Color Doppler imaging of retrobulbar hemodynamics in Sturge-Weber syndrome-associated glaucoma

PURPOSE. Sturge-Weber syndrome (SWS) is frequently associated with early onset glaucoma in the eye on the same side as the facial angioma. The exact cause of glaucoma in SWS is poorly understood and difficult to treat. The purpose of this study is to investigate the ocular hemodynamics of children with SWS-associated glaucoma using color Doppler imaging techniques. METHODS. This is a prospective study of 10 pediatric patients with unilateral SWS-associated glaucoma. Color Doppler imaging was used to measure the peak systolic velocity and the end diastolic velocity of both the ophthalmic and central retinal arteries in the glaucomatous eye compared to the fellow healthy eye. RESULTS. Twenty eyes of 10 children with SWS (6 boys) with unilateral glaucoma were included in the prospective study. The mean age of the 10 participants was 5.5 years. When compared to their contralateral normal eyes, the glaucomatous eyes had greater CDR (p<0.001) and a myopic shift (p=0.04). No significant differences were found in the measurements of ocular blood flow velocities of the ophthalmic and central retinal arteries. CONCLUSIONS. Vascular pathology has been proposed to play a role in SWS glaucoma etiology. The authors did not find arterial retrobulbar blood flow differences between the glaucomatous and the fellow normal eye. Since the primary vascular anomaly in patients with SWS is in the venous plexus, a bigger prospective trial is warranted in order to better understand and treat children with SWS glaucoma.