Methods: Multimodal imaging is presented, including fundus photographs, optical coherence tomography, adaptive optics, autofluorescence, fluorescein angiography, and ultra-widefield retinal imaging in three cases of torpedo maculopathy. Results: An oval-shaped well-delimited chorioretinal lesion both hypopigmented centrally and with a hyperpigmented border in the temporal macula, consistent with torpedo maculopathy, was observed in three patients. Optical coherence tomography showed a preservation of the inner retina, a mild atrophy of the outer retina, an alteration of the ellipsoid zone and of the retinal pigment epithelium layer, and a neurosensory detachment. These lesions were hypoautofluorescent with a hyperautofluorescent border. Fluorescein angiography showed a hyperfluorescence by window effect. Adaptive optics imaging showed an alteration of the cone mosaic within the lesions, with a lower cone density and a higher spacing between cones. Conclusion: The alteration of the cone mosaic suggested by adaptive optics in torpedo maculopathy has never been described and could be explained by the alteration of the retinal pigment epithelium. Our results support the existing hypothesis on the pathogenesis of torpedo maculopathy that a persistent defect in the development of the retinal pigment epithelium may be responsible for this clinical entity.


2021 ◽  
pp. 112067212110136
Author(s):  
Supriya Arora ◽  
Alexei N Kulikov ◽  
Dmitrii S Maltsev

Purpose: To study the implementation of the new multimodal imaging-based classification system of central serous chorioretinopathy (CSCR). Methods: Ninety-three eyes with CSCR with available fundus autofluorescence (FAF), optical coherence tomography (OCT), and OCT angiography at presentation were included in this study. An anonymous data set was classified by two masked graders. Each case was classified as per presence of (i) simple versus complex (< or >2 disc diameters of retinal pigment epithelium abnormality) CSCR; (ii) primary versus recurrent versus resolved CSCR; (iii) persistent (presence of subretinal fluid >6 months) or not; (iv) outer retinal atrophy (ORA); (v) foveal involvement; and (vi) macular neovascularization (MNV). Agreement between the graders was calculated. Results: Kappa value was 0.91 (95% CI 0.8–1.0) for the entire classification; 0.84 (95% CI 0.73–0.95) for simple versus complex; 1.0 (95% CI 1.0–1.0) for primary versus recurrent versus resolved CSCR; 1.0 (95% CI 1.0–1.0) for persistent or not; 0.9 (95% CI 0.81–0.99) for ORA or not; 0.95 (95% CI 0.84–1.0) for presence or absence of MNV; 1.0 (95% CI 1.0–1.0) for presence or absence of foveal involvement. Conclusion: The new multimodal imaging based CSCR classification showed “near perfect” agreement between two retinal experts.


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