Retinal Vascular Density and Vessel Diameter in Sturge–Weber Syndrome Assessed by OCT-Angiography

TL;DR

This study uses OCT-Angiography to find that retinal blood vessels in Sturge–Weber syndrome patients are denser and wider compared to healthy individuals.

Contribution

The study introduces OCTA as a novel tool to detect and monitor vascular changes specific to Sturge–Weber syndrome.

Findings

SWS patients showed significantly increased deep capillary plexus density compared to controls.

Vascular diameter was increased in multiple retinal plexuses in SWS patients.

OCTA can detect morphological vascular changes linked to disease progression.

Abstract

Background: Sturge–Weber syndrome (SWS) typically presents with a port-wine stain on the face, accompanied by leptomeningeal capillary malformations and ocular vascular anomalies. The aim of our study was to evaluate retinal vascular density and vessel diameter to better characterize the presence of vascular alterations. Methods: 15 patients diagnosed with Sturge–Weber syndrome and 15 healthy controls underwent comprehensive ophthalmologic evaluation, Optical Coherence Tomography (OCT) and Optical Coherence Tomography Angiography (OCTA), to evaluate the microvascular architecture of the retina and choroid. Results: Analysis of the processed images revealed a significant increase (p < 0.05 *) in the density of the deep capillary plexus in patients with SWS compared to healthy controls. Vascular diameter was found to be increased overall in several retinal vascular plexuses in SWS patients compared to controls, reaching statistical significance (p < 0.05 *) in the deep vascular complex. Conclusions: The findings from our analysis highlight the potential role of OCTA in predicting the progression or worsening of ocular diseases over time. The introduction of new assessment parameters offers additional perspectives in evaluating ocular health. Since this examination allows for the detection of changes in the morphology and density of blood vessels as revealed by OCTA, these changes can be correlated with disease progression and the effectiveness of therapies.

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