The prognostic value of optical coherence tomography biomarkers in Stargardt Disease

Author: Faruque Ghanchi (United Kingdom)

Co-authors: Mohammud Musleh, Kiran Ahmed, Faruque Ghanchi

Purpose

To describe longitudinal structural OCT and visual acuity changes in patients with Stargardt disease and evaluate OCT changes ass a predictive biomarker for Stargardt disease.

Setting/Venue

Electronic patient records (EPR) and digital images of patients diagnosed with Stargardt disease at a specialist medical retina/genetics eye clinic in large teaching hospital in UK.

Methods

Patients of any age with a diagnosis of Stargardt disease of any subtype were identified using hospital EPR Medisoft (Leeds, United Kingdom) and their digital retinal images and OCT scans were sourced from the image database. Baseline and follow up OCT (Spectralis OCT, Heidelberg Engineering, Heidelberg Germany) scans and documented visual acuity (VA) necessary for inclusion. Eyes with coexisting macular pathology were excluded. Data was systematically harvested and analyzed independently by two ophthalmologists. At each eligible visit, visual acuity, central retinal thickness (CRT), retinal pigment epithelium (RPE) and inner segment/outer segment (IS/OS) junction disruption sizes at the geographic centre of the macula were recorded. All discrepancies exceeding 100 microns were resolved by joint review or a third assessor if still remaining. A mean of these values was calculated and used for the data analysis. .

Results

Patients of any age with a diagnosis of Stargardt disease of any subtype were identified using hospital EPR Medisoft (Leeds, United Kingdom) and their digital retinal images and OCT scans were sourced from the image database. Baseline and follow up OCT (Spectralis OCT, Heidelberg Engineering, Heidelberg Germany) scans and documented visual acuity (VA) necessary for inclusion. Eyes with coexisting macular pathology were excluded. Data was systematically harvested and analyzed independently by two ophthalmologists. At each eligible visit, visual acuity, central retinal thickness (CRT), retinal pigment epithelium (RPE) and inner segment/outer segment (IS/OS) junction disruption sizes at the geographic centre of the macula were recorded. All discrepancies exceeding 100 microns were resolved by joint review or a third assessor if still remaining. A mean of these values was calculated and used for the data analysis. .

Conlusions

OCT can pick up photoreceptor and RPE disruption in Stargardt patients at baseline although visual acuity can be highly variable. A progressive increase in mean RPE defect size and IS/OS junction disruption size on the OCT scans at each follow up is associated with a decline in visual acuity. Central macular IS/OS and RPE defects on OCT images correlate with visual acuity. OCT scan using retinal photoreceptor- RPE complex appears to be a useful prognostic biomarker for vision. Further studies are needed to confirm this useful, non-invasive biomarker for Stargardt disease. Future studies with machine learning of these OCT biomarkers and structural OCT analysis can help with predicting course of the disease as well as outcome of treatments.

Financial Disclosure

None

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