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ELLIPSOID ZONE MAPPING AND OUTER RETINAL ASSESSMENT IN STARGARDT DISEASE
Sruthi Arepalli1, Elias I Traboulsi1, Justis P Ehlers1,2
1Cole Eye Institute, Cleveland Clinic, Cleveland, Ohio.
Retina (Philadelphia, Pa.)
|June 15, 2017
Summary
Stargardt disease significantly damages the ellipsoid zone and photoreceptor outer segments, impacting visual acuity. A new analysis tool quantifies these changes, revealing direct correlations between structural integrity and visual function.
Area of Science:
- Ophthalmology
- Retinal Degenerative Diseases
- Medical Imaging Analysis
Background:
- Stargardt disease is a leading inherited macular dystrophy.
- Progressive loss of photoreceptor cells, particularly in the macula, leads to central vision impairment.
- Quantifying structural changes in the outer retina is crucial for understanding disease progression and visual function.
Purpose of the Study:
- To quantify and correlate ellipsoid zone (EZ) and photoreceptor outer segment (OS) changes with visual acuity in Stargardt disease.
- To evaluate a novel analysis tool for assessing EZ and OS integrity.
Main Methods:
- Spectral domain optical coherence tomography (SD-OCT) was used to acquire macular cube data from 32 eyes with Stargardt disease and 12 control eyes.
- A novel analysis tool was employed for volumetric assessment of the EZ to retinal pigment epithelium (RPE) layer.
- Quantification of EZ/OS atrophy (EZ-RPE thickness = 0 μm) and attenuation (EZ-RPE thickness <20 μm) was performed and correlated with visual acuity.
Main Results:
- Patients with Stargardt disease showed significantly reduced central foveal EZ/OS area and thickness compared to controls.
- Higher percentages of EZ/OS atrophy and attenuation were observed in Stargardt disease eyes (22% vs. 1% and 43% vs. 1%, respectively).
- Visual acuity directly correlated with EZ/OS volume and inversely correlated with atrophy and attenuation (R = 0.57 and -0.53 to -0.57, P < 0.005).
Conclusions:
- Stargardt disease frequently causes significant disruption of the EZ and OS.
- The novel assessment platform successfully quantified these degenerative changes and identified correlates with visual function.
- This software offers a quantitative approach for assessing Stargardt disease and potential longitudinal monitoring.
