scholarly journals Correlation of features on OCT with visual acuity and Gass lesion type in best vitelliform macular dystrophy

2021 ◽  
Vol 6 (1) ◽  
pp. e000860
Author(s):  
Razek G Coussa ◽  
Christopher R Fortenbach ◽  
D Brice Critser ◽  
Malia M Collins ◽  
Budd A Tucker ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Case Series ◽  
Structural Features ◽  
Macular Dystrophy ◽  
Retrospective Case ◽  
Vitelliform Macular Dystrophy ◽  
Best Vitelliform Macular Dystrophy ◽  
Lesion Type ◽  
Prognostic Importance ◽  
Endothelial Growth Factor

ObjectiveTo correlate structural features seen on optical coherence tomography (OCT) with best-corrected visual acuity (BCVA) and Gass lesion type in patients with best vitelliform macular dystrophy (BVMD).Methods and analysisThis is a retrospective case series of consecutive patients with molecularly confirmed BEST1-associated BVMD. OCT scans were reviewed for lesion status and presence of subretinal pillar, focal choroidal excavation (FCE), intraretinal fluid or atrophy. Available OCT angiography images were used to evaluate for the presence of choroidal neovascularisation (CNV). These features were then correlated with BCVA and Gass lesion type.Results95 eyes from 48 patients (mean age 38.9 years, range 4–87) were included. The presence of a pillar (24.2%), FCE (20.0%) and atrophy (7.4%) were associated with poor BCVA (p<0.05). Gass lesion type 1 eyes were correlated with good BCVA (LogMAR <0.4) whereas type 5 eyes had poor BCVA (LogMAR >0.4). Among 65 eyes with longitudinal data (mean follow-up 5.1 years), 7 eyes (10.8%) reverted from higher to lower Gass lesion type; of these, 4 eyes (57.1%) had CNV responsive to intravitreal anti-vascular endothelial growth factor treatment.ConclusionOCT-based structural features are readily identifiable in patients with BVMD and have prognostic importance due to their correlation with BCVA.

Intravitreal bevacizumab treatment for exudative choroidal neovascularisation in best vitelliform macular dystrophy

2021 ◽  
pp. 112067212110576
Author(s):  
Martina Jarc-Vidmar ◽  
Rok Sega ◽  
Polona Jaki-Mekjavic
Keyword(s):  
Visual Acuity ◽  
Paediatric Patient ◽  
Case Series ◽  
Intravitreal Bevacizumab ◽  
Choroidal Neovascularisation ◽  
Macular Dystrophy ◽  
Central Scar ◽  
Vitelliform Macular Dystrophy ◽  
Best Vitelliform Macular Dystrophy ◽  
Bevacizumab Treatment

Purpose To describe results of intravitreal bevacizumab treatment of the secondary choroidal neovascularisation in Best vitelliform macular dystrophy in an adult and paediatric patient, and present the management of three asymptomatic patients with confirmed BEST1 gene mutation. Case series description Five patients from the same family with the Best vitelliform macular dystrophy are presented. In two patients (aged 63 and 4 years) secondary choroidal neovascularisation caused a rapid decline in visual acuity. In the adult patient with advanced Best vitelliform macular dystrophy, visual acuity did not improve despite eight intravitreal bevacizumab injections to the right eye. The formation of a central scar and rapid reoccurrence of choroidal neovascularisation three months after completing the initial treatment affected the outcome. As for the paediatric patient with bilateral choroidal neovascularisation in the vitelliform stage of Best vitelliform macular dystrophy, a complete recovery of visual acuity was observed after two (left eye) and three (right eye) bevacizumab injections, with adjunctive amblyopia treatment. The other three patients with an abnormal electrooculogram reported no visual problems during more than 10 years of follow-up. Minimal changes were seen on optical coherence tomography in the youngest patient. Conclusions Intravitreal bevacizumab seems to be an effective treatment for exudative choroidal neovascularisation in the vitelliform stage of Best vitelliform macular dystrophy; however, it may not be beneficial in the advanced stages of Best vitelliform macular dystrophy. It is important to regularly screen all family members with an abnormal electrooculogram and confirmed mutation for vitelliform changes and choroidal neovascularisation from an early age. The decision for anti-vascular endothelial growth factor treatment should be made on a case-to-case basis as complications may arise.

Visual Acuity in Patients with Best Vitelliform Macular Dystrophy

Ophthalmology ◽  
1993 ◽  
Vol 100 (11) ◽  
pp. 1665-1670 ◽  
Author(s):  
Gerald A. Fishman ◽  
Wendy Baca ◽  
Kenneth R. Alexander ◽  
Deborah J. Derlacki ◽  
Andrew M. Glenn ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Macular Dystrophy ◽  
Vitelliform Macular Dystrophy ◽  
Best Vitelliform Macular Dystrophy

scholarly journals Association of Optic Nerve Head Drusen with Best Vitelliform Macular Dystrophy: A Case Series

2018 ◽  
Vol 9 (1) ◽  
pp. 82-92 ◽  
Author(s):  
Robert J. White ◽  
Daniel J. Watson ◽  
Dara D. Koozekanani ◽  
Sandra R. Montezuma
Keyword(s):  
Optic Nerve ◽  
Ultrasound Imaging ◽  
Optic Nerve Head ◽  
Multimodal Imaging ◽  
Chart Review ◽  
Case Series ◽  
Macular Dystrophy ◽  
Vitelliform Macular Dystrophy ◽  
Best Vitelliform Macular Dystrophy ◽  
Optic Nerve Head Drusen

Purpose: To report the association of optic nerve head (ONH) drusen with Best vitelliform macular dystrophy (BVMD). Methods: Chart review. Patients: Five patients from 3 families. Results: Multimodal imaging and ophthalmic examination demonstrated findings consistent with ONH drusen, in association with BVMD, in 5 patients. Conclusion: We report the association of BVMD with ONH drusen in 5 patients. This combination has previously been reported only once. We recommend that patients with a diagnosis of BVMD undergo autofluorescence and ultrasound imaging of the optic nerve to help facilitate this diagnosis, as some ONH drusen can be buried.

Multimodal imaging in subclinical best vitelliform macular dystrophy

2020 ◽  
pp. bjophthalmol-2020-317635
Author(s):  
Maurizio Battaglia Parodi ◽  
Alessandro Arrigo ◽  
Alessandro Calamuneri ◽  
Emanuela Aragona ◽  
Francesco Bandello
Keyword(s):  
Multimodal Imaging ◽  
Near Infrared ◽  
Case Series ◽  
Foveal Avascular Zone ◽  
Macular Dystrophy ◽  
Cross Sectional ◽  
Vitelliform Macular Dystrophy ◽  
Best Vitelliform Macular Dystrophy ◽  
Capillary Plexus ◽  
Growth Factor Production

BackgroundTo analyse multimodal imaging alterations in the subclinical form of best vitelliform macular dystrophy (BVMD).MethodsThe study was designed as an observational, cross-sectional case series. Eleven eyes of 7 subclinical patients with BVMD and 12 age-matched and sex-matched controls were included. Multimodal imaging included fundus blue-light autofluorescence, near-infrared autofluorescence (NIR-AF), structural optical coherence tomography (OCT) and OCT angiography (OCTA). The quantitative analysis included the calculation of the following parameters: vessel density (VD), vessel tortuosity (VT), vessel dispersion (Vdisp), vessel rarefaction (VR), foveal avascular zone (FAZ) area, reflectivity of the outer retinal bands and choriocapillaris porosity (CCP).ResultsMean best-corrected visual acuity was 0.0±0.0 LogMAR in both groups. The round central hypoautofluorescent alteration on NIR-AF corresponded to a significant reflectivity attenuation of the outer retinal bands on structural OCT (0.55±0.18 vs 0.75±0.08; p<0.001). VD, VT, VR and Vdisp were normal compared with controls (all p>0.05). The FAZ area turned out to be significantly restricted at the level of the deep capillary plexus in subclinical BVMD eyes (p<0.001). Furthermore, quantitative OCTA revealed a significant central increase of CCP, compared with controls (18.25±2.43 vs 4.58±1.36; p<0.001).ConclusionsThe subclinical stage of BVMD is characterised by significant alterations of the outer retinal bands and the choriocapillaris. Quantitative multimodal imaging assessment suggests that subclinical BVMD is affected by the functional impairment of the outer retinal structures, leading to an alteration in melanin and growth factor production.

scholarly journals Incidence, Risk, and Visual Outcomes after Repositioning of Acute Non-Traumatic Flap Dislocations Following Femtosecond-Assisted LASIK

2021 ◽  
Vol 10 (11) ◽  
pp. 2478
Author(s):  
Majid Moshirfar ◽  
David G. West ◽  
Chase M Miller ◽  
William B. West ◽  
Shannon E. McCabe ◽  
...  
Keyword(s):  
Risk Factors ◽  
Visual Acuity ◽  
High Myopia ◽  
Case Series ◽  
Retrospective Case ◽  
Visual Outcomes ◽  
Uncorrected Distance Visual Acuity ◽  
Incidence Risk ◽  
Mechanical Devices

Although the use of femtosecond lasers instead of mechanical devices has decreased the incidence of flap complications following laser-assisted in situ keratomileusis (LASIK), dislocations and striae still occur. Flap repositioning is an effective intervention to improve visual outcomes after acute flap complications in both microkeratome-assisted and femtosecond-assisted LASIK. This retrospective case series included patients undergoing flap repositioning secondary to acute flap dislocation and/or visually significant striae within the first two weeks following femtosecond LASIK (FS-LASIK) from 2015 to 2020 at a single institution. Preoperative, intraoperative, and postoperative de-identified data were analyzed for incidence, risk factors, and visual acuity outcomes. The incidence of flap repositioning was 0.35% in 21,536 eyes (n = 70). Indications for repositioning included acute flap dislocation (35.7%) and visually significant striae (64.3%). High myopia (OR = 3.04, p = 0.001) and patient age over 50 years (OR = 3.69, p = 0.001) were the strongest risk factors for these complications. Prior to flap repositioning, uncorrected distance visual acuity (UDVA) of 20/20 or better and 20/40 or better occurred in 19% and 57% of eyes, respectively. After repositioning, a final UDVA of 20/20 or better and 20/40 or better occurred in 78% and 98% of eyes, respectively. After repositioning, one line of UDVA was lost in two eyes (2.8%) and two lines were lost in one eye (1.4%). Risk factors for acute flap dislocation included high myopia and age over 50 years. Flap repositioning was effective in salvaging visual outcomes.

PREFERENTIAL HYPERACUITY PERIMETER IN BEST VITELLIFORM MACULAR DYSTROPHY

Retina ◽  
2011 ◽  
Vol 31 (5) ◽  
pp. 959-966 ◽  
Author(s):  
Giuseppe Querques ◽  
Karim Atmani ◽  
Rislie Bouzitou-Mfoumou ◽  
Nicolas Leveziel ◽  
Nathalie Massamba ◽  
...  
Keyword(s):  
Macular Dystrophy ◽  
Vitelliform Macular Dystrophy ◽  
Best Vitelliform Macular Dystrophy

scholarly journals Combined Pars Plana Glaucoma Drainage Device Placement and Vitrectomy Using a Vitrectomy Sclerotomy Site for Tube Placement: A Case Series

2020 ◽  
Author(s):  
Enchi Kristina Chang ◽  
Sanchay Gupta ◽  
Marika Chachanidze ◽  
John B. Miller ◽  
Ta Chen Chang ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Statistical Tests ◽  
Case Series ◽  
Tube Placement ◽  
Glaucoma Drainage Device ◽  
Retrospective Case ◽  
Tube Obstruction ◽  
Pars Plana ◽  
Sclerotomy Site

Abstract Background: The purpose of this study is to report the safety and efficacy of pars plana (PP) glaucoma drainage devices (GDDs) with pars plana vitrectomy (PPV) using one of the vitrectomy sclerotomy sites for tube placement in patients with refractory glaucoma.Methods: Retrospective case series of 28 eyes of 28 patients who underwent combined PP GDD and PPV between November 2016 and September 2019 at Massachusetts Eye and Ear. Main outcome measures were intraocular pressure (IOP), glaucoma medication burden, best corrected visual acuity (BCVA), and complications. Statistical tests were performed with R and included Kaplan-Meier analyses, Wilcoxon paired signed-rank tests, and Fisher tests.Results: Mean IOP decreased from 22.8 mmHg to 11.4 mmHg at 1.5 years (p = 0.009), and mean medication burden decreased from 4.3 to 1.7 at 1.5 years (p = 0.009). Both IOP and medication burden were significantly lower at all follow-up time points. The probability of achieving 5 ≤ IOP ≤ 18 mmHg with at least 20% IOP reduction from preoperative levels was 77.7% at 1 year and 45.8% at 1.5 years. At their last visit, four eyes (14.3%) achieved complete success with IOP reduction as above without medications, and 13 eyes (46.2%) achieved qualified success with medications. Visual acuity was unchanged or improved in 23 eyes (82.1%) at their last follow-up. Two patients had a visual acuity decrease of >2 lines. Two eyes required subsequent PPV for tube obstruction, and one eye had transient hypotony.Conclusions: The results of pars plana GDD and vitrectomy using one of the vitrectomy sclerotomy sites for tube placement are promising, resulting in significant IOP and medication-burden reductions through postoperative year 1.5 without additional risk of postoperative complications. Inserting GDDs into an existing vitrectomy sclerotomy site may potentially save surgical time by obviating the need to create another sclerotomy for tube placement and suture one of the vitrectomy ports.

Best Vitelliform Macular Dystrophy (BVMD) is a phenocopy of North Carolina Macular Dystrophy (NCMD/MCDR1)

2021 ◽  
pp. 1-11
Author(s):  
Kent W. Small ◽  
Lee M. Jampol ◽  
Benjamin Bakall ◽  
Leslie Small ◽  
Robert Wiggins ◽  
...  
Keyword(s):  
North Carolina ◽  
Macular Dystrophy ◽  
Vitelliform Macular Dystrophy ◽  
Best Vitelliform Macular Dystrophy
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