Multimodal imaging to aid in diagnosis of uveal effusion syndrome type 3

2021 ◽  
Vol 14 (3) ◽  
pp. e239556
Author(s):  
Ashish Markan ◽  
Bruttendu Moharana ◽  
Mohit Dogra ◽  
Ramandeep Singh

A 47-year-old man presented to our retina clinic with gradual onset diminution of vision in his right eye for the last 3 months. Anterior segment evaluation was normal in both eyes. Fundus evaluation showed the presence of leopard spot appearance in the right eye with inferior exudative retinal detachment. Multimodal imaging was done to evaluate retinal pigment epithelium function, choroidal thickness, choroidal vascularity and scleral thickness. Multimodal imaging showed increased choroidal thickness, choroidal congestion on indocyanine green angiography and peripheral choroidal detachment with normal sclera thickness in our patient, thus pointing towards a diagnosis of uveal effusion syndrome type 3.

2020 ◽  
Vol 13 (9) ◽  
pp. e235731
Author(s):  
Padmaja Kumari Rani ◽  
Aniruddh Soni

A middle-aged man presented with exudative retinal detachment in the left eye. He was previously diagnosed as a case of large pigment epithelial detachment in the same eye, for which he was asked to follow-up closely, citing the risk of an retinal pigment epithelium (RPE) rip. Multimodal imaging confirmed the presence of a large RPE rip with exudative retinal detachment. He is a known retrovirus patient on anti-retroviral therapy with stable CD4 counts. He was diagnosed as a possible case of bullous variant of central serous chorioretinopathy and underwent external subretinal fluid drainage. We highlight the multimodal imaging findings of RPE rip with exudative retinal detachment and its impact on the patients visual acuity.


Medicina ◽  
2021 ◽  
Vol 57 (2) ◽  
pp. 120
Author(s):  
Ivajlo Popov ◽  
Veronika Popova ◽  
Vladimir Krasnik

Nanophthalmic uveal effusion syndrome (UES) is an extremely rare idiopathic disease characterized by a short axial length of the eye, extremely thick sclera and choroid. These structural changes can lead to spontaneous serous detachment of the retina and peripheral choroid. There are many other causes of UES such as trauma, inflammation, cataract surgery, glaucoma, or retinal detachment. UES is classified into three types. All are characterized by a relapsing-remitting clinical course. The loss of visual acuity ranges from mild to very severe, depending on macular involvement. Changes of the retinal pigment epithelium develop secondary after long-standing choroidal effusion and retinal detachment. Subretinal exudates could be seen and mistakenly diagnosed as chorioretinitis. UES can be very difficult to treat. The most commonly used treatment is surgery involving the creation of surgical sclerostomies (scleral window surgery) or partial thickness sclerectomies to support transscleral drainage. In our case, we present a bilateral nanophthalmic UES, which was misdiagnosed as bilateral ocular Vogt−Koyanagi−Harada disease. We documented the course of the disease and the results of the different surgical approaches in both eyes. A pars plana vitrectomy was performed in the right eye and a sclerectomy with sclerostomies in the left eye. In the left eye, even long lasting loss of visual acuity due to a serous retinal detachment was partially reversed.


2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Jae Hui Kim ◽  
Young Suk Chang ◽  
Jong Woo Kim ◽  
Chul Gu Kim ◽  
Dong Won Lee

Purpose. To investigate the incidence and timing of prechoroidal cleft development and its association with visual prognosis in type 3 neovascularization. Methods. This retrospective study included 166 eyes that were diagnosed with type 3 neovascularization. All eyes were treated with antivascular endothelial growth factor therapy. The incidence and timing of prechoroidal cleft development were evaluated. Best-corrected visual acuity (BCVA) at diagnosis and at final follow-up was compared between eyes with (cleft group) and without (no-cleft group) prechoroidal cleft. The incidence of retinal pigment epithelium (RPE) tear and subretinal hemorrhage was also compared between the two groups. Results. During the mean 39.7 ± 18.5 months of follow-up, prechoroidal cleft developed in 37 eyes (22.3%) at an average of 14.6 ± 10.4 months. The BCVA at final follow-up was significantly worse in the cleft group than in the no-cleft group (P=0.024), whereas the difference was not significant at diagnosis (P=0.969). The incidence of RPE tear (P=0.002) and subretinal hemorrhage (P<0.001) was significantly higher in the cleft group. Conclusions. Prechoroidal cleft is a frequently observed finding during the treatment course of type 3 neovascularization. Eyes with prechoroidal cleft are at high risk of RPE tear or subretinal hemorrhage and subsequently associated with poor prognosis.


2020 ◽  
Vol 6 (4) ◽  
pp. 238-243
Author(s):  
Jasmine H. Francis ◽  
Ethan K. Sobol ◽  
Molly Greenberg ◽  
Robert Folberg ◽  
David H. Abramson

Purpose: This study evaluates and characterizes the choroid underlying congenital hypertrophy of the retinal pigment epithelium (CHRPE). Methods: Retrospective observational study of CHRPE at least 2 mm in diameter. Choroidal vascular architecture was qualitatively examined. Choroidal thickness was measured by 2 independent observers using enhanced depth imaging spectral domain optical coherence tomography. Results: Forty-six eyes of 46 patients with CHRPE were included. Thirty-two lesions had imaging sufficient for analysis. Haller’s layer was healthy in 18 (56%), thin in 13 (41%), and absent in 1 (2%). Sattler’s layer was atrophic in 30 (94%), and choriocapillaris was atrophic in 31 (97%). CHRPE with thinned Haller’s layer had significantly larger diameter. The mean sub-CHRPE choroidal thickness was 82.4 ± 7.9 µm, compared to a thickness of 148.4 ± 9.6 µm in the normal adjacent choroid (p < 0.0001). Mean retinal thickness overlying the CHRPE was 77.3 ± 4.3 µm, compared to a retinal thickness of 137.8 ± 2.9 µm overlying the normal adjacent choroid (p < 0.0001). Sub-CHRPE choroidal thickness was a mean of 56.2 ± 3.1% of the adjacent normal choroidal thickness. Conclusion: The underlying choroid CHRPE is thinner than the adjacent normal choroid. All layers of the choroid can be thin with a preference of the inner Sattler’s and choriocapillaris layers.


2019 ◽  
Vol 2019 ◽  
pp. 1-8 ◽  
Author(s):  
Mona Kamal Abdellatif ◽  
Yasser Abdelmaguid Mohamed Elzankalony ◽  
Ahmed Abdelmonsef Abdelhamid Ebeid ◽  
Weam Mohamed Ebeid

Purpose. To identify and correlate age-related changes in outer retinal layers’ thickness and choroidal thickness (CT) in the normal eyes using spectral-domain optical coherence tomography (SD-OCT) and to investigate factors affecting these changes. Study Design. Observational cross-sectional study. Subjects and Methods. We studied 125 healthy Egyptians between 20 and 79 years old. Patients were divided into 3 groups: group 1 (20–40 years), group 2 (40–60 years), and group 3 (>60 years). All patients had full ophthalmic examination. SD-OCT was done to measure the 9 ETDRS macular grid sectors of retinal pigment epithelium and photoreceptor outer segment (RPE-OS), outer nuclear layer and photoreceptor inner segment (ONL-IS), and choroidal thickness (CT) (by enhanced depth imaging). Results. RPE-OS was significantly thinner in group 3 than in the other 2 groups (central: P<0.001). Moreover, the 3 groups were significantly different from each other regarding the CT (central: P<0.001); significant thinning was noticed in the choroid with age. The 3 groups did not show significant difference concerning the ONL-IS thickness. RPE-OS and CT showed statistically significant negative correlation with age (central RPE-OS: r = −0 C.345, P<0.001, and central CT: r = −0.725, P<0.001) while ONL-IS showed statistically nonsignificant correlation with age (central ONL-IS: r = −0.08, P=0.376). Multiple regression analysis revealed that the most important determinant of central 1 mm RPE-OS thickness in this study was age (β = −0.087, P=0.010) rather than choroidal thinning (β = 0.001, P=0.879). Conclusion. RPE-OS layer thickness shows significant thinning with increasing age, and with decrease in CT, however, age is the most determinant factor of this thinning.


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.


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.


2019 ◽  
Vol 104 (6) ◽  
pp. 874-878 ◽  
Author(s):  
Sarah Vandefonteyne ◽  
Jean-Pierre Caujolle ◽  
Laurence Rosier ◽  
John Conrath ◽  
Gabriel Quentel ◽  
...  

PurposePeripheral exudative haemorrhagic chorioretinopathy (PEHCR) is a rare disorder that is often misdiagnosed. The aim of this study was to better characterise PEHCR and to assess treatment options.Material and methodsRetrospective multicentric chart review.ResultsOf 84 eyes (69 patients) with PEHCR referred between 2005 and 2017, the most common referral diagnosis was choroidal melanoma (41.3%). Bilateral involvement was found in 21.7% of cases. Haemorrhagic retinal pigment epithelium detachment was the most common peripheral lesion (53.6%). Maculopathy was associated with peripheral lesions in 65.8% of cases. PEHCR lesions were mostly heterogeneous (58.8%) on B-scan ultrasonography. Choroidal neovascularisation was found in 10 eyes (26.3%) out of 38 eyes that underwent fluorescein angiography. Polyps were observed in 14 eyes (58.3%) out of 24 eyes that underwent indocyanine green angiography. Fifty-one eyes were treated (62.2%). Intravitreal injections (IVTI) of antivascular endothelial growth factor (VEGF) were the most used treatment (36.6%) before laser photocoagulation, photodynamic therapy, vitrectomy and cryotherapy. Only vitrectomy improved visual acuity. Most lesions (65.6%) regressed at the last follow-up visit.ConclusionIn case of PEHCR, multimodal imaging is useful to avoid misdiagnosis, to characterise PEHCR lesions and to guide treatment strategies. Regression of PEHCR lesions was observed in two-thirds of the patients. Vitrectomy improved visual acuity. More than a third of patients underwent anti-VEGF IVTI. Further studies are needed to assess IVTI’s efficacy.


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.


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