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

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.