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1.
Artigo em Inglês | MEDLINE | ID: mdl-31568221

RESUMO

BACKGROUND/PURPOSE: To determine which retinal layer is primarily involved in intraretinal vascular processes associated with Toxoplasma retinochoroiditis using multimodal imaging, including optical coherence tomography angiography (OCTA). METHODS: Toxoplasma retinal lesions were analyzed through multimodal imaging, including color fundus photographs, fluorescein angiography, indocyanine green angiography, spectral-domain OCT, and OCTA. RESULTS: Two patients with atypical features of Toxoplasma retinochoroiditis are described in the acute phase. The first patient presented with a primary episode of acute Toxoplasma retinitis associated with an intraretinal abnormal vascular process that was detected at the acute phase by indocyanine green angiography and was better delineated by OCTA. Seven months later, the intraretinal vascular process had fully resolved on OCTA imaging. The second patient presented with a recurrence of active Toxoplasma retinochoroiditis adjacent to a pre-existing pigmented scar. He had a similar abnormal intraretinal vascular process that was identified on both fluorescein angiography at the early phase and indocyanine green angiography, which was also confirmed by OCTA imaging. CONCLUSION: We report a new finding associated with Toxoplasma chorioretinitis: an abnormal intraretinal vascular process that resolved without anti-vascular endothelial growth factor therapy but after toxoplasmosis treatment alone.

2.
Am J Ophthalmol ; 2019 Aug 26.
Artigo em Inglês | MEDLINE | ID: mdl-31465755

RESUMO

PURPOSE: To describe outer retinal structure in Best vitelliform macular dystrophy (BVMD) patients using spectral-domain optical coherence tomography (SD-OCT) and correlate these RESULTS with best corrected visual acuity (BCVA) and patient age. DESIGN: Retrospective cross-sectional study. METHODS: Patients with molecularly confirmed BVMD were compared to normal control subjects (NC). Complete clinical evaluation was performed including BCVA, fundus photography, SD-OCT and fundus auto-fluorescence. SD-OCT images were analyzed to determine the stage of the lesion, the central macular thickness (CMT), the foveal outer nuclear layer (ONL) thickness, and tomographic structural changes. RESULTS: Forty-two BVMD patients (42 eyes) with a molecular diagnosis and 42 NC (42 eyes) were included. Clinical stages (Gass clinical classification) were distributed as follow: 4.8% for stage 1, 23.8% for stage 2, 16.6% for stage 3, 45.2% for stage 4 and 9.5% for stage 5. The presence of subretinal fluid and vitelliform material was noted in 76% and 79% of the BVMD eyes examined, and was not associated with BCVA modification (P=0.758 and P=0.968, respectively). The median ONL thickness was significantly lower compared to the control group (P<0.001). BCVA was significantly correlated with stage (R=0.710; P<0.01), age (R=0.448; P<0.01), CMT (R=-0.411; P<0.01) and ONL thickness (R=-0.620; P<0.01). The disruption of the external limiting membrane and the ellipsoid zone was associated with a decreased BCVA (P<0.001 for both). Among the 32 eyes with subretinal detachment, the photoreceptor outer segment (PROS) length was significantly correlated with BCVA (R=-0.467; P<0.01) and ONL thickness (R=0.444; P= P<0.01). CONCLUSION: This study demonstrates the correlation between BCVA, age and SD-OCT features in BVMD patients. The ONL thickness as well as PROS length are relevant functional correlates and outcome measures to follow photoreceptor impairments and disease progression.

5.
Ophthalmology ; 126(4): 576-588, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30659849

RESUMO

PURPOSE: To evaluate the long-term visual outcomes and causes of vision loss in chronic central serous chorioretinopathy (CSC). DESIGN: Retrospective, longitudinal study. PARTICIPANTS: A total of 133 participants (217 eyes) with chronic CSC. METHODS: A retrospective review of clinical and multimodal imaging data of patients with chronic CSC managed by 3 of the authors between May 1977 and March 2018. Multimodal imaging comprised color photography, fluorescein angiography, indocyanine green angiography, fundus autofluorescence (FAF), and OCT. MAIN OUTCOME MEASURES: Best-corrected visual acuity (BCVA) at the final visit; change in BCVA between first visit and 1-, 5-, and 10-year follow-up visits; and causes of vision loss at final visit. RESULTS: Data from 6228 individual clinic visits were analyzed. Mean age of patients at the first visit was 60.7 years, and mean period of follow-up from first to last visit was 11.3 years. The cohort included 101 male patients (75.9%). At the final visit, 106 patients (79.7%) maintained driving-standard vision with BCVA of 20/40 or better in at least 1 eye, and 17 patients (12.8%) were legally blind with BCVA of 20/200 or worse in both eyes. Mean BCVA at first visit was not significantly different from mean BCVA at 1- or 5-year follow-up visits (both P ≥ 0.65) but was significantly better than the mean BCVA at the 10-year follow-up visit (P = 0.04). Seventy-nine percent of eyes with 20/40 or better vision at the first visit maintained the same level of vision at the 10-year follow-up visit. Ninety-two percent of eyes with 20/200 or worse vision at the first visit maintained the same level of vision at the 10-year follow-up visit. Cystoid macular degeneration, choroidal neovascularization (CNV), outer retinal disruption on OCT, and FAF changes were associated with poorer vision at the final visit (all P ≤ 0.001). Multivariable analysis revealed that greater age at first visit was associated with greater BCVA change at the 10-year follow-up visit (P = 0.001). CONCLUSIONS: Chronic CSC can be a sight-threatening disease leading to legal blindness. Age at presentation and outer retinal changes on multimodal imaging were associated with long-term BCVA changes and may be predictors of long-term visual outcomes.

6.
Graefes Arch Clin Exp Ophthalmol ; 257(3): 485-493, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30535969

RESUMO

PURPOSE: To investigate morphological changes in myopic choroidal neovascularization (mCNV) using optical coherence tomography-angiography (OCT-A) after treatment with ranibizumab. METHODS: Retrospective analysis of consecutive patients over a 24-month period. All treatment-naïve mCNV were imaged at baseline with color pictures, spectral-domain OCT and OCT-A, and fluorescein angiography in selected cases. CNV morphology was classified at baseline and at 6 months. The CNV lesion surface was also compared. RESULTS: Twenty-nine patients with a mean age of 70.3 ± 10.1 years were included. They received a mean number of 2.65 injections over 6 months. Best-corrected visual acuity improved from 62.2 to 68.5 letters (p = 0.004), with regression of exudation in 24 eyes (82.7%). Baseline CNV was classified into tree-in-bud (16 eyes), medusa (9 eyes), or sea-fan (4 eyes) pattern. At 6 months, no abnormal blood flow was observed in CNV in 13 eyes. Eyes with complete regression or evolution towards an indistinct pattern showed more often a complete regression of exudation than eyes with unchanged pattern (p = 0.007). The mean CNV surface significantly decreased from 0.19 to 0.08 mm2 (p < 0.0001). CONCLUSION: An unchanged pattern was more often associated with exudation persistence, while a complete regression or evolution towards indistinct pattern was always associated with vascular inactivity. However, variable changes in mCNV were observed after anti-VEGF. Thus, OCT-A could be more useful in the diagnosis than in the follow-up of mCNV.


Assuntos
Neovascularização de Coroide/tratamento farmacológico , Miopia/complicações , Ranibizumab/administração & dosagem , Fluxo Sanguíneo Regional/fisiologia , Vasos Retinianos/fisiopatologia , Remodelação Vascular/fisiologia , Idoso , Inibidores da Angiogênese , Corioide/irrigação sanguínea , Neovascularização de Coroide/diagnóstico , Neovascularização de Coroide/etiologia , Progressão da Doença , Relação Dose-Resposta a Droga , Feminino , Angiofluoresceinografia/métodos , Seguimentos , Fundo de Olho , Humanos , Injeções Intravítreas , Masculino , Miopia/fisiopatologia , Refração Ocular , Fluxo Sanguíneo Regional/efeitos dos fármacos , Vasos Retinianos/diagnóstico por imagem , Estudos Retrospectivos , Tomografia de Coerência Óptica/métodos , Resultado do Tratamento , Fator A de Crescimento do Endotélio Vascular/antagonistas & inibidores
7.
Am J Ophthalmol ; 2018 Oct 08.
Artigo em Inglês | MEDLINE | ID: mdl-30308202

RESUMO

PURPOSE: To assess the efficacy and safety of mineralocorticoid receptor antagonists (MRA) in the treatment of non-resolving central serous chorioretinopathy (CSC) and identify predictive factors of treatment response. DESIGN: Retrospective, multicenter, non-comparative, interventional case series. METHODS: Clinical and imaging data from consecutive patients with non-resolving CSC treated with eplerenone or spironolactone for 3-6 months between 2012 and 2016 were reviewed. Outcome measurements were: the resolution of foveal subretinal detachment (SRD), changes in SRD height, central macular thickness (CMT), subfoveal choroidal thickness (SFCT), best-corrected visual acuity (BCVA) and the occurrence of adverse events assessed at 3 and 6 months. The response to treatment was defined by a decrease by more than 50% in SRD height under treatment. Comparisons between responder and non-responder groups were performed using univariate and multivariate regression analyzes to identify predictive factors of treatment response. RESULTS: Fifty-nine patients (64 eyes) were included. The mean SRD height and CMT significantly decreased while the mean BCVA significantly improved at 3 and 6 months. The mean SFCT significantly decreased at 3 months. Among the 64 eyes included, 67.2% responded to treatment among which 38.3% and 40.5% had a complete resolution of the foveal SRD at 3 and 6 months, respectively. The baseline SFCT was the only factor associated with a treatment response in the multivariate analysis. CONCLUSION: Our study suggests that MRA could be a safe and effective treatment in non-resolving CSC patients. MRA treatment is more effective in cases with a thicker baseline choroid.

8.
Retin Cases Brief Rep ; 12(4): 331-335, 2018 Fall.
Artigo em Inglês | MEDLINE | ID: mdl-30226832

RESUMO

PURPOSE: To report the posterior segment and retinal vascular manifestations of calcific uremic arteriolopathy (calciphylaxis). Clinical findings are correlated with multimodal imaging results. METHODS: Observational case report. RESULTS: A 65-year-old white woman on hemodialysis was referred for assessment of poor vision bilaterally. Clinical examination demonstrated a crystalline retinopathy with stigma of previous retinal arterial occlusion. Fluorescein angiography revealed delayed retinal arterial filling bilaterally, sheathing of vessels, and peripheral nonperfusion. The crystals were hyperautofluorescent. Spectral domain and enhanced depth imaging optical coherence tomography localized the crystals within the retina with a predilection for the retinal arterial vasculature. The choriocapillaris was not involved. Two years prior, the patient developed necrotic skin lesions which were biopsied and confirmed the diagnosis of calciphylaxis. CONCLUSION: Calcific uremic arteriolopathy is an extremely rare cause of thrombogenic microangiopathy in end-stage renal disease patients. Retinal arterial occlusion appears to be a rare but significant cause of visual loss in this disease and is likely to be consequent to crystalline deposition in the retinal vasculature.


Assuntos
Calciofilaxia/complicações , Doenças Retinianas/etiologia , Idoso , Cristalinas/metabolismo , Feminino , Humanos , Falência Renal Crônica/complicações
9.
Artigo em Inglês | MEDLINE | ID: mdl-30181904

RESUMO

Purpose: To report the clinical course and multimodal imaging features of acute zonal occult outer retinopathy (AZOOR) complicated by choroidal neovascularization (CNV) treated with anti-vascular endothelial growth factor (VEGF) treatment or photodynamic therapy (PDT). Methods: Observational case series. Retrospective analysis of patients presenting to different institutions with evidence of AZOOR and neovascular lesions. Diagnosis of AZOOR was made on the basis of clinical presentation and multimodal imaging. All patients underwent a comprehensive ophthalmic evaluation and multimodal retinal imaging, including color fundus photos, fundus autofluorescence, fundus fluorescein angiography and spectral-domain optical coherence tomography. Results: Four patients (three males, mean age 53.5 years) were included in the study. Mean follow-up was 5.1 years. Presentation of AZOOR was unilateral in two patients and bilateral in the remainder two patients. One of the patients presenting with unilateral AZOOR developed zonal lesions in the fellow eye during follow-up. All patients presented with unilateral type 2 (subretinal) CNV. Three patients underwent intravitreal anti-VEGF injections and one patient underwent a single PDT. Multimodal retinal imaging showed zonal or multizonal progression during treatment. After treatment, visual acuity and CNV stabilization was observed in all patients. Conclusions: The presence of CNV expands the clinical spectrum of AZOOR. CNV complicating AZOOR may be effectively treated with intravitreal injections of anti-VEGF, despite progression of the zonal lesions. Further studies are required to define the role of treatment in the progression of the zonal lesions.

10.
Prog Retin Eye Res ; 66: 1-16, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30010022

RESUMO

Adaptive optics (AO)-enhanced en face retinal imaging, termed here AO ophthalmoscopy (AOO) has reached a level of robustness which fuels its increasing use in research and clinical centers. Here we will review the contribution of clinical AOO to the understanding and monitoring of 1) age-related macular degeneration and 2) vascular diseases. The main contributions of AOO to the phenotyping of AMD are a better identification of drusen, a better delineation of the limits of atrophy, and the identification of novel features such as punctate hyperreflectivity and mobile melanin-containing clumps. Characterization of progression of atrophy is facilitated by time-lapse imaging. In vessels, AOO enables the observation and measurement of parietal structures and the observation of microscopic pathological features such as small hemorrhages and inflammatory cell accumulations.

11.
Retina ; 2018 Jul 13.
Artigo em Inglês | MEDLINE | ID: mdl-30015765

RESUMO

PURPOSE: To evaluate the ability of optical coherence tomography angiography (OCTA) to detect abnormal vascular blood flow in Type 1 neovascularization (NV) with or without significant pigment epithelial detachment (PED). METHODS: Consecutive age-related macular degeneration patients with either treatment-naive or anti-vascular endothelial growth factor-treated Type 1 NV were divided into 2 groups based on the PED height on structural OCT: greater than 250 µm (Group 1) versus less than 250 µm (Group 2). Two independent senior retina specialists analyzed the OCTA images (Zeiss Angioplex OCT, Carl Zeiss AG, Jena, Germany) using the automatic slabs alone (first reader) versus automatic and manual segmentation slabs (second reader). RESULTS: In Group 1, 15 men and 42 women, aged from 51 years to 97 years (mean: 87.5), were included. Optical coherence tomography angiography was able to show an abnormal blood flow suggestive of Type 1 NV in 23 (40.3%) of 57 eyes for the first reader and in 32 (56.1%) of 57 eyes for the second reader. In Group 2, 7 men and 30 women, aged from 60 years to 96 years (mean: 80.2), were included. The first and second readers were able to observe an image suggestive of Type 1 NV in 33/37 (89.2%) and 37/37 (100%) of eyes, respectively. CONCLUSION: The ability of OCTA to detect an abnormal blood flow in Type 1 NV was found to highly depend on the height of the associated PED and the use of manual segmentation slabs. Our results suggest that automatic slabs of OCTA should be interpreted with caution for the diagnosis of vascularized PED. The diagnosis of Type 1 NV using OCTA requires the use of manual segmentation and a multimodal imaging approach, especially when the height of the associated PED is >250 µm.

12.
Eur J Ophthalmol ; 28(5): 491-502, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29554812

RESUMO

A review of the literature from 2014 to 2016 was conducted, focusing on the results of optical coherence tomography angiography in different chorioretinal diseases. In only 3 years, optical coherence tomography angiography has been shown to be an effective tool for diagnosing choroidal neovascularization complicating age-related macular degeneration, pathologic myopia, and inflammatory conditions. The technique has sometimes been considered superior to conventional multimodal imaging, for example, in choroidal neovascularization associated with chronic central serous chorioretinopathy or multifocal choroiditis. In retinal vascular diseases, optical coherence tomography angiography has helped to understand the condition described as paracentral acute middle maculopathy and has been considered highly effective for the analysis of retinal vascular macular changes secondary to retinal vein occlusion or macular telangiectasia. Changes in the foveal avascular zone, also reported in diabetic maculopathy, have been shown to occur before any angiographic signs. A reduction in capillary vascular density has been reported in the fovea of eyes with malignant melanoma, but not in eyes with choroidal nevus. However, optical coherence tomography angiography is a recent technique that probably needs refinements and further studies. Nevertheless, the first 3 years of optical coherence tomography angiography use suggest its clinical relevance and useful applications in daily clinical practice.


Assuntos
Doenças da Coroide/diagnóstico , Angiofluoresceinografia/métodos , Doenças Retinianas/diagnóstico , Tomografia de Coerência Óptica/métodos , Humanos
13.
Retin Cases Brief Rep ; 12 Suppl 1: S19-S24, 2018 Fall.
Artigo em Inglês | MEDLINE | ID: mdl-29369084

RESUMO

PURPOSE: To document directional reflectivity of fundus lesions in a case of acute macular neuroretinopathy. METHODS: Case report. Clinical and imaging data from a patient with acute macular neuroretinopathy were reviewed. Imaging comprised infrared scanning laser ophthalmoscopy, optical coherence tomography and flood-illumination adaptive optics images acquired through different entry pupils in the cardinal directions (approximately 2° eccentricity). RESULTS: The patient reported acute bilateral paracentral scotoma revealing dark, wedge-shaped macular plaques which by optical coherence tomography were associated with focal loss of the visibility of the cone outer segment tip and inner/outer segment lines. Comparing scanning laser ophthalmoscopy images taken at different entry points in the pupil showed that macular plaques varied from hyporeflectance to isoreflectance. Cone counts by flood-illumination adaptive optics within plaques and optical coherence tomography features of the cone outer segment tip showed also a strong directional variability, peaking at near-normal values. Within each modality, fusion images showed that directional variability covered most of macular plaques. CONCLUSION: The characteristic fundus abnormalities of acute macular neuroretinopathy may show a strong directional variability. Our findings suggest that the Stiles-Crawford effect may be an important factor in signs and symptoms of acute macular neuroretinopathy.


Assuntos
Macula Lutea/patologia , Doenças Retinianas/patologia , Adolescente , Feminino , Humanos , Oftalmoscopia/métodos , Escotoma/diagnóstico , Tomografia de Coerência Óptica/métodos
14.
Retina ; 38(3): 629-638, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28267114

RESUMO

PURPOSE: To assess the rate of choroidal neovascularization (CNV) detected by optical coherence tomography angiography (OCTA) in flat irregular pigment epithelium detachment (PED) in chronic central serous chorioretinopathy. METHODS: Data on all consecutive patients with chronic central serous chorioretinopathy who underwent OCTA over a 1-year period were reviewed. The presence of flat irregular PED, which was defined as an irregular elevation of the retinal pigment epithelium allowing the visualization of a distinct Bruch's membrane was assessed on high-resolution OCT B-scan. Clinical, multimodal imaging, and OCTA data were reviewed by two graders for the detection of CNV. RESULTS: Eighty-eight eyes of 61 patients with chronic central serous chorioretinopathy were included. Patient mean age (±SD) was 54.5 ± 12.2 years, and 78.7% were males. Mean subfoveal choroidal thickness (±SD) was 452.6 ± 145.6 µm. Flat irregular PEDs were detected in 59 eyes of 51 patients. OCTA detected the presence of CNV in flat irregular PEDs in 35.6% of cases. Conversely, using the combination of spectral domain optical coherence tomography angiography, fluorescein and indocyanine green angiography, CNV was detected in only 25% of flat irregular PEDs. All hyporeflective flat irregular PEDs on OCT were avascular on OCTA while they were at least partially hyperreflective when associated with CNV. CONCLUSION: One-third of flat irregular PEDs in chronic central serous chorioretinopathy contained CNV. OCTA detected CNV more frequently than the other imaging modalities. Further longitudinal studies are needed to assess the indication of antivascular endothelial growth factor treatments in such cases.


Assuntos
Coriorretinopatia Serosa Central/diagnóstico , Angiofluoresceinografia , Descolamento Retiniano/diagnóstico , Epitélio Pigmentado da Retina/patologia , Tomografia de Coerência Óptica/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Imagem Multimodal
15.
Retina ; 38(2): 299-309, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-28368976

RESUMO

PURPOSE: To characterize the macular lesions in multifocal choroiditis using multimodal imaging (MMI) and to evaluate optical coherence tomography angiography (OCTA) in distinguishing neovascular from inflammatory lesions. METHODS: Retrospective review of medical records of consecutive patients diagnosed with multifocal choroiditis and macular involvement, between September 2014 and May 2016, were included. All patients underwent standard examination and MMI, including fundus color photography, fundus autofluorescence, fluorescein angiography, and spectral domain optical coherence tomography. They also underwent OCTA examination. Multimodal imaging and OCTA characteristics of inflammatory lesions and choroidal neovascularization (CNV) were compared. RESULTS: Eighteen eyes of 13 patients (11 females) were analyzed. The mean age was 42.9 ± 13.4 years. The lesions were first categorized as active or inactive CNV and active or inactive inflammatory lesions through conventional MMI. Using OCTA, an abnormal blood flow was observed in all active CNV (9/9) and most inactive CNV (5/6), but also in 2 of 14 lesions previously classified as active inflammatory lesions. On the contrary, no case of inactive inflammatory lesions showed abnormal blood flow. Therefore, the use of OCTA allowed a diagnosis of CNV that was not made through conventional MMI in 14% of cases of active inflammatory lesions. CONCLUSION: The combined findings of conventional imaging and OCTA demonstrate distinctive features of inflammatory lesions and CNV in multifocal choroiditis, allowing an appropriate management of these sight-threatening lesions. However, OCTA alone did not distinguish between active and inactive CNVs and should be integrated into an MMI approach.


Assuntos
Corioide/patologia , Neovascularização de Coroide/diagnóstico , Corioidite/diagnóstico , Angiofluoresceinografia/métodos , Macula Lutea/patologia , Tomografia de Coerência Óptica/métodos , Adulto , Corioide/irrigação sanguínea , Neovascularização de Coroide/fisiopatologia , Diagnóstico Diferencial , Feminino , Seguimentos , Fundo de Olho , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Acuidade Visual , Adulto Jovem
16.
Ophthalmology ; 125(1): 100-118, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-28964580

RESUMO

PURPOSE: To define the range and life cycles of cuticular drusen phenotypes using multimodal imaging and to review the histologic characteristics of cuticular drusen. DESIGN: Retrospective, observational cohort study and experimental laboratory study. PARTICIPANTS: Two hundred forty eyes of 120 clinic patients with a cuticular drusen phenotype and 4 human donor eyes with cuticular drusen (n = 2), soft drusen (n = 1), and hard drusen (n = 1). METHODS: We performed a retrospective review of clinical and multimodal imaging data of patients with a cuticular drusen phenotype. Patients had undergone imaging with various combinations of color photography, fluorescein angiography, indocyanine green angiography, near-infrared reflectance, fundus autofluorescence, high-resolution OCT, and ultrawide-field imaging. Human donor eyes underwent processing for high-resolution light and electron microscopy. MAIN OUTCOME MEASURES: Appearance of cuticular drusen in multimodal imaging and the topography of a cuticular drusen distribution; age-dependent variations in cuticular drusen phenotypes, including the occurrence of retinal pigment epithelium (RPE) abnormalities, choroidal neovascularization, acquired vitelliform lesions (AVLs), and geographic atrophy (GA); and ultrastructural and staining characteristics of druse subtypes. RESULTS: The mean age of patients at the first visit was 57.9±13.4 years. Drusen and RPE changes were seen in the peripheral retina, anterior to the vortex veins, in 21.8% of eyes. Of eyes with more than 5 years of follow-up, cuticular drusen disappeared from view in 58.3% of eyes, drusen coalescence was seen in 70.8% of eyes, and new RPE pigmentary changes developed in 56.2% of eyes. Retinal pigment epithelium abnormalities, AVLs, neovascularization, and GA occurred at a frequency of 47.5%, 24.2%, 12.5%, and 25%, respectively, and were significantly more common in patients older than 60 years of age (all P < 0.015). Occurrence of GA and neovascularization were important determinants of final visual acuity in eyes with the cuticular drusen phenotype (both P < 0.015). Small cuticular drusen typically demonstrated a homogenous ultrastructural appearance similar to hard drusen, whereas fragmentation of the central and basal contents was seen frequently in larger cuticular drusen. CONCLUSIONS: Although the ultrastructural characteristics of cuticular drusen appear more similar to those of hard drusen, their lifecycle and macular complications are more comparable with those of soft drusen. Cuticular drusen phenotype may confer a unique risk for the development of GA and neovascularization.


Assuntos
Lâmina Basilar da Corioide/patologia , Oftalmopatias Hereditárias/diagnóstico , Angiofluoresceinografia , Imagem Multimodal/métodos , Drusas Retinianas/diagnóstico , Epitélio Pigmentado da Retina/patologia , Tomografia de Coerência Óptica , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Fundo de Olho , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo , Estudos Retrospectivos , Adulto Jovem
17.
BMC Ophthalmol ; 17(1): 67, 2017 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-28506260

RESUMO

BACKGROUND: To report patients with age-related macular degeneration and atypical central retinal pigment epithelium (RPE) defects not attributable to geographic atrophy (GA) or RPE-tears with overlying preserved photoreceptor layers. METHODS: Multimodal imaging case-series evaluating the course of atypical RPE- defects in patients with AMD using Color fundus images, Optical coherence tomography (OCT), OCT-Angiography, fundus autofluorescence (FAF) and fluorescein-angiography (FA). RESULTS: Ten patients were identified. Three patients had a prior RPE-rip and were excluded. Seven patients with a mean follow-up period of 47 ± 38 months after the occurrence of the RPE-defect were included (age range 71-87 years). Mean distance Best corrected visual acuity (BCVA) at initial presentation was 0.36 ± 0.29logMAR and at last follow-up visit 0.51 ± 0.43logMAR. Patients presented with clinically apparent GA on funduscopy and FAF, but preserved photoreceptor layers on optical coherence tomography (OCT). On FA there was early hyperfluorescence and late pooling visible. Over time, migration of RPE/drusenoid material right above the Bruch's membrane with concomitant decrease of hypoautofluorescence was detectable in 4 cases. An enlargement of the RPE-defect was apparent in the remaining 3 cases. The majority (n = 4) showed a drusenoid pigment epithelium detachment (PED) preceding the lesion. CONCLUSIONS: Beside GA and characteristic RPE-tears, another atypical form of RPE-defect with overlying preserved photoreceptor layers are found in AMD. This so far disregarded subgroup of patients present with reasonable visual function and long-term survival of photoreceptors layers. Repair mechanisms such as ingrowth of RPE/drusenoid material and persistent subretinal fluid (SRF), but also a RPE-independent visual cycle for cone photopigment within the neurosensory retina may contribute to their favorable course.


Assuntos
Angiofluoresceinografia/métodos , Degeneração Macular/diagnóstico , Células Fotorreceptoras de Vertebrados/patologia , Epitélio Pigmentado da Retina/patologia , Tomografia de Coerência Óptica/métodos , Acuidade Visual , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Fundo de Olho , Humanos , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença
19.
Dev Ophthalmol ; 58: 191-201, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28351048

RESUMO

Retinitis pigmentosa (RP) is a heterogeneous group of inherited retinal degenerations characterized by progressive degeneration of rod and cone cells that affects predominantly peripheral visual fields. Macular edema may cause additional central visual acuity decrease. Cystoid macular edema (CME) is one of the few treatable causes of visual loss in RP. The prevalence of CME in RP has been found to be between 10 and 20% on fluorescein angiography-based studies, and as high as 49% on reports based on optical coherence tomography. Macular edema can manifest at any stage of the disease and may be unilateral or bilateral. It can be found in any genetic form, but is more often associated with RP caused by CRB1 mutations. The origin of macular edema in RP patients still remains poorly understood. Some mechanisms have been suggested, including antiretinal antibodies (retinal, carbonic anhydrase, and enolase antibodies), vitreous traction, retinal pigment epithelium dysfunction, and Müller cell edema. There is no gold standard therapeutic strategy. Drug therapy is the primary treatment. Systemic carbonic anhydrase inhibitors, such as oral acetazolamide or topical dorzolamide, are still the mainstays of initial therapy. If CME is refractory to acetazolamide, intravitreal corticosteroid injections may be a therapeutic option. However, antivascular endothelium growth factor injections have limited effect and should be avoided. Vitrectomy has also been evaluated, but its exact role remains to be determined. The benefits of these therapies are variable among patients. The establishment of therapeutic approaches is limited by our poor understanding of the pathophysiology of CME in patients with RP. Autoimmune retinopathies (AIRs) are a group of rare diseases characterized by acute or subacute progressive vision loss and are thought to be mediated by autoantibodies specific to retinal antigens. The AIRs encompass paraneoplastic syndromes, such as cancer-associated retinopathy and melanoma-associated retinopathy, and a larger group of AIRs that have similar clinical and immunological findings but without underlying malignancy. These diseases may also be complicated by macular edema. RP is one of the most common forms of inherited retinal degeneration. It displays extensive clinical and genetic variations and leads to progressive blindness with variable onset.


Assuntos
Gerenciamento Clínico , Angiofluoresceinografia/métodos , Degeneração Macular/diagnóstico , Retina/patologia , Retinite Pigmentosa/diagnóstico , Tomografia de Coerência Óptica/métodos , Fundo de Olho , Humanos , Degeneração Macular/terapia , Retinite Pigmentosa/terapia
20.
Retina ; 37(11): 2025-2034, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28098736

RESUMO

PURPOSE: To describe atypical cases of multiple evanescent white dot syndrome (MEWDS) associated with foveal exudation, increased choroidal thickness, and secondary Type 2 (subretinal) neovascularization. METHODS: Four cases of atypical MEWDS were studied at a retina referral center. Patients underwent evaluation with multimodal retinal imaging, including fluorescein angiography, indocyanine green angiography, spectral-domain and enhanced depth imaging optical coherence tomography (OCT). Two patients were imaged with OCT angiography. RESULTS: Four patients (3 female, 1 male) with a median age of 23.5 years presented with acute onset, painless, decreased central vision. All cases demonstrated fundus findings consistent with MEWDS on color photography, indocyanine green angiography, fluorescein angiography, fundus autofluorescence, and structural OCT imaging. On structural OCT, all 4 patients were noted to have hyperreflective subretinal material and increased subfoveal choroidal thickness ranging from 307 µm to 515 µm. Type 2 neovascularization was diagnosed in all four patients using fluorescein angiography, indocyanine green angiography, and/or OCT angiography. Two patients had poor visual acuity at the last follow-up despite resolution of characteristic clinical findings of MEWDS. CONCLUSION: A subset of patients with atypical MEWDS may develop persistent poor vision due to subfoveal exudation and secondary Type 2 neovascularization. Patients showing increased choroidal thickness at presentation may be more susceptible to this unusual presentation.


Assuntos
Neovascularização de Coroide/diagnóstico , Angiofluoresceinografia/métodos , Fóvea Central/patologia , Líquido Sub-Retiniano/diagnóstico por imagem , Tomografia de Coerência Óptica/métodos , Adolescente , Adulto , Feminino , Seguimentos , Fundo de Olho , Humanos , Masculino , Síndrome , Adulto Jovem
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