Investigation of Ocular Hemodynamics in Sturge-Weber Syndrome

Abstract

PURPOSE: Sturge-Weber syndrome (SWS) is a condition often associated with facial cutaneous angioma, vascular malformations in the brain, and ocular anomalies such as glaucoma. Reduced cerebral blood flow and ischemia have been well documented. Less is known about ocular blood flow despite the frequent associations between altered hemodynamics and the mechanisms underlying glaucomatous optic neuropathy. The aim of this research was to investigate retrobulbar hemodynamics in patients diagnosed with SWS. METHODS: The sample comprised 16 patients diagnosed with SWS and 16 age- and gender-matched normal control subjects. Four patients were diagnosed with both SWS and primary open-angle glaucoma (mean age 34.3 years; SD 26.9 years), three patients with both SWS and closed-angle glaucoma (mean age 23.3 years; SD 18.0 years), and nine patients with SWS and no glaucoma (mean age 17.2 years; SD 9.1 years). Systemic blood pressure and intraocular pressure were measured to determine the mean arterial pressure and ocular perfusion pressure. All patients and subjects underwent ultrasonography of the ophthalmic artery, central retinal artery, and short posterior ciliary arteries. RESULTS: No significant difference between groups for mean arterial pressure or ocular perfusion pressure (p > 0.05) was recorded. Participants diagnosed with SWS and primary open-angle glaucoma showed significantly reduced end-diastolic velocity (mean 0.036 m/s; SD 0.005 m/s) in their central retinal artery (p = 0.016) when compared against their age-matched normal controls (mean 0.054 m/s; SD 0.010 m/s). Participants diagnosed with SWS and no glaucoma also showed significantly reduced end-diastolic velocity (mean 0.038 m/s; SD 0.015 m/s) in their central retinal artery (p = 0.046) when compared against their age-matched normal controls (mean 0.054 m/s; SD 0.014 m/s). CONCLUSIONS: Retrobulbar hemodynamics appear to be altered in participants diagnosed with SWS irrespective of their diagnosis of glaucoma. Further research is needed to ascertain whether there are any long-term consequences of such changes to ocular physiology.