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
53 patients with Stargardt disease were identified from the EPR, of which 59 eyes of 31 patients (average age 46 years, 15- 81, 48% male) were eligible for inclusion. (Exclusions: 13 missing OCT images, 6 missing data and 3 coexisting macular pathology). 29% (n=9) of patients had one clinic visit, 35% (n=11) had two visits, 13% (n=4) had three visits, 10% (n=3) had four visits and 10% (n=3) had six visits. The mean time interval between the baseline and sixth visit was 63.4 +/- 6.4 months. Average VA deteriorated from 0.94 LogMAR to 1.33 over the course of the patients visits. Correspondingly, a deterioration was reported in all OCT biomarkers (average CRT deteriorated from 110 to 84 microns, mean RPE defect from 4007 to 5039 microns and IS/OS junction defect from 4361 to 5820 microns. The VA at baseline ranged from 6/6 to Hand Movements. At individual eye level, longitudinal OCT scan showed progressive increase in RPE defect size as well as IS/OS junction disruptions with progressive loss of vision. In eyes with good baseline VA, progressive loss- especially of central island of IS/ OS junction was associated with significant loss of vision.
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. None of the Authors have a financial interest in the subject matter or receives money from any mentioned company.
Financial Disclosure
None
Comments
-
