scholarly journals Pigmented Paravenous Retinochoroidal Atrophy: A Case Report Supported by Multimodal Imaging Studies

Medicina ◽  
2021 ◽  
Vol 57 (12) ◽  
pp. 1382
Author(s):  
Ilyoung Jung ◽  
Yeojin Lee ◽  
Seungbum Kang ◽  
Jaeyon Won

Background and Objectives: Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare disease with bilateral retinal pigment epithelium and choroidal atrophy. We present a case of PPRCA using multimodal imaging studies. Case summary: A 61-year-old female was referred to our department for floaters. Funduscopic examination revealed pigment clumps and grayish lesions along the retinal vein and the peripheral area, bilaterally. She did not have nyctalopia or any other visual symptoms including visual loss. She was diagnosed with pigmented paravenous retinochoroidal atrophy based on the typical findings of fundus. The findings of wide fluorescein angiography (FA), wide indocyanine green angiography (ICGA), fundus autofluorescence (FAF), spectral domain-optical coherence tomography (SD-OCT), optical coherence tomography angiography (OCTA), the visual field (VF) and an electroretinogram (ERG) could help us to confirm the diagnosis. The patient did not have any specific treatment for PPRCA in our study and there was no change in visual acuity and multimodal imaging of both eyes over one year. Conclusions: We report a case of PPRCA and the multimodal imaging of this patient. PPRCA is very rare disease and sometimes it is easy to get confused with other diseases such as retinitis pigmentosa and vasculitis when it comes to diagnosis. Multimodal imaging features of PPRCA will improve our understanding, diagnosis and prediction of the prognosis of this disease.

2019 ◽  
Vol 30 (3) ◽  
pp. 595-599 ◽  
Author(s):  
Andrea Scupola ◽  
Gabriela Grimaldi ◽  
Maria G Sammarco ◽  
Paola Sasso ◽  
Michele Marullo ◽  
...  

Purpose: Combined hamartoma of the retina and retinal pigment epithelium is a rare benign tumor characterized by a variable combination of glial, vascular, and pigmented components. The purpose of our study was to analyze the features of combined hamartoma of the retina and retinal pigment epithelium on optical coherence tomography angiography. Methods: Small case series of two cases of combined hamartoma of the retina and retinal pigment epithelium with macular and optic nerve involvement, evaluated with multimodal imaging including optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography. Results: On optical coherence tomography, combined hamartoma of the retina and retinal pigment epithelium is characterized by disruption of the inner neurosensory retina and a variable degree of involvement of the external retina. Optical coherence tomography angiography showed diffuse alterations of the retinal vessels of the superficial and deeper layers, extended to the peripapillary area. Vessel abnormalities included increased tortuosity and caliber of vessels, vascular traction, and vessel stretching within the lesion. Conclusion: Optical coherence tomography angiography allows in-depth multilayer analysis of tumor vascular network, highlighting the fine abnormalities of retinal vasculature characteristic of combined hamartoma of the retina and retinal pigment epithelium.


2017 ◽  
Vol 27 (1) ◽  
pp. 13-15 ◽  
Author(s):  
Livia Tomasso ◽  
Lucia Benatti ◽  
Carlo La Spina ◽  
Rosangela Lattanzio ◽  
Giovanni Baldin ◽  
...  

Purpose Handheld laser pointer thermal injury affects primarily the retinal pigment epithelium (RPE). However, so far no study has reported on the possible effects of laser pointers in the deeper layers, beneath the RPE. Here, we describe the spectral-domain optical coherence tomography angiography findings in the choriocapillaris of a patient with laser maculopathy. Methods A 13-year-old boy presented to our department with decreased vision in the left eye 12 hours after having stared at the beam of a laser pointer. Results Structural optical coherence tomography (OCT) showed 2 focal hyperreflective columns at the fovea extending from the RPE, involving all outer retinal layers, and terminating at the outer plexiform layer. The patient also underwent OCT angiography (OCT-A), which in the choriocapillary segmentation revealed 2 hypointense lesions in correspondence of the focal hyperreflectivities detected on structural OCT. We hypothesize that the OCT-A findings could represent a rarefaction of the choriocapillaris. However, the choriocapillary OCT-A findings could also represent artifacts due to the overlaying hyperreflective lesions. Conclusions It is known that the RPE is primarily damaged by the laser injury. Our findings suggest that the thermal injury could involve also the choriocapillaris, and thus not limited to the RPE. Multimodal imaging in laser maculopathy including OCT-A may lead to a better comprehension of the pathogenesis of laser retinal damages.


2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Tahsin Khundkar ◽  
Syed R. Hasan ◽  
Mark P. Breazzano ◽  
Constance Mei ◽  
Brandon B. Johnson

Purpose. To present a case of acute idiopathic maculopathy (AIM) and illustrate primary choroidal perfusion defect using multimodal imaging. Case Description. We report a case of a 24-year-old man with a paracentral scotoma of the right eye and recent flu-like illness. The patient was found to have a unilateral ovoid-shaped, placoid lesion just inferior to the fovea. Multimodal imaging confirmed findings most consistent with a diagnosis of acute idiopathic maculopathy (AIM). Serologic studies confirmed a strongly positive immunoglobulin G (IgG) titer for coxsackievirus A. Spectral-domain optical coherence tomography angiography (SD-OCTA) showed bilateral areas of vascular reduction at the level of the choriocapillaris and choroid, sparing the retinal circulation. Conclusions and Importance. The changes in outer retina and retinal pigment epithelium, classically described in AIM, are likely secondary to choroidal hypoperfusion.


2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Eliana Costanzo ◽  
Salomon Yves Cohen ◽  
Alexandra Miere ◽  
Giuseppe Querques ◽  
Vittorio Capuano ◽  
...  

Purpose. To analyze optical coherence tomography angiography (OCTA) findings in eyes with central serous chorioretinopathy (CSC) and to compare them with those obtained with multimodal imaging.Methods. A series of consecutive patients diagnosed with CSC, underwent OCTA and multimodal imaging, including spectral domain OCT, fluorescein, and indocyanine green angiography. OCTA images were performed at three main depth intervals: automatically segmented outer retina, manually adjusted outer retina, and automatically segmented choriocapillaris.Results. Thirty-three eyes of 32 consecutive patients were analyzed. OCTA showed 3 main anomalies at the choriocapillaris: the presence of dark areas (19/33 eyes) which were frequently associated with serous retinal detachment, presence of dark spots (7/33 eyes) which were frequently associated with retinal pigment epithelium detachment, and presence of abnormal vessels (12/33 eyes) which were frequently, but not systematically, associated with choroidal neovascularization, as confirmed by multimodal imaging.Conclusions. OCTA revealed dark areas and dark spots, which were commonly observed. An abnormal choroidal pattern was also observed in one-third of cases, even when multimodal imaging did not evidence any choroidal neovascularization. Abnormal choroidal vessels should be interpreted with caution, and we could assume that this pathological choroidal vascular pattern observed in many CSC cases could be distinct from CNV.


2018 ◽  
Vol 2018 ◽  
pp. 1-7 ◽  
Author(s):  
Lan Mi ◽  
Chengguo Zuo ◽  
Xiongze Zhang ◽  
Bing Liu ◽  
Yuting Peng ◽  
...  

Purpose. To determine whether fluorescein leakage within subretinal hemorrhage is definitely suggestive of choroidal neovascularization (CNV) by multimodal imaging including optical coherence tomography angiography (OCTA). Methods. Twenty-five consecutive highly myopic patients (25 eyes) with fluorescein leakage within subretinal hemorrhage detected within 1 month were prospectively included. All patients underwent OCTA and spectral-domain optical coherence tomography (SD-OCT). The OCTA and SD-OCT findings at the site of fluorescein leakage were analyzed. In cases of a doubtful diagnosis, indocyanine green angiography (ICGA) was also performed to differentiate myopic CNV from lacquer crack if necessary; all patients were followed up by SD-OCT and/or OCTA for at least 2 weeks. Results. In terms of the site of fluorescein leakage, OCTA revealed an abnormal vascular network in the outer retina and a choriocapillaris slab in 22 out of 25 eyes (88%), which were confirmed to be CNV. However, no high-flow signal was observed in 3 of 25 eyes (12%). In these 3 cases, SD-OCT showed a focal rupture of the retinal pigment epithelium-Bruch’s membrane-choriocapillaris (RPE-BM-CC) complex and a columnar hyperreflective signal of blood originating from defects with a volcanic geyser-like appearance, and no exudative signs were detected. Notably, all ruptures of the RPE-BM-CC complex were located exactly at lacquer crack sites. Moreover, with the absorption of subretinal hemorrhage, ruptures of the RPE-BM-CC complex spontaneously resolved without any intervention. Considering the multimodal imaging appearance and follow-up outcomes, these 3 eyes were eventually diagnosed as simple bleeding associated with lacquer cracks. Conclusions. Dye leakage within recent subretinal hemorrhage on FA could be caused by new-onset lacquer cracks in pathologic myopia. Multimodal imaging including OCTA is helpful to differentiate lacquer cracks from myopic CNV.


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. 112067212110559
Author(s):  
Isil Sayman Muslubas ◽  
Serra Arf ◽  
Mumin Hocaoglu ◽  
M. Giray Ersoz ◽  
Murat Karacorlu

Purpose To report clinical and multimodal imaging features of Best disease in patients presenting with subretinal pigment epithelium hyperreflective lesions. Design Retrospective study. Methods Clinical examination findings and multimodal imaging features, including color fundus photography, spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence, fluorescein and indocyanine green angiography (ICGA), and optical coherence tomography angiography (OCTA) images were evaluated retrospectively. Results We assessed 27 eyes of 16 patients with the diagnosis of Best disease. Only patients presenting with serous macular detachment and subretinal pigment epithelium hyperreflective lesion in one or both eyes were included in this study. In 17 of 27 eyes (63%), fibrosis was identified by multimodal imaging techniques. Although there was no sign of active neovascularization on fundus examination or SD-OCT, a vascular network could be identified in 7 eyes (26%) (in 1 eye with OCTA only and in 6 eyes with both OCTA and ICGA). Active neovascularization was seen in 3 eyes (11%). Treatment was recommended for eyes with active neovascularization, and follow-up was scheduled for eyes with quiescent neovascularization and fibrosis. Conclusion Eyes with Best disease with subretinal pigment epithelium hyperreflective lesion and serous macular detachment may show fibrosis, quiescent neovascularization, or active neovascularization. Multimodal imaging techniques are very important for differentiation of these lesions.


2021 ◽  
Author(s):  
Isil Sayman Muslubas ◽  
Serra Arf ◽  
Mumin Hocaoglu ◽  
M. Giray Ersoz ◽  
Murat Karacorlu

Abstract PurposeTo report clinical and multimodal imaging features of Best disease in patients presenting with subretinal pigment epithelium hyperreflective lesion.DesignRetrospective study.MethodsClinical examination findings and multimodal imaging features, including color fundus photography, spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence, fluorescein and indocyanine green angiography (ICGA), and optical coherence tomography angiography (OCTA) images were evaluated retrospectively. ResultsWe assessed 27 eyes of 16 patients with the diagnosis of Best disease. Only patients presenting with serous macular detachment and subretinal pigment epithelium hyperreflective lesion in one or both eyes were included in this study. In 17 of 27 eyes (63%), fibrosis was identified by multimodal imaging techniques. Although there was no sign of active neovascularization on fundus examination or SD-OCT, a vascular network could be identified in 7 eyes (26%) (in 1 eye with OCTA only and in 6 with both OCTA and ICGA). Active neovascularization was seen in 3 eyes (11%). Treatment was recommended for eyes with active neovascularization, and follow-up was scheduled for eyes with quiescent neovascularization and fibrosis.ConclusionEyes with Best disease with subretinal pigment epithelium hyperreflective lesion and serous macular detachment may show fibrosis, quiescent neovascularization, or active neovascularization. Multimodal imaging techniques are very important for differentiation of these lesions.


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