SD-OCT in Pigmented Paravenous Retinochoroidal Atrophy

2012 ◽  
Author(s):  
Basudeb Ghosh ◽  
Neha Goel ◽  
Supriya Batta ◽  
Usha Kaul Raina
Keyword(s):  
Pigmented Paravenous Retinochoroidal Atrophy ◽  
Sd Oct

A mutation in CRX causing pigmented paravenous retinochoroidal atrophy

2020 ◽  
pp. 112067212095759
Author(s):  
Jin Kyun Oh ◽  
Yan Nuzbrokh ◽  
Winston Lee ◽  
Jose Ronaldo Lima de Carvalho ◽  
Nan Kai Wong ◽  
...  
Keyword(s):  
Vision Loss ◽  
Retinal Dystrophy ◽  
Cone Dystrophy ◽  
Full Field ◽  
Inherited Retinal Dystrophy ◽  
History Of ◽  
Pigmented Paravenous Retinochoroidal Atrophy ◽  
Retinal Thinning ◽  
Bull's Eye ◽  
Sd Oct

Introduction: Mutations in the cone-rod homeobox ( CRX) gene, a known cause of inherited retinal dystrophy, are characterized by extensive phenotypic heterogeneity. We describe a novel presentation of rod-cone dystrophy (RCD) phenocopying pigmented paravenous retinochoroidal atrophy associated with a mutation in CRX. Case description: A 53-year-old man and his 48-year-old brother presented with a history of progressive vision loss and nyctalopia. Fundus examination revealed a bull’s eye lesion with chorioretinal atrophy and intraretinal pigment migration, while spectral-domain optical coherence tomography (SD-OCT) demonstrated retinal thinning with outer retinal atrophy. On short-wavelength autofluorescence (SW-AF) imaging, an atypical paravenous pattern of atrophy with a surrounding hyperautofluorescent border was observed. Full-field electroretinogram (ffERG) revealed a rod-cone pattern of dysfunction. A heterozygous pathogenic variant, c.119G>A:p.(Arg40Gln), in the CRX gene was identified in both brothers and segregated in their family. Conclusion: This case report broadens the currently known phenotypic presentations of CRX-associated retinopathy and suggests that mutations in CRX may be associated with pigmented paravenous retinochoroidal atrophy.

scholarly journals Pattern of inner retinal layers involvement in pigmented paravenous retinochoroidal atrophy as determined by SD-OCT: case report

2013 ◽  
Vol 76 (6) ◽  
pp. 380-382 ◽  
Author(s):  
Daniela Laura Melo Junqueira ◽  
Flavio Siqueira Santos Lopes ◽  
Luís Gustavo Biteli ◽  
Tiago Santos Prata
Keyword(s):  
Case Report ◽  
Retinal Layers ◽  
Pigmented Paravenous Retinochoroidal Atrophy ◽  
Sd Oct

Misalignment of foveal pit and foveal bulge determined by ultrahigh-resolution SD-OCT in normal eyes

2020 ◽  
Vol 258 (10) ◽  
pp. 2131-2139
Author(s):  
Yoshitsugu Matsui ◽  
Ryohei Miyata ◽  
Eriko Uchiyama ◽  
Hisashi Matsubara ◽  
Mineo Kondo
Keyword(s):  
Ultrahigh Resolution ◽  
Sd Oct

scholarly journals Optimization of spectral-domain optical coherence tomography with a supercontinuum source for in vivo motion detection of low reflective outer hair cells in guinea pig cochleae

2021 ◽  
Author(s):  
Fumiaki Nin ◽  
Samuel Choi ◽  
Takeru Ota ◽  
Zhang Qi ◽  
Hiroshi Hibino
Keyword(s):  
Optical Coherence Tomography ◽  
High Resolution ◽  
Guinea Pig ◽  
Hair Cells ◽  
Sensory Epithelium ◽  
Outer Hair Cells ◽  
Acquisition Time ◽  
Total Acquisition Time ◽  
Sd Oct

AbstractSound evokes sub-nanoscale vibration within the sensory epithelium. The epithelium contains not only immotile cells but also contractile outer hair cells (OHCs) that actively shrink and elongate synchronously with the sound. However, the in vivo motion of OHCs has remained undetermined. The aim of this work is to perform high-resolution and -accuracy vibrometry in live guinea pigs with an SC-introduced spectral-domain optical coherence tomography system (SD-OCT). In this study, to reveal the effective contribution of SC source in the recording of the low reflective materials with the short total acquisition time, we compare the performances of the SC-introduced SD-OCT (SCSD-OCT) to that of the conventional SD-OCT. As inanimate comparison objects, we record a mirror, a piezo actuator, and glass windows. For the measurements in biological materials, we use in/ex vivo guinea pig cochleae. Our study achieved the optimization of a SD-OCT system for high-resolution in vivo vibrometry in the cochlear sensory epithelium, termed the organ of Corti, in mammalian cochlea. By introducing a supercontinuum (SC) light source and reducing the total acquisition time, we improve the axial resolution and overcome the difficulty in recording the low reflective material in the presence of biological noise. The high power of the SC source enables the system to achieve a spatial resolution of 1.72 ± 0.00 μm on a mirror and reducing the total acquisition time contributes to the high spatial accuracy of sub-nanoscale vibrometry. Our findings reveal the vibrations at the apical/basal region of OHCs and the extracellular matrix, basilar membrane.

scholarly journals P009 Service evaluation to optimise ophthalmological services for hydroxychloroquine retinal screening

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Chan Ning Lee ◽  
Mrinalini Dey ◽  
Mooikhin Hng ◽  
Simon Peterson ◽  
Imna Rahiman ◽  
...  
Keyword(s):  
Patient Education ◽  
Service Evaluation ◽  
Continuous Treatment ◽  
Fundus Photography ◽  
Baseline Risk ◽  
Service Development ◽  
Cross Sectional ◽  
Retinal Pathology ◽  
Retinal Screening ◽  
Sd Oct

Abstract Background/Aims  Hydroxychloroquine (HCQ), a frequently-used therapy in rheumatology, can be associated with retinal toxicity. More stringent screening and monitoring guidelines for HCQ-related retinopathy were published by the Royal College of Ophthalmologists (RCOphth) in 2018. Recommendations include: 1) baseline retinal screening within six-twelve months of commencing HCQ; 2) subsequent annual monitoring for at-risk patients, specifically: concurrent tamoxifen-use, estimated glomerular filtration rate (eGFR) <60ml/min/1.73m2, HCQ dose >5mg/kg/day; 3) annual monitoring for all other patients after five years continuous treatment; 4) patient education on HCQ retinopathy. We quantified the rheumatology HCQ exposure and estimated burden on ophthalmology, to inform the development of HCQ retinal screening services. Methods  Cross-sectional data were extracted for patients established on HCQ between 1995-2020 including: weight; eGFR; concomitant retino-toxic medication; concomitant retinal pathology; HCQ dose and duration; documentation of patient education on retinal side-effects. Documentation of relevant ophthalmic testing at baseline (i.e. at time of starting HCQ) were recorded, specifically colour fundus photography, spectral domain optical coherence tomography (SD-OCT) and 10-2 Humphrey visual field (HVF) testing as necessary. Subsequent monitoring with 10-2 HVFs, SD-OCT, autofluorescence (AF) and electrodiagnostic tests (EDTs) as appropriate annually from baseline and from 5 years was recorded. Results  150 patients were included, 84% female, with a mean baseline age of 50.7 years (SD 14.8) and mean weight of 76.4kg (SD 17.6). 63% were on HCQ >5 years (mean duration 7.0 years, SD 5.1). At time of auditing, 50% (75/150) patients had permanently ceased HCQ (62% due to treatment >5 years). 60% patients had documented evidence of education regarding HCQ retinopathy. Of the 150 patients, 39% had baseline risks for retinopathy. 6% had a baseline eGFR <60ml/min/1.73m2. 32% were commenced on HCQ dose greater than 5mg/kg/day. No patients were on concomitant tamoxifen; one patient was on a known retino-toxic drug (quinine). 4% of patients had pre-existing retinal pathology. Of the 75 patients still taking HCQ at time of auditing, 27 (36%) had baseline risk factors warranting yearly retinal screening and 44 (58%) had been on treatment >5years. Most ophthalmology reviews were put on hold until a screening service was established. Of the small number (5.3%) that had baseline ophthalmic screening, 12.5% had colour photography and 75% had SD-OCT. No patients required HVF testing or EDTs. Annual screening revealed 50% of patients had 10-2 HVFs; 75% had SD-OCT; 75% had AF. Only one patient developed maculopathy, not attributed to HCQ. Conclusion  Our results demonstrate the need for service development to facilitate adherence to RCOphth guidance. Up to 40% of patients started on HCQ have baseline risks for retinopathy, most due to dosing >5mg/kg/day warranting yearly screening. Our findings will inform development of a rheumatology HCQ retinal screening pathway and services, to ensure safe long-term use of HCQ. Disclosure  C. Lee*: None. M. Dey*: None. M. Hng: None. S. Peterson: None. I. Rahiman: None. M. Elshafei: None. C. Estrach: None. N.J. Goodson: None.

scholarly journals FRI0649-HPR HYDROXYCHLOROQUINE PRESCRIBING AND OPHTHALMOLOGY SCREENING WITHIN RHEUMATOLOGY DEPARTMENTS IN THE NORTH-WEST OF THE UNITED KINGDOM: A PROSPECTIVE REGIONAL AUDIT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 928.2-929
Author(s):  
S. Juman ◽  
T. David ◽  
L. Gray ◽  
R. Hamad ◽  
S. Horton ◽  
...  
Keyword(s):  
High Risk ◽  
Risk Group ◽  
Service Level ◽  
High Risk Group ◽  
North West ◽  
The North ◽  
The United Kingdom ◽  
The Uk ◽  
Treatment Dosage ◽  
Sd Oct

Background:Hydroxychloroquine (HCQ) is widely used in the management of rheumatoid arthritis and connective tissue disease. The prevalence of retinopathy in patients taking long-term HCQ is approximately 7.5%, increasing to 20-50% after 20 years of therapy. Hydroxychloroquine prescribed at ≤5 mg/kg poses a toxicity risk of <1% up to five years and <2% up to ten years, but increases sharply to almost 20% after 20 years. Risk factors for retinopathy include doses >5mg/kg/day, concomitant tamoxifen or chloroquine use and renal impairment. The UK Royal College of Ophthalmologists (RCOphth) 2018 guidelines for HCQ screening recommend optimal treatment dosage and timing for both baseline and follow-up ophthalmology review for patients on HCQ, with the aim of preventing iatrogenic visual loss. This is similar to recommendations made by the American Academy of Ophthalmology (2016).Objectives:To determine adherence to the RCOphth guidelines for HCQ screening within the Rheumatology departments in the North-West of the UK.Methods:Data for patients established on HCQ and those initiated on HCQ therapy were collected over a 7 week period from 9 Rheumatology departments.Results:473 patients were included of which 56 (12%) were new starters and 417 (88%) were already established on HCQ. 79% of the patients were female, with median ages of 60.5 and 57 years for new and established patients respectively. The median (IQR) weight for new starters was 71 (27.9) kg and for established patients, 74 (24.7) kg.20% of new starters exceeded 5mg/kg daily HCQ dose. 16% were identified as high risk (9% had previously taken chloroquine, 5% had an eGFR <60ml/min/m2and 2% had retinal co-pathology). Of the high-risk group, 44% were taking <5mg/kg. In total, 36% of new starters were referred for a formal baseline Ophthalmology review.In the established patients, 74% were taking ≤5mg/kg/day HCQ dose and 16% were categorized as high risk (10% had an eGFR less than 60ml/min/m2, 3% had previous chloroquine or tamoxifen use and 2% had retinal co-pathology). In the high-risk group, 75% were not referred for spectral domain optical coherence tomography (SD-OCT). 41% of patients established on HCQ for <5 years, and 33% of patients on HCQ for >5 years were not referred for SD-OCT. Reasons for not referring included; awaiting 5 year review, previous screening already performed and optician review advised.Since the introduction of the RCOphth guidelines, 29% patients already established on HCQ had an alteration in the dosage of HCQ in accordance with the guidelines. In the high-risk group, 16% were not on the recommended HCQ dose.Conclusion:This audit demonstrates inconsistencies in adherence to the RCOphth guidelines for HCQ prescribing and ophthalmology screening within Rheumatology departments in the North-West of the UK for both new starters and established patients. Plans to improve this include wider dissemination of the guidelines to Rheumatology departments and strict service level agreements with ophthalmology teams to help optimize HCQ prescribing and screening for retinopathy.Acknowledgments:Drs. S Jones, E MacPhie, A Madan, L Coates & Prof L Teh. Co-1st author, T David.Disclosure of Interests:None declared

scholarly journals Corneal hysteresis as a risk factor for optic nerve head surface depression and retinal nerve fiber layer thinning in glaucoma patients

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Guihua Xu ◽  
Zilin Chen
Keyword(s):  
Risk Factor ◽  
Visual Field ◽  
Proportional Hazards ◽  
Cox Proportional Hazards ◽  
Corneal Hysteresis ◽  
Visual Field Progression ◽  
Increased Risk ◽  
Head Surface ◽  
Sd Oct ◽  
Surface Depression

AbstractTo evaluate the role of corneal hysteresis (CH) as a risk factor for progressive ONH surface depression and RNFL thinning measured by confocal scanning laser ophthalmoscopy (CSLO) and spectral-domain optical coherence tomography (SD-OCT), respectively in glaucoma patients. Prospective study. A total of 146 eyes of 90 patients with glaucoma were recruited consecutively. The CH measurements were acquired at baseline and 4-months interval using the Ocular Response Analyzer (Reichert Instruments, Depew, NY). Eyes were imaged by CSLO (Heidelberg Retinal Tomograph [HRT]; Heidelberg Engineering, GmbH, Dossenheim, Germany) and SD-OCT (Cirrus HD-OCT; Carl Zeiss Meditec AG, Dublin, CA) at approximately 4-month intervals for measurement of ONH surface topography and RNFL thickness, respectively. Significant ONH surface depression and RNFL thinning were defined with reference to Topographic Change Analysis (TCA) with HRT and Guided Progression Analysis (GPA) with Cirrus HD-OCT, respectively. Multivariate cox proportional hazards models were used to investigate whether CH is a risk factor for ONH surface depression and RNFL progression after adjusting potential confounding factors. All patients with glaucoma were followed for an average of 6.76 years (range, 4.56–7.61 years). Sixty-five glaucomatous eyes (44.5%) of 49 patients showed ONH surface depression, 55 eyes (37.7%) of 43 patients had progressive RNFL thinning and 20 eyes (13.7%) of 17 patients had visual field progression. In the cox proportional hazards model, after adjusting baseline diastolic IOP, CCT, age, baseline disc area and baseline MD, baseline CH was significantly associated with ONH surface depression and visual field progression (HR = 0.71, P = 0.014 and HR = 0.54, P = 0.018, respectively), but not with RNFL thinning (HR = 1.03, P = 0.836). For each 1-mmHg decrease in baseline CH, the hazards for ONH surface depression increase by 29%, and the hazards for visual field progression increase by 46%. The CH measurements were significantly associated with risk of glaucoma progression. Eyes with a lower CH were significantly associated with an increased risk of ONH surface depression and visual field progression in glaucoma patients.

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