scholarly journals Multimodal Imaging of Torpedo Maculopathy With Fluorescence Adaptive Optics Imaging of Individual Retinal Pigmented Epithelial Cells

2021 ◽  
Vol 8 ◽  
Author(s):  
Kari V. Vienola ◽  
Kunal K. Dansingani ◽  
Andrew W. Eller ◽  
Joseph N. Martel ◽  
Valerie C. Snyder ◽  
...  
Keyword(s):  
Adaptive Optics ◽  
Imaging Techniques ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Tissue Level ◽  
Cellular Level ◽  
Clinical Imaging ◽  
Rpe Cells ◽  
Torpedo Maculopathy

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.

scholarly journals Assessment of fundus autofluorescence and adaptive optics high-resolution images in macular disorders

2021 ◽  
Author(s):  
Radu Ochinciuc ◽  
Uliana Ochinciuc ◽  
George Balta ◽  
Adrian Teodoru ◽  
Leila Al Barri ◽  
...  
Keyword(s):  
High Resolution ◽  
Adaptive Optics ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Subretinal Fluid ◽  
Cellular Level ◽  
Cone Mosaic ◽  
High Resolution Images ◽  
Cone Density

Abstract Purpose This study analyzed and compared the results of adaptive optics (AO) and fundus autofluorescence (FAF) in various maculopathies. Methods The study included four different types of maculopathy: central serous chorioretinopathy (CSC), retinitis pigmentosa (RP), Stargardt disease (STGD) and phototoxic retinopathy. In all four cases cone mosaic and cone density were obtained using AO fundus camera. Further the high resolution images were compared with the FAF and optical coherence tomography (OCT) results. Results In CSC, FAF and AO were able to show changes in the macula even two years after the subretinal fluid resorption, as opposed to a normal OCT. The improvement of FAF and cone mosaic appearance was concomitant with the visual acuity growth. Several cone mosaic phenotypes were observed in RP and STGD. In RP the cone density was 24.240cones/mm2 in the center, and decreased to 8.163cones/mm2 in the parafoveolar area. In STGD the cone density was lower in the center, 9.219cones/mm2, and higher at the periphery, 12.594cones/mm2. In the case of phototoxic retinopathy, AO and OCT were more effective than FAF in highlighting the photoreceptor and retinal pigment epithelium lesions. Conclusions FAF and AO are very useful tools in macular pathologies examination. FAF can give us a true picture about metabolic changes in the macula while AO allows us to view changes up to the cellular level.

Torpedo Maculopathy Presenting With Fovea Plana in a 21-Year-Old Female

2017 ◽  
Vol 1 (6) ◽  
pp. 424-427 ◽  
Author(s):  
Douglas S. M. Iaboni ◽  
Mark E. Seamone ◽  
Netan Choudhry ◽  
R. Rishi Gupta
Keyword(s):  
Optical Coherence Tomography ◽  
Fluorescein Angiography ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Spectral Domain ◽  
Optical Coherence ◽  
En Face Oct ◽  
En Face ◽  
Torpedo Maculopathy

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.

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
Keyword(s):  
Visual Field ◽  
Multimodal Imaging ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Chinese Woman ◽  
Fluorescence Angiography ◽  
Macular Lesion ◽  
Funduscopic Examination ◽  
Torpedo Maculopathy

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.

scholarly journals Near-infrared fundus autofluorescence imaging in a case of photic maculopathy

2014 ◽  
Vol 1 (1) ◽  
pp. 28
Author(s):  
Alberto Neri ◽  
Alessandra Protti ◽  
Monica Camparini ◽  
Claudio Macaluso
Keyword(s):  
Near Infrared ◽  
Imaging Modality ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Parallel Evolution ◽  
Conventional Imaging ◽  
Autofluorescence Imaging ◽  
Rpe Cells

Herein we describe the characteristics of scanning laser ophthalmoscope imaging (SLO), and particularly of near-infrared fundus autofluorescence modality (IRAF), in photic maculopathy. IRAF visualizes selectively the melanin granules contained in the cells of the Retinal Pigment Epithelium (RPE), which are normally localized in the apical portion of RPE cells, a favorite target of the photic damage to the retina. In the present work we report a three-month follow-up of a case of photic maculopathy, showing that the retinal alterations found by IRAF precisely matched the retinal anomalies found by macular optical coherence tomography, and had a parallel evolution. We think that IRAF could represent a useful imaging modality, together with more conventional imaging, in the management of photic maculopathy. 

Multimodal imaging of torpedo maculopathy including adaptive optics

2019 ◽  
Vol 30 (2) ◽  
pp. NP27-NP31 ◽  
Author(s):  
Juliette Hugo ◽  
Marie Beylerian ◽  
Eric Denion ◽  
Aurore Aziz ◽  
Pierre Gascon ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Retinal Pigment Epithelium ◽  
Fluorescein Angiography ◽  
Adaptive Optics ◽  
Multimodal Imaging ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Optical Coherence ◽  
Cone Mosaic ◽  
Torpedo Maculopathy

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.

scholarly journals Hyperpigmented Torpedo Maculopathy with Pseudo-Lacuna: A 5-Year Follow-Up

2016 ◽  
Vol 7 (1) ◽  
pp. 184-190 ◽  
Author(s):  
Austin Rohl ◽  
Sushma Vance
Keyword(s):  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Vertical Axis ◽  
Enhanced Depth Imaging ◽  
Fundus Photography ◽  
Unknown Etiology ◽  
And Migration ◽  
Torpedo Maculopathy

Purpose: The aim of the study was to describe a case of globally hyperpigmented torpedo maculopathy that also contained a novel central lesion resembling a ‘pseudo-lacuna’. We compare the morphology of the lesion after 5 years of follow-up. Case Presentation: An asymptomatic 10-year-old Caucasian male was referred by his optometrist after having found a hyperpigmented lesion on routine dilated examination in 2010. Color fundus photography OS from October 2015 showed a 1.74 × 0.67 mm hyperpigmented oval-shaped lesion temporal to the macula. Since June 2010, the hyperpigmented torpedo lesion appeared to have assumed a more ovoid shape and increased in size in the vertical axis. Centrally, there was a small pearlescent-colored pseudo-lacuna lesion that seemed to also have significantly increased in size since June 2010. Enhanced depth imaging optical coherence tomography of this pseudo-lacuna showed retinal pigment epithelium clumping and migration. Fundus autofluorescence revealed reduced autofluorescence of the torpedo lesion and marked hyperautofluorescence of the pseudo-lacuna. Fluorescein angiography shows no neovascular disease or leakage. Conclusion: Torpedo maculopathy has been described previously as a hypopigmented, nonprogressive lesion of unknown etiology. The findings of global hyperpigmentation, pseudo-lacuna formation, and morphologic changes over time in this lesion challenge these classically held descriptions, and necessitate long-term follow-up with multimodal imaging.

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
Keyword(s):  
Visual Field ◽  
Multimodal Imaging ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Chinese Woman ◽  
Fluorescence Angiography ◽  
Macular Area ◽  
Macular Lesion ◽  
Torpedo Maculopathy

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.

scholarly journals A human model of Batten disease shows role of CLN3 in phagocytosis at the photoreceptor–RPE interface

2021 ◽  
Vol 4 (1) ◽  
Author(s):  
Cynthia Tang ◽  
Jimin Han ◽  
Sonal Dalvi ◽  
Kannan Manian ◽  
Lauren Winschel ◽  
...  
Keyword(s):  
Photoreceptor Cell ◽  
Vision Loss ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Cell Loss ◽  
Human Model ◽  
Lysosomal Storage ◽  
Rpe Cells ◽  
Cln3 Disease

AbstractMutations in CLN3 lead to photoreceptor cell loss in CLN3 disease, a lysosomal storage disorder characterized by childhood-onset vision loss, neurological impairment, and premature death. However, how CLN3 mutations cause photoreceptor cell death is not known. Here, we show that CLN3 is required for phagocytosis of photoreceptor outer segment (POS) by retinal pigment epithelium (RPE) cells, a cellular process essential for photoreceptor survival. Specifically, a proportion of CLN3 in human, mouse, and iPSC-RPE cells localized to RPE microvilli, the site of POS phagocytosis. Furthermore, patient-derived CLN3 disease iPSC-RPE cells showed decreased RPE microvilli density and reduced POS binding and ingestion. Notably, POS phagocytosis defect in CLN3 disease iPSC-RPE cells could be rescued by wild-type CLN3 gene supplementation. Altogether, these results illustrate a novel role of CLN3 in regulating POS phagocytosis and suggest a contribution of primary RPE dysfunction for photoreceptor cell loss in CLN3 disease that can be targeted by gene therapy.

The cytoskeletal elements of human retinal pigment epithelium: in vitro and in vivo

1988 ◽  
Vol 91 (2) ◽  
pp. 303-312
Author(s):  
N.M. McKechnie ◽  
M. Boulton ◽  
H.L. Robey ◽  
F.J. Savage ◽  
I. Grierson
Keyword(s):  
Retinal Pigment Epithelium ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Cytokeratin 18 ◽  
Rpe Cells ◽  
Human Retinal Pigment Epithelium ◽  
Cytoskeletal Elements

The cytoskeletal elements of normal (in situ) and cultured human retinal pigment epithelium (RPE) were studied by a variety of immunocytochemical techniques. Primary antibodies to vimentin and cytokeratins were used. Positive immunoreactivity for vimentin was obtained with in situ and cultured material. The pattern of reactivity obtained with antisera and monoclonals to cytokeratins was more complex. Cytokeratin immunoreactivity could be demonstrated in situ and in cultured cells. The pattern of cytokeratin expression was similar to that of simple or glandular epithelia. A monoclonal antibody that specifically recognizes cytokeratin 18 identified a population of cultured RPE cells that had particularly well-defined filamentous networks within their cytoplasm. Freshly isolated RPE was cytokeratin 18 negative by immunofluorescence, but upon culture cytokeratin 18 positive cells were identifiable. Cytokeratin 18 positive cells were identified in all RPE cultures (other than early primaries), regardless of passage number, age or sex of the donor. In post-confluent cultures cytokeratin 18 cells were identified growing over cytokeratin 18 negative cells, suggesting an association of cytokeratin 18 immunoreactivity with cell proliferation. Immunofluorescence studies of retinal scar tissue from two individuals revealed the presence of numerous cytokeratin 18 positive cells. These findings indicate that RPE cells can be identified by their cytokeratin immunoreactivity and that the overt expression of cytokeratin 18 may be associated with proliferation of human RPE both in vitro and in vivo.

scholarly journals Giant Idiopathic Angioid Streaks

2020 ◽  
Author(s):  
Brijesh Takkar ◽  
Anubha Rathi ◽  
Pradeep Venkatesh ◽  
Atul Kumar
Keyword(s):  
Imaging Techniques ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Young Male ◽  
Significant Finding ◽  
Angioid Streaks ◽  
Retinal Hemorrhages ◽  
Swept Source ◽  
Steroid Abuse ◽  
Membrane Complex

Purpose: To present a case of gigantic idiopathic angioid streaks. Case Report: A young male presented with macular choroidal neovascular membrane (CNVM) and peripheral retinal hemorrhages secondary to angioid streaks. Swept source optical coherence tomography (SSOCT) and ultrawide field imaging were performed. The latter revealed extension of the angioid streaks up to the equator in both eyes. SSOCT showed breaks in the retinal pigment epithelium-Bruch’s membrane complex in the area of peripheral retinal hemorrhages. The patient was extensively worked up for systemic associations, and the only significant finding was a long history of steroid abuse in the past. Conclusion: Advanced imaging techniques helped to diagnose angioid streaks in this patient. The possible role of steroid abuse in accentuating the presentation of angioid streaks may be explored further.

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