To visualize and investigate intraretinal changes in macular dystrophies with ultrahigh resolution optical coherence tomography (UHR OCT).

Prospective observational case series.

setting: Department of Ophthalmology and Center for Biomedical Engineering and Physics, Christian Doppler Laboratory, Medical University of Vienna, Vienna, Austria. patients: Thirteen patients (23 eyes) with adult-onset foveomacular vitelliform dystrophy (AOFVD) and 14 patients (27 eyes) with Stargardt’s disease (SD) or fundus flavimaculatus (FF).

Imaging using a compact, new generation UHR OCT system, achieving considerably improved visualization of intraretinal layers, especially the photoreceptor layer. main outcome measures: UHR OCT tomograms visualizing intraretinal differences in morphology of AOFVD and SD/FF as location and extension of deposits and loss of photoreceptors. Central foveal thickness defined as distance between internal limiting membrane and photoreceptors/retinal pigment epithelium interface.

Patients with AOFVD had a mostly intact photoreceptor layer, a central foveal thickness of 142 ± 23 μm as well as subretinal deposits. Patients with SD generally had a diffuse degenerative change with a visible reduction in thickness of all intraretinal layers, resulting in a corresponding reduction of central foveal thickness (94 ± 38 μm) and central loss of photoreceptors (PRs). Comparative central foveal thickness of patients with AOFVD and SD/FF was significantly different (P < .001). Patients with FF had pigment epithelial deposits and paracentral focal photoreceptor loss.

UHR OCT is a clinically feasible tool for examining intraretinal changes, in particular photoreceptor atrophy in macular dystrophies and, therefore, has the potential to be an adequate imaging system for monitoring the course of disease.