Abstract
Purpose :
Sturge-Weber Syndrome (SWS) is a rare neurological disorder characterized by seizures, intellectual delay, leptomeningeal angiomas, choroidal hemangiomas, and a characteristic port-wine stain in the trigeminal nerve distribution. 30-70% of patients present with glaucoma ipsilateral to their port-wine stain with associated visual deficits. We undertook a comparison of iris and anterior segment parameters between SWS and control patients to describe different structural patterns.
Methods :
Subjects were aged 0.25-12.5 years. Controls were age-matched pediatric subjects with normal eye exam undergoing general surgical procedure. Six subjects with SWS glaucoma and 13 controls were enrolled. Ultrasound biomicroscopy (UBM) images (n=58) of the anterior segment were analyzed using ImageJ software by an observer masked to glaucoma status.
Results :
Five iris measurements were found to be significantly different between SWS glaucoma and control eyes. Glaucomatous eyes had flat, non-convex irises (p<0.0001) compared to controls (0.168 mm for controls and 0 for SWS) and were thinner at their maximum pupillary point (p=0.028; 0.900 mm for controls and 0.548 mm for SWS). Glaucomatous eyes were also significantly less dense at the peripupillary, minimal, and maximal thickness points (p<0.01 for all 3 variables). When assessing anterior chamber, SWS patients with glaucoma were found to have a wider ciliary body-corneal angle (p=0.02) than controls.
Conclusions :
Sturge-Weber Syndrome is one of many etiologies on congenital glaucoma, and it is important to understand the anomalous iris and angle anatomy associated with the disorder. Iris thickness, iris convexity, and angle width may be markers for glaucoma severity. In the future, these associations can improve diagnosis with ultrasound biomicroscopy and lead to improved treatment heuristics in the context of this severe, progressive neurological disorder.
This abstract was presented at the 2024 ARVO Imaging in the Eye Conference, held in Seattle, WA, May 4, 2024.