Outer retinal alterations associated with visual outcomes in Best vitelliform macular dystrophy.

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ID: 24294
2019
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Ranked #48 of 48 articles by views in American journal of ophthalmology

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Abstract
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.Retrospective cross-sectional study.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.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).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.
Reference Key
augstburger2019outeramerican Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Augstburger, Edouard;Orès, Raphaëlle;Mohand-Said, Saddek;Mrejen, Sarah;Keilani, Chafik;Antonio, Aline;Condroyer, Christel;Andrieu, Camille;Sahel, José-Alain;Zeitz, Christina;Audo, Isabelle;
Journal American journal of ophthalmology
Year 2019
DOI
S0002-9394(19)30403-9
URL
Keywords Keywords not found

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