Optical Coherence Tomography Examination of the Retinal Pigment Epithelium in Best Vitelliform Macular Dystrophy

Qian, Cheng; Charran, Dionisio; Strong, Cameron R.; Steffens, Timothy; Jayasundera, Thiran; Heckenlively, John R.

doi:10.1016/j.ophtha.2016.11.022

Cited by 34 publications

(30 citation statements)

References 47 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In patients with dominant BEST1 disease (BVMD), there has been some controversy regarding the structural features of retinal regions surrounding vitelliform or later-stage lesions, or retinas in the previtelliform stage of disease. Some studies have demonstrated minor abnormalities at the level of the RPE–PR interface ( 34 , 70 , 71 ), whereas results from others support no detectable structural defects ( 72 – 74 ). Contributing to this controversy could be genotype ( 75 ), the resolution of different methodological approaches used, or light history preceding the imaging ( 36 ).…”

Section: Discussionmentioning

confidence: 99%

BEST1 gene therapy corrects a diffuse retina-wide microdetachment modulated by light exposure

Guziewicz

Cideciyan

Beltran

et al. 2018

Proc. Natl. Acad. Sci. U.S.A.

View full text Add to dashboard Cite

SignificanceOne of the most common forms of monogenic macular degeneration worldwide is caused by dominant or recessive bestrophinopathies associated with mutations in the BEST1 gene. Disease expression is known to start with a retina-wide electrophysiological defect leading to localized vitelliform and atrophic lesions and vision loss. To develop lasting therapies for this incurable disease, there is a need for greater understanding of the early pathophysiology before lesion formation. Here we find that the loss of retinal pigment epithelium apical microvilli and resulting microdetachment of the retina represent the earliest features of canine bestrophinopathies. We show that retinal light exposure expands, and dark adaptation contracts, the microdetachments. Subretinal adeno-associated virus-based gene therapy corrects both the vitelliform lesions and the light-modulated microdetachments.

show abstract

Section: Discussionmentioning

confidence: 99%

BEST1 gene therapy corrects a diffuse retina-wide microdetachment modulated by light exposure

Guziewicz

Cideciyan

Beltran

et al. 2018

Proc. Natl. Acad. Sci. U.S.A.

View full text Add to dashboard Cite

show abstract

“…Her most recent FA demonstrated no leakage. Despite the absence of FA leakage, OCT showed persistence of an optically clear subretinal cleft [11]. En-face OCTA revealed two main trunks, one superior and one inferior, with a lack of small capillaries, anastomoses, and vessel loops.…”

Section: Patientmentioning

confidence: 91%

Appearance of pediatric choroidal neovascular membranes on optical coherence tomography angiography

Ong

Hsu

Grewal

et al. 2019

Graefes Arch Clin Exp Ophthalmol

View full text Add to dashboard Cite

Purpose-Compared with fluorescein angiography (FA), the gold standard for diagnosing choroidal neovascularization (CNV) activity, optical coherence tomography angiography (OCTA) is non-invasive without risks associated with fluorescein dye use, and may be especially advantageous in the diagnosis and monitoring of children with CNV. Methods-Eight eyes from eight patients aged 12 months to 18 years were imaged with the investigational Spectralis OCTA (version 6.9, Heidelberg Engineering, Heidelberg, Germany) and the RTVue XR Avanti (Optovue Inc., Fremont, CA, USA). Two patients were imaged during examination under anesthesia while six patients were imaged in the clinic. Demographic information, ocular characteristics, treatment history, and imaging studies (color photos, fluorescein angiography, OCT) were collected and reviewed. Results-Three eyes had active CNV while five had quiescent CNV at the time of imaging. CNV was idiopathic or secondary to trauma, retinal vascular dysgenesis versus retinopathy of prematurity, pigmentary retinopathy, Best vitelliform macular dystrophy, panuveitis, morning glory disc anomaly, and optic disc drusen. OCTA of two active CNV demonstrated presence of a main trunk with multiple fine capillaries, vessel loops, and anastomoses. OCTA was repeated after treatment for two CNV and demonstrated a decrease in size with loss of fine capillaries, vessel loops, and anastomoses. For the third active CNV, OCTA verified flow in the CNV complex despite the uncertainty of FA hyperfluorescence in the setting of grossly abnormal retinal vasculature. The five quiescent CNV all lacked fine capillaries, vessel loops, and anastomoses on OCTA. Lejla Vajzovic, Lejla.vajzovic@duke.edu. Conflict of interest STH declares that she has no conflict of interest. SSO declares that she has no conflict of interest. Ethical approval All procedures performed in studies involving human participants were in accordance with the ethical standards of the Duke University and Johns Hopkins University and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors. Informed consent Informed consent was obtained from all individual participants included in the study from Duke University. One participant was retrospectively included from Johns Hopkins University and no consent was obtained from this single case report.

show abstract

“…The vision-affecting-diseases and therapeutic progress change the characters of eye layers [10][11][12][13][14]. The most commonly used markers in ophthalmological OCT diagnosing are retinal thickness, retinal layer structure and choroidal thickness.…”

Section: Oct In Ophthalmologymentioning

confidence: 99%

Application of optical coherence tomography in clinical diagnosis

Wang

Liu

Lou

et al. 2020

XST

View full text Add to dashboard Cite

BACKGROUND: Optical coherence tomography (OCT) is a non-invasive diagnosing tool used in clinics. Due to its high resolution (<10um), it is appropriate for the early detection of tiny infections. It has been widely used in diagnosis and treatment of diseases, evaluation of therapeutic efficacy, and monitoring of various physiological and pathological processes. OBJECTIVE: To systemically review literature to summarize the clinic application of OCT in recent years. METHODS: For clinic applications that OCT has been applied, we selected studies that describe the most relevant works. The discussion included: 1) which tissue could be used in the OCT detection, 2) which character of different tissue could be used as diagnosing criteria, 3) which diseases and pathological process have been diagnosed or monitored using OCT imaging, and 4) the recent development of clinic OCT diagnosing. RESULTS: The literature showed that the OCT had been listed as a routine test choice for ophthalmic diseases, while the first commercial product for cardiovascular OCT detection had gotten clearance. Meanwhile, as the development of commercial benchtop OCT equipment and tiny fiber probe, the commercial application of OCT in dermatology, dentistry, gastroenterology and urology also had great potential in the near future. CONCLUSIONS: The analysis and discussions showed that OCT, as an optical diagnosing method, has been used successfully in many clinical fields, and has the potential to be a standard inspection method in several clinic fields, such as dermatology, dentistry and cardiovascular.

show abstract

Optical Coherence Tomography Examination of the Retinal Pigment Epithelium in Best Vitelliform Macular Dystrophy

Cited by 34 publications

References 47 publications

BEST1 gene therapy corrects a diffuse retina-wide microdetachment modulated by light exposure

BEST1 gene therapy corrects a diffuse retina-wide microdetachment modulated by light exposure

Appearance of pediatric choroidal neovascular membranes on optical coherence tomography angiography

Application of optical coherence tomography in clinical diagnosis

Contact Info

Product

Resources

About