Morphology and Vascular Layers of the Choroid in Stargardt Disease Analyzed Using Spectral-Domain Optical Coherence Tomography

Abstract

Purpose: To analyze total thickness, morphology and individual vascular layers of the choroid in eyes with Stargardt disease using spectral-domain optical coherence tomography (SD OCT).

Design: Cross-sectional retrospective review.

Methods: Twenty-eight patients with Stargardt disease (53 eyes) with a mean age of 46 (15-79) years and 30 healthy subjects (30 eyes) with a mean age of 49 (22-79) years who underwent 1-line raster scanning with SD OCT were identified. Diagnosis of Stargardt disease was based on ophthalmic history and complete ophthalmic evaluation. The healthy subjects had best-corrected visual acuity of 20/20 or better with no chorioretinal pathology. Two independent raters assessed the total thickness, morphology, and the individual vascular layers of the choroid.

Results: The choroid was irregularly shaped in 26 of 41 eyes (64%) with Stargardt disease when compared to 0 of 30 healthy eyes (0%). Mean subfoveal total choroidal thickness and mean subfoveal large choroidal vessel layer thickness were significantly reduced in eyes with Stargardt disease when compared to healthy eyes (272.8 ± 32.8 μm vs 225.4 ± 69.9 μm; P = .03 and 219.5 ± 30.6 vs169.2 ± 70.1; P = .04, respectively). The maximal choroidal thickness was subfoveal in 9 of 41 eyes (22%), focal choroidal thinning was observed in 21 of 41 eyes (51%), and attenuation of large choroidal vessel layer was observed in 8 of 41 eyes (20%) with Stargardt disease. There was no association of the best-corrected visual acuity with any choroidal morphologic feature, except that it was better by a mean of 0.61 ± 0.21 in eyes that had preservation of large choroidal vessel layer (33 of 41, 80%) when compared to those that had attenuation of large choroidal vessel layer (P = .007).

Conclusion: This study shows alterations in the total thickness, morphology, and the individual vascular layers of the choroid in eyes with Stargardt disease on SD OCT. These findings may potentially contribute to the clinical staging and monitoring of Stargardt disease.