Spontaneous Regression of Choroidal Neovascularization in a Patient with Pattern Dystrophy

Purpose. To present a case of a patient with pattern dystrophy (PD) associated choroidal neovascularization (CNV) that resolved spontaneously without treatment.Methods. A 69-year-old male patient was referred to our unit, for evaluation of a recent visual loss (metamorphopsias) in his left eye. Fundus examination, fundus autofluorescence imaging, and fluorescein angiography showed a choroidal neovascular membrane in his left eye. Since visual acuity was satisfactory the patient elected observation. Clinical examination and OCT testing were repeated at 6 and 12 months after presentation.Results. Visual acuity remained stable at the level of 0.9 (baseline BCVA) during the follow-up period (12 months). Repeat OCT testing showed complete spontaneous regression of the choroidal neovascular membrane without evidence of intra- or subretinal fluid in both follow-up visits.Conclusions. Spontaneous regression of choroidal neovascularization can occur in patients with retinal dystrophies and associated choroidal neovascular membranes. The decision to treat or observe these patients relies strongly on the presenting visual acuity, since, in isolated instances, spontaneous resolution of choroidal neovascularization may occur.

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Pattern Dystrophies and Choroidal Neovascularization

Güncel Retina Dergisi (Current Retina Journal) ◽

10.37783/crj-0053 ◽

2017 ◽

pp. 313-320

Keyword(s):

Visual Acuity ◽

Choroidal Neovascularization ◽

Middle Ages ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Subretinal Fluid ◽

Age Related Macular Degeneration ◽

Pattern Dystrophy ◽

Good Visual Acuity ◽

Age Related

Pattern dystrophies are hereditary dystrophies that come from retinal pigment epithelium and located in the macula. The diagnosis is usually made around the middle ages. Pigment accumulations in the macula and around the macula, are seen bilaterally and symmetrical in the beginning, are sub-divided depending on pigment scattering pattern. These are adult-onset foveomacular vitelliform dystrophy, butterfly-like pattern dystrophy, reticular pattern dystrophy, and fundus pulverulentus. Usually in patients with pattern dystrophy have good visual acuity, after progression central visual acuity lessens with RPE atrophy or choroidal neovascularization development. In choroidal neovascularization secondary to age-related macular degeneration there is no typical pigmentation in the macula. On the other hand, in choroidal neovascularization related to pattern dystrophy, hemorrhage, and intra/subretinal fluid do not exist. Today choroidal neovascularization prognosis, which is treated with intravitreal anti-VEGF injections, is quite good. At least, one eye of the patients has satisfactory vision. Sometimes, in especially patients with isolated pattern dystrophy, not hereditary, there may be spontaneous regression of choroidal neovascularization.

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Treatment of Punctate Inner Choroidopathy with Choroidal Neovascularization Using Corticosteroid and Intravitreal Ranibizumab

BioMed Research International ◽

10.1155/2018/1585803 ◽

2018 ◽

Vol 2018 ◽

pp. 1-7 ◽

Cited By ~ 1

Author(s):

Wei Wu ◽

Shiying Li ◽

Haiwei Xu ◽

Yong Liu ◽

Yi Wang ◽

...

Keyword(s):

Visual Acuity ◽

Choroidal Neovascularization ◽

Oral Corticosteroid ◽

Fundus Autofluorescence ◽

Intravitreal Ranibizumab ◽

Punctate Inner Choroidopathy ◽

Corrected Visual Acuity ◽

Before And After ◽

Ranibizumab Treatment

Background. To evaluate the treatment outcomes of patients with punctate inner choroidopathy (PIC) and secondary choroidal neovascularization (CNV). Methods. This is a retrospective study of 24 eyes in 22 patients suffering from PIC with CNV. Patients were treated with intravitreal ranibizumab monotherapy (14 eyes) or combined oral corticosteroid and intravitreal ranibizumab therapy (corticosteroid-ranibizumab group, 10 eyes). Mean follow-up duration was 24.0 months. We evaluated best-corrected visual acuity (BCVA), fundus autofluorescence, fluorescein angiography, indocyanine green angiography, and optical coherence tomography, before and after treatment. The following variables were compared between groups: number of intravitreal ranibizumab injections, BCVA, recurrence of CNV, and change in PIC lesions. Results. The ranibizumab monotherapy group received an average of 3 intravitreal ranibizumab injections; mean logMAR visual acuity improvement was 0.34, and 8 eyes developed recurrent CNV during follow-up. The corticosteroid-ranibizumab group received an average of 1.9 intravitreal ranibizumab injections; mean logMAR visual acuity improvement was 0.61, and there was no recurrence of CNV. Combined corticosteroid-ranibizumab therapy also resulted in better resolution of PIC lesions and fewer new PIC lesions. Conclusion. Both corticosteroid-ranibizumab treatment and ranibizumab monotherapy could significantly improve the vision of PIC patients with CNV. Combined corticosteroid and intravitreal ranibizumab treatment appeared to reduce CNV recurrence and development of new PIC lesions compared with ranibizumab monotherapy.

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Clinical Phenotype of PDE6B-Associated Retinitis Pigmentosa

International Journal of Molecular Sciences ◽

10.3390/ijms22052374 ◽

2021 ◽

Vol 22 (5) ◽

pp. 2374

Author(s):

Laura Kuehlewein ◽

Ditta Zobor ◽

Katarina Stingl ◽

Melanie Kempf ◽

Fadi Nasser ◽

...

Keyword(s):

Visual Acuity ◽

Genetic Testing ◽

Retinitis Pigmentosa ◽

Fundus Autofluorescence ◽

Retinal Disease ◽

New Drugs ◽

Autofluorescence Imaging ◽

Full Field ◽

Tertiary Referral Center ◽

The Right

In this retrospective, longitudinal, observational cohort study, we investigated the phenotypic and genotypic features of retinitis pigmentosa associated with variants in the PDE6B gene. Patients underwent clinical examination and genetic testing at a single tertiary referral center, including best-corrected visual acuity (BCVA), kinetic visual field (VF), full-field electroretinography, full-field stimulus threshold, spectral domain optical coherence tomography, and fundus autofluorescence imaging. The genetic testing comprised candidate gene sequencing, inherited retinal disease gene panel sequencing, whole-genome sequencing, and testing for familial variants by Sanger sequencing. Twenty-four patients with mutations in PDE6B from 21 families were included in the study (mean age at the first visit: 32.1 ± 13.5 years). The majority of variants were putative splicing defects (8/23) and missense (7/23) mutations. Seventy-nine percent (38/48) of eyes had no visual acuity impairment at the first visit. Visual acuity impairment was mild in 4% (2/48), moderate in 13% (6/48), and severe in 4% (2/48). BCVA was symmetrical in the right and left eyes. The kinetic VF measurements were highly symmetrical in the right and left eyes, as was the horizontal ellipsoid zone (EZ) width. Regarding the genetic findings, 43% of the PDE6B variants found in our patients were novel. Thus, this study contributed substantially to the PDE6B mutation spectrum. The visual acuity impairment was mild in 83% of eyes, providing a window of opportunity for investigational new drugs. The EZ width was reduced in all patients and was highly symmetric between the eyes, making it a promising outcome measure. We expect these findings to have implications on the design of future PDE6B-related retinitis pigmentosa (RP) clinical trials.

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Retinal Vasculitis Associated With Dermatomyositis Treated With Intravenous Steroids

Journal of VitreoRetinal Diseases ◽

10.1177/24741264211018960 ◽

2021 ◽

pp. 247412642110189

Author(s):

Austen N. Knapp ◽

Jawad I. Arshad ◽

Daniel F. Martin ◽

Rula Hajj-Ali ◽

Kimberly Baynes ◽

...

Keyword(s):

Visual Acuity ◽

Macular Edema ◽

Retinal Vasculitis ◽

Subretinal Fluid ◽

Retrospective Case ◽

Hand Motion ◽

Frosted Branch Angiitis ◽

Intravenous Steroids

Purpose: This work aims to present treatment and long-term follow-up of a 31-year-old woman with dermatomyositis who presented with hemorrhagic retinal vasculitis and macular edema. Methods: A retrospective case report is presented. Results: A 31-year-old woman with dermatomyositis treated with systemic immunosuppression was evaluated for acute, reduced vision. Best-corrected visual acuity was hand motion in the right eye and 20/200 in the left eye. Fundus examination revealed diffuse intraretinal hemorrhages, cotton-wool spots, and vascular sheathing with a frosted branch angiitis–like appearance. Optical coherence tomography revealed significant macular edema and subretinal fluid that quickly resolved after admission and treatment with intravenous steroids. Multimodal imaging at 7-year follow-up disclosed long-term sequelae including peripheral nonperfusion and retinal neovascularization. Conclusions: When vasculitis associated with dermatomyositis is treated aggressively with intravenous steroids at initial presentation, good visual acuity outcomes can be achieved, but long-term consequences of retinal nonperfusion and neovascularization persist.

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Genotypes Predispose Phenotypes—Clinical Features and Genetic Spectrum of ABCA4-Associated Retinal Dystrophies

Genes ◽

10.3390/genes11121421 ◽

2020 ◽

Vol 11 (12) ◽

pp. 1421

Author(s):

Yu-Chi Sung ◽

Chang-Hao Yang ◽

Chung-May Yang ◽

Chao-Wen Lin ◽

Ding-Siang Huang ◽

...

Keyword(s):

Visual Acuity ◽

Retinal Degeneration ◽

Medical Center ◽

Fundus Autofluorescence ◽

Genetic Diagnosis ◽

Common Disease ◽

Genetic Characteristics ◽

Retinal Dystrophies ◽

High Prevalence ◽

Abca4 Gene

The ABCA4 gene is one of the most common disease-causing genes of inherited retinal degeneration. In this study, we report different phenotypes of ABCA4-associated retinal dystrophies in the Taiwanese population, its clinical progression, and its relationship with genetic characteristics. Thirty-seven subjects were recruited and all patients underwent serial ophthalmic examinations at a single medical center. Fundus autofluorescence (FAF) images were quantified for clinical evaluation, and panel-based next-generation sequencing testing was performed for genetic diagnosis. Visual preservation, disease progression, and genotype–phenotype correlation were analyzed. In this cohort, ABCA4-associated retinal degeneration presented as Stargardt disease 1 (STGD1, 62.16%), retinitis pigmentosa (32.43%), and cone-rod dystrophy (5.41%). STGD1 could be further divided into central and dispersed types. In each phenotype, the lesion areas quantified by FAF increased with age (p < 0.01) and correlated with poorer visual acuity. However, three patients had the foveal sparing phenotype and had relatively preserved visual acuity. Forty-two ABCA4 variants were identified as disease-causing, with c.1804C>T (p.Arg602Trp) the most frequent (37.84%). Patients with a combination of severe/null variants could have more extensive phenotypes, such as arRP and dispersed STGD1. This is the first cohort study of ABCA4-associated retinal degeneration in Taiwan with wide spectrums of both genotypic and phenotypic characteristics. An extremely high prevalence of c.1804C>T, which has not been reported in East Asia before, was noted. The extensiveness of retinal involvement might be regarded as a spectrum of ABCA4-associated retinal dystrophies. Different types of genetic variations could lead to distinctive phenotypes, according to the coding impact of variants.

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Photodynamic therapy of subfoveal choroidal neovascularization

Srpski arhiv za celokupno lekarstvo ◽

10.2298/sarh0712629g ◽

2007 ◽

Vol 135 (11-12) ◽

pp. 629-634

Author(s):

Ana Georgijevic ◽

Zoran Tomic

Keyword(s):

Visual Acuity ◽

Photodynamic Therapy ◽

Blood Vessels ◽

Choroidal Neovascularization ◽

Retinal Pigment ◽

Photochemical Reactions ◽

Pathologic Myopia ◽

Target Tissue ◽

Choroidal Hemangioma

Introduction Photodynamic therapy (PDT) is a method of treatment of choroidal neovascularization (CNV) with a diode laser used after intravenously administered verteporfin. Verteporfin is a light-activated drug initiating photochemical reactions in the target tissue. This leads to the selective occlusion of blood vessels in the CNV with no damage of photoreceptors, retinal pigment epithelium and retinal blood vessels. Objective To show the results of the treatment of predominantly classic subfoveal CNV with PDT with verteporfin used for the first time in our country. Method From 2003 to 2005, we treated 15 eyes in 15 patients using PDT and verteporfin, because of predominantly classic subfoveal CNV. If macular oedema was present as proved by fluorescein angiography, triamcinolone was administered intravitreally after PDT. Average follow-up period was 7 months (3 months to 2 years). Study design: retrospective, noncomparative, consecutive case series. Results Two thirds of patients had CNV due to AMD, while in others it was caused by pathologic myopia, chorioretinitis, angioid streaks, choroidal hemangioma, except for one patient who had idiopathic CNV. Visual acuity was stabile in 60% (9/15) of patients, of whom in 60% (6/10) of patients with AMD, as well as in patients with pathologic myopia, idiopathic CNV and choroidal hemangioma. Retreatment with PDT was indicated in 40% (6/15) and in 50% (5/10) of patients with AMD, mostly 4-6 months after first PDT, but was done only in one patient (economic reasons). In two patients with AMD, triamcinolone was administered intravitreally for 2-4 months, which resulted in the stabilization of visual acuity. Conclusion Visual acuity was stabile in 60% of all treated patients with predominantly classic subfoveal CNV after only one application of PDT with verteporfin during the average follow-up of 7 months (3 months to 2 years). Retreatment was indicated in 40% of the treated patients, and in 50% of patients with AMD. As confirmed, intravitreal administration of triamcinolone after PDT could stabilize visual acuity. Side effects were not noticed.

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Polypoidal choroidal vasculopathy in pachychoroid: combined treatment with photodynamic therapy and Aflibercept

10.21203/rs.3.rs-307308/v1 ◽

2021 ◽

Author(s):

Maria Vadalà ◽

Massimo Castellucci ◽

Giulia Guarrasi ◽

Giovanni Cillino ◽

Vincenza Maria Elena Bonfiglio ◽

...

Keyword(s):

Visual Acuity ◽

Photodynamic Therapy ◽

Polypoidal Choroidal Vasculopathy ◽

Combined Therapy ◽

Combined Treatment ◽

Subretinal Fluid ◽

Clinical Activity ◽

Monthly Basis ◽

Choroidal Vasculopathy

Abstract Introduction To evaluate the effects of combined therapy using intravitreal Aflibercept (IVA) and photodynamic therapy (PDT) on polypoidal choroidal vasculopathy related to pachychoroid disease (PPCV).Methods Patients with PPCV were treated with PDT combined with 3 IVA injections on a monthly basis, followed by pro re nata criteria. The 12-month follow-up consisted of multiple revaluations of visual acuity and SSOCT parameters of clinical activity.Results Nineteen eyes were included in the study; mean age was 65.5 years. Visual acuity improved after 12 months (0.35 ± 0.25 to 0.2 ± 0.20 logMAR, p = 0.005). Percentage of eyes with intraretinal and subretinal fluid reduced from baseline to the 12-month follow-up (from 52.6–10.5%, p = 0.12, and from 89.5–5.3% p = 0.0009, respectively). Central retinal and mean macular thicknesses reduced (258 ± 39.6 to 204.8 ± 38.8 µm p = 0.04 and 293.8 ± 32.1 to 248.1 ± 29.6 µm p = 0.017 respectively). Central choroidal and mean choroidal thicknesses also displayed a reduction (328.6 ± 54.9 to 289.8 ± 44.6 µm p = 0.001 and 314.5 ± 55.3 to 287.9 ± 47.6 µm p = 0.015 respectively). The mean number of injections was 4.6/year.Conclusion The results support the use of a combined therapy with Aflibercept and PDT in PPCV. This treatment would act in synergy, with anti-VEGF controlling exudation and PDT closing the aneurysmal vessel and reducing choroidal congestion.

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Fundus autofluorescence imaging of retinal dystrophies

Vision Research ◽

10.1016/j.visres.2008.01.010 ◽

2008 ◽

Vol 48 (26) ◽

pp. 2569-2577 ◽

Cited By ~ 37

Author(s):

Camiel J.F. Boon ◽

B. Jeroen Klevering ◽

Jan E.E. Keunen ◽

Carel B. Hoyng ◽

Thomas Theelen

Keyword(s):

Fundus Autofluorescence ◽

Autofluorescence Imaging ◽

Retinal Dystrophies

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Acute Zonal Occult Outer Retinopathy in a Patient Suffering from Epilepsy: Five-Year Follow-Up

Medicina ◽

10.3390/medicina57111276 ◽

2021 ◽

Vol 57 (11) ◽

pp. 1276

Author(s):

Izabella Karska-Basta ◽

Bożena Romanowska-Dixon ◽

Dorota Pojda-Wilczek ◽

Alina Bakunowicz-Łazarczyk ◽

Agnieszka Kubicka-Trząska ◽

...

Keyword(s):

Visual Acuity ◽

Visual Field ◽

Multimodal Imaging ◽

Fundus Autofluorescence ◽

Medical Intervention ◽

Fundus Photography ◽

Unknown Etiology ◽

Visual Field Examination ◽

Laboratory Findings

We report an unprecedented case of a young patient with epilepsy coexisting with acute zonal occult outer retinopathy (AZOOR), a rare white dot syndrome of unknown etiology, associated with damage to the large zones of the outer retina. Recently, it has been established that epileptic episodes contribute to an inflammatory response both in the brain and the retina. A 13-year-old male patient with epilepsy was referred by a neurologist for an ophthalmologic consultation due to a sudden deterioration of visual acuity in the left eye. The examination, with a key role of multimodal imaging including color fundus photography, fluorescein angiography, indocyanine green angiography (ICGA), fundus autofluorescence (FAF), swept-source optical coherence tomography (SS-OCT) with visual field assessment, and electroretinography indicated AZOOR as the underlying entity. Findings at the first admission included enlargement of the blind spot in visual field examination along a typical trizonal pattern, which was revealed by FAF, ICGA, and SS-OCT in the left eye. The right eye exhibited no abnormalities. Seminal follow-up revealed no changes in best corrected visual acuity, and multimodal imaging findings remain unaltered. Thus, no medical intervention is required. Our case and recent laboratory findings suggest a causative link between epilepsy and retinal disorders, although this issue requires further research.

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Result of Intravitreal Aflibercept Injection for Myopic Choroidal Neovascularization

10.21203/rs.3.rs-146536/v1 ◽

2021 ◽

Author(s):

Shih-Lin Chen ◽

Tsung-Tien Wu ◽

Pei-Ling Tang

Keyword(s):

Visual Acuity ◽

Choroidal Neovascularization ◽

Minimum Duration ◽

Foveal Thickness ◽

Flow Area ◽

Myopic Choroidal Neovascularization ◽

Central Foveal Thickness ◽

Intravitreal Aflibercept ◽

Myopic Cnv

Abstract Background: The current study aimed to evaluate the efficacy of intravitreal aflibercept injections as the primary treatment for subfoveal/juxtafoveal myopic choroidal neovascularization (CNV) using optical coherence tomography angiography (OCTA) to detect the changes in shape, size, and flow area pertaining to CNV, with a minimum duration of follow-up of one year. Methods: In the present study, 21 treatment-naive eyes of 21 patients with subfoveal/juxtafoveal myopic CNV received primary intravitreal aflibercept injections and were under follow-up for a minimum duration of 12 months. Among the 21 patients, 12 underwent OCTA to evaluate the changes in central foveal thickness, selected CNV area, and flow area. Results: The mean best-corrected visual acuity (BCVA) pertaining to all the patients significantly improved from the baseline value of 0.7 to 0.3 logMAR after treatment for 12 months (P = 0.001). However, the improvements in the median BCVA after treatment for three and twelve months were not statistically significant in the younger group (< 50 years), compared to the older group (≥ 50 years). A single aflibercept injection resolved the CNV in 47.6% (10/21) of the patients. The younger group displayed greater improvement in the median central foveal thickness, compared to the older group. OCTA revealed interlacing or disorganized patterns at the level of the outer retinal layer in 12 among the 21 subjects with myopic CNV. After three months of treatment, both the groups displayed a decrease in the size of the selected CNV area and flow area. The interlacing group displayed a trend towards better anatomical improvements. Conclusion: Intravitreal aflibercept injection provides long-term improvement in visual acuity in patients with myopic CNV. A single aflibercept injection was observed to resolve myopic CNV in approximately half of the patients. The interlacing group displayed greater resolution of the selected CNV area and flow area after aflibercept injection. Trial registration: Before data collection, written informed consent was obtained from each participant, whose identity information was protected by encryption and conversion to a non-identifiable format and removing data links. This study was approved by the Institutional Review Board of Kaohsiung Veterans General Hospital (KSVGH21-CT1-17).

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