Diagnosis and treatment of peripheral exudative haemorrhagic chorioretinopathy

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.

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Combined Hamartoma of the Retina and Retinal Pigment Epithelium in a Patient with Gorlin Syndrome: Spontaneous Partial Resolution of Traction Caused by Epiretinal Membrane

Case Reports in Ophthalmological Medicine ◽

10.1155/2016/2312196 ◽

2016 ◽

Vol 2016 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

José L. Sánchez-Vicente ◽

Miguel Contreras-Díaz ◽

Trinidad Rueda ◽

Enrique Rodríguez de la Rúa-Franch ◽

Fredy E. Molina-Socola ◽

...

Keyword(s):

Visual Acuity ◽

Retinal Pigment Epithelium ◽

Epiretinal Membrane ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Spontaneous Resolution ◽

Fundus Examination ◽

Gorlin Syndrome ◽

Clinical Context

Purpose. To describe the case of spontaneous resolution of epiretinal membrane in a patient with Combined Hamartoma of the Retina and Retinal Pigment Epithelium (CHR-RPE), in the clinical context of Gorlin Syndrome (GS).Methods. Observational case report of a 12-year-old female patient is presented. The diagnosis of CHRRPE was made by OCT and fundus examination, which showed a mound of disorganized tissue originating from retina and retinal pigment epithelium. Epiretinal membrane (EM) was also detected. Genetic study was performed to confirm the diagnosis of GS.Results. The patient was observed for 39 months, showing spontaneous resolution of the traction caused by the EM and improvement in visual acuity (VA), which was 20/80 at initial presentation, rising to 20/40 after follow-up period.Conclusions. The presence of EM in CHR-REP is a cause of reduction of visual acuity. Management of this condition is controversial; however, we would like to highlight that spontaneous resolution of the traction caused by EM is possible, resulting in recovery of VA.

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Prechoroidal Cleft in Type 3 Neovascularization: Incidence, Timing, and Its Association with Visual Outcome

Journal of Ophthalmology ◽

10.1155/2018/2578349 ◽

2018 ◽

Vol 2018 ◽

pp. 1-8 ◽

Cited By ~ 1

Author(s):

Jae Hui Kim ◽

Young Suk Chang ◽

Jong Woo Kim ◽

Chul Gu Kim ◽

Dong Won Lee

Keyword(s):

Pigment Epithelium ◽

Retinal Pigment ◽

Visual Outcome ◽

Subretinal Hemorrhage ◽

Type 3 Neovascularization ◽

The Difference ◽

Type 3 ◽

Endothelial Growth Factor ◽

Rpe Tear

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.

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Traumatic submacular hemorrhage: available treatment options and synthesis of the literature

International Journal of Retina and Vitreous ◽

10.1186/s40942-019-0200-0 ◽

2019 ◽

Vol 5 (1) ◽

Cited By ~ 1

Author(s):

Giamberto Casini ◽

Pasquale Loiudice ◽

Martina Menchini ◽

Francesco Sartini ◽

Stefano De Cillà ◽

...

Keyword(s):

Intravitreal Injection ◽

Case Reports ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Treatment Options ◽

Young Patients ◽

Treatment Strategies ◽

Mechanical Damage ◽

Invasive Treatment ◽

Pneumatic Displacement

AbstractSub-macular hemorrhage (SMH) is a hematic collection between the neurosensory retina and the retinal pigment epithelium; one of its causes is ocular blunt trauma, that usually affects young patients. Persisting SMH leads to a damage of photoreceptors mediated by three main mechanisms: iron-related toxicity, impairment of diffusion of oxygen and nutriment, mechanical damage due to clot contraction. Since early photoreceptors’ damage has been reported within 24 h, it is suggested to provide an early treatment, although there are no guidelines or consensus between authors regarding treatment strategies. The aim of this review was to present and compare available treatment options, like intravitreal tissue plasminogen activator (tPA) associated with pneumatic displacement, pneumatic displacement alone, subretinal tPA injection with pneumatic displacement, and intravitreal anti-vascular endothelial growth factor (VEGF) injection. All procedures obtained consistent results, though the most effective seemed to be pars plana vitrectomy, subretinal tPA and gas tamponade, probably due to a quicker liquefaction and displacement of the clot. Limitations concern the greater invasiveness and the higher incidence of complications. Alternatively, intravitreal injection of tPA and gas may represent a less invasive option with fewer complications. Intravitreal injection of gas and prone position could be preferred in young patients without coexisting ocular pathology, being a minimally invasive treatment, with lower risk of complications and a good visual recovery. Anti-VEGF agent have found, to date, limited employment in cases of traumatic SMH even though they may be useful as alternative or adjuvant therapy. Most of the published literature consists of small studies and case reports, therefore further investigations and larger clinical trials are required to fully understand safety and efficacy of the procedures. A preoperative comprehensive evaluation may be helpful to realize a surgical plan tailored on patient.

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Syphilitic Outer Retinopathy: A Case Report and Review of the Literature

Journal of VitreoRetinal Diseases ◽

10.1177/24741264211018300 ◽

2021 ◽

pp. 247412642110183

Author(s):

Claudia Amaral ◽

Leilani Joy ◽

Hiram Jimenez ◽

Yousef J. Cruz-Inñigo ◽

Jan P. Ulloa-Padilla ◽

...

Keyword(s):

Visual Acuity ◽

Case Report ◽

Vision Loss ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Oral Prednisone ◽

Posterior Uveitis ◽

Central Scotoma ◽

Demarcation Line

Purpose: This work presents a case of syphilitic outer retinopathy with findings similar to those of acute zonal occult outer retinopathy (AZOOR). We also discuss the clinical characteristics, treatment, and prognosis of this entity. Methods: A case report and systematic literature review are presented. Results: A 56-year-old woman presented with acute vision loss, localized photopsia, a central scotoma, and retinal findings that were all consistent with AZOOR. A further workup led to a diagnosis of syphilis. Oral prednisone and intravenous penicillin resulted in the resolution of the posterior uveitis and the restoration of visual acuity. However, the central scotoma remained at the 3-year follow-up visit. Conclusions: Syphilitic outer retinopathy is a distinct entity characterized by the disruption of the ellipsoid zone visible on optical coherence tomography and a corresponding increase in fundus hyperautofluorescence in the affected areas. Although some patients may present with a demarcation line, as is seen with AZOOR, the fundus is oftentimes unremarkable or may show only subtle retinal pigment epithelium changes. Uveitis resolution and visual acuity restoration may be expected following treatment; however, visual field disturbances may persist.

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Retinal Pigment Epithelium Tears After Anti-Vascular Endothelial Growth Factor Therapy for Neovascular Age-Related Macular Degeneration

Ophthalmologica ◽

10.1159/000514991 ◽

2021 ◽

Author(s):

Jayoung Ahn ◽

Daniel Duck-Jin Hwang ◽

Joon Hong Sohn ◽

Gisung Son

Keyword(s):

Vascular Endothelial Growth Factor ◽

Visual Acuity ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Age Related Macular Degeneration ◽

Vascular Endothelial ◽

Anti Vegf ◽

Age Related ◽

Endothelial Growth Factor ◽

Rpe Tear

Purpose: To assess the visual prognostic factors of retinal pigment epithelium (RPE) tears and describe their clinical features. Methods: The medical records of treatment-naive neovascular age-related macular degeneration patients who received intravitreal anti-vascular endothelial growth factor (VEGF) injections were retrospectively reviewed. Results: The incidence of RPE tears was 1.36% (10 out of 733 eyes). The type of anti-VEGF agent administered did not affect the incidence (p = 0.985). The median best-corrected visual acuity (BCVA) of 10 patients decreased after an RPE tear (0.4 to 0.6 logMAR); however, subsequent injections restored the BCVA to a level similar to that before the RPE tear (0.4 logMAR, p = 0.436). Central macular thickness improved significantly during the study (794.4 to 491.9 μm, p = 0.013). The final BCVA was positively correlated with the BCVA before and immediately after the RPE tear (p = 0.025 and 0.002, respectively) and was weakly correlated with foveal involvement of the RPE tear (p = 0.061). Conclusion: The incidence of RPE tears did not differ according to the type of anti-VEGF agent. The final BCVA was proportional to the BCVA before and after RPE tears. Continuous treatment with anti-VEGF after the occurrence of RPE tears can benefit the final visual acuity and macular anatomy.

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Fluorescein Leakage within Recent Subretinal Hemorrhage in Pathologic Myopia: Suggestive of CNV?

Journal of Ophthalmology ◽

10.1155/2018/4707832 ◽

2018 ◽

Vol 2018 ◽

pp. 1-7 ◽

Cited By ~ 3

Author(s):

Lan Mi ◽

Chengguo Zuo ◽

Xiongze Zhang ◽

Bing Liu ◽

Yuting Peng ◽

...

Keyword(s):

Optical Coherence Tomography ◽

Multimodal Imaging ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Pathologic Myopia ◽

Subretinal Hemorrhage ◽

Optical Coherence ◽

Sd Oct ◽

Myopic Cnv

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.

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Multimodal imaging in retinal pigment epithelium rip with exudative bullous retinal detachment

BMJ Case Reports ◽

10.1136/bcr-2020-235731 ◽

2020 ◽

Vol 13 (9) ◽

pp. e235731

Author(s):

Padmaja Kumari Rani ◽

Aniruddh Soni

Keyword(s):

Retinal Pigment Epithelium ◽

Retinal Detachment ◽

Multimodal Imaging ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Subretinal Fluid ◽

Exudative Retinal Detachment ◽

Bullous Retinal Detachment ◽

Subretinal Fluid Drainage

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.

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Long-Term Follow-Up of Congenital Simple Hamartoma of the Retinal Pigment Epithelium: A Case Report

Case Reports in Ophthalmology ◽

10.1159/000487631 ◽

2018 ◽

Vol 9 (1) ◽

pp. 215-220 ◽

Cited By ~ 2

Author(s):

Yuka Ito ◽

Masahito Ohji

Keyword(s):

Visual Acuity ◽

Retinal Pigment Epithelium ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Japanese Woman ◽

Initial Examination ◽

Macular Lesion ◽

Long Term Follow Up

Background: Congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is a rare benign tumor of the retinal pigment epithelium characterized by a focal, darkly pigmented nodule in the macular lesion in healthy persons. We report a case of CSHRPE with long-term follow-up. Case: A 41-year-old Japanese woman was incidentally discovered to have a dark lesion on the fundus of the left eye. We evaluated the patient by measuring her best-corrected visual acuity (BCVA) and by slit-lamp biomicroscopy, fundus color photography, and optical coherence tomography (OCT) over a 10-year period. The BCVA gradually declined during the early follow-up period, having decreased from 1.2 to 0.8 in the left eye 3 years after the initial examination, and then has been maintained for the following 7 years. The lesion did not show a change in OCT 10 years after the first examination. Conclusion: It is important to follow a CSHRPE carefully over the long term because visual acuity might decrease.

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Fundus Autofluorescence Change as an Early Indicator of Treatment Effect of Brachytherapy for Choroidal Melanomas

Ocular Oncology and Pathology ◽

10.1159/000499403 ◽

2019 ◽

Vol 5 (6) ◽

pp. 445-453 ◽

Cited By ~ 1

Author(s):

Jesintha Navaratnam ◽

Thomas P. Bærland ◽

Nils A. Eide ◽

Rowan T. Faber ◽

Bernt L. Rekstad ◽

...

Keyword(s):

Retrospective Study ◽

Retinal Pigment Epithelium ◽

Pigment Epithelium ◽

Fundus Autofluorescence ◽

Retinal Pigment ◽

Choroidal Melanoma ◽

Choroidal Melanomas ◽

Rpe Atrophy ◽

Time Point

Background: Early confirmation of the effect of brachytherapy for choroidal melanoma showing that tumour coverage is valuable. The irradiated retinal pigment epithelium (RPE) commonly develops atrophy. This study compares the fundus autofluorescence (AF) changes to the development of RPE atrophy following brachytherapy. Methods: Retrospective study of 19 patients treated with 106Ru and 2 with 125I plaques with either a 3- or 6-month follow-up period. Ultra-widefield (UW) composite colour and AF images were obtained with Optomap 200Tx and interpreted as complete, partial, or no RPE changes and complete or partial hyperautofluorescence, hypoautofluorescence, or isoautofluorescence. Results: At the 3-month follow-up, 9 of 13 patients (69%) (95% confidence interval [CI], 0.389–0.896) treated with 106Ru plaques developed complete homogenous hyperautofluorescence surrounding the tumour, but only 1 of 13 (8%) (95% CI, 0.004–0.379) developed complete RPE atrophy at the same time point. Six patients in the 106Ru plaque group had their first follow-up with UW imaging at 6 months. Four of them developed homogenous hyperautofluorescence and none developed complete RPE atrophy around the tumour. The 2 patients treated with 125I did not demonstrate any clear RPE or AF changes. Conclusion: The effect of 106Ru plaque treatment on fundus UW imaging is detected as homogenous and well-demarcated hyperautofluorescence before visible RPE atrophy.

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