Spectral Domain Optical Coherence Tomography of Choroidal and Outer Retinal Layer Thickness in the Sturge Weber Syndrome

ABSTRACT

Purpose: To evaluate choroidal thickness and its effect on the outer retinal layers in patients with Sturge Weber syndrome (SWS).

Materials and methods: Twenty eyes of 10 patients with SWS and 20 eyes of 10 healthy controls were evaluated at the ophthalmology unit of the Umberto I Policlinic, Rome from December 2015 to May 2015. Manual segmentation measurements of choroidal and retinal pigment epithelium (RPE)-photorec eptor layer (PHL) thickness were performed at the subfovea and at 500 µm intervals over 3 mm-long horizontal and vertical segments using enhanced depth spectral domain optical coherence tomography.

Results: Mean choroidal thickness of the affected (561.6 µm ± 208.8) and fellow eyes (322.0 µm ± 56.6) of patients with SWS was significantly higher with respect to controls (266.5 µm ± 48.5 µm), p = 0.001 and p = 0.017, respectively. Mean RPE-PHL thickness was significantly lower in both the affected and fellow eyes of patients with respect to controls (p = 0.039 and p = 0.025, respectively).

Conclusions: The choroid is thickened in patients with SWS, but the RPE-PHL is thinner. Choroidal thickening may lead to functional impairment causing disruption in the fine equilibrium between the choroid and retina and consequent outer retinal layer thinning.

KEYWORDS:

• Choroid
• enhanced depth imaging
• retinal pigment epithelium-photoreceptor layer
• Sturge Weber syndrome
• spectral domain optical coherence tomography

Acknowledgments

The authors are grateful to Alessia Mammone, PhD in statistics, CIBB-Centre for Biostatistics and Bioinformatics, University of Rome “Tor Vergata,” Rome, Italy, for the statistical analysis.

Conflicts of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper. No financial support was received.