PURPOSE: Ocular manifestations of Sturge-Weber syndrome (SWS) include choroidal hemangioma and glaucoma. Intraocular pressure (IOP) reduction in these patients commonly is associated with sight-threatening choroidal effusions. Oral propranolol is the standard of care for infantile cutaneous hemangioma, but its role in choroidal hemangioma largely is unexplored. We studied the role of perioperative oral propranolol during glaucoma surgery in SWS. DESIGN: Prospective, nonrandomized case series with historical controls. PARTICIPANTS: Fourteen eyes of 12 patients with SWS scheduled for glaucoma surgery were included, and the outcomes were compared with those of historical controls without propranolol use (15 eyes of 14 patients). METHODS: Patients in the prospective cohort received oral propranolol 2 mg/kg of body weight daily in 2 divided doses 1 week before surgery and continued for 6 weeks after surgery. There was no modification (e.g., posterior sclerotomy) in the existing surgical technique. The historical control group was identified from records and SWS diagnosis validated by chart review. MAIN OUTCOME MEASURES: The incidence and extent of postoperative choroidal effusion, additional procedures required compared with the control group, and adverse effects of the drug in the prospective cohort. RESULTS: Average follow-up was 25.7±12.1 months (95% confidence interval, 19.3-32.1 months). The intraocular pressure reduced from 25.2±9.7 mmHg at presentation to 16.25±6.2 mmHg, 14.6±4.5 mmHg, 13.7±6.4 mmHg, and 16.5±8.0 mmHg at 1 week, 1 month, 3 months, and 1 year after surgery, respectively. In the perioperative propranolol group, no patient demonstrated sight-threatening choroidal effusion within the vascular arcades. In the 2 patients with bilateral disease, both eyes of each patient showed peripheral choroidal effusion, which settled with medical treatment. Surgery was a repeat procedure in 3 of the 4 eyes. There were no adverse effects of propranolol in any patient. In the control group, 5 of 12 eyes showed peripheral choroidal effusion after primary glaucoma surgery, whereas 5 of 6 eyes that underwent repeat surgery failed demonstrated sight-threatening choroidal effusion requiring surgical intervention. CONCLUSIONS: Oral propranolol seems to be an effective method to minimize the development of sight-threatening choroidal effusion after glaucoma surgery in SWS.
PURPOSE: Ocular manifestations of Sturge-Weber syndrome (SWS) include choroidal hemangioma and glaucoma. Intraocular pressure (IOP) reduction in these patients commonly is associated with sight-threatening choroidal effusions. Oral propranolol is the standard of care for infantile cutaneous hemangioma, but its role in choroidal hemangioma largely is unexplored. We studied the role of perioperative oral propranolol during glaucoma surgery in SWS. DESIGN: Prospective, nonrandomized case series with historical controls. PARTICIPANTS: Fourteen eyes of 12 patients with SWS scheduled for glaucoma surgery were included, and the outcomes were compared with those of historical controls without propranolol use (15 eyes of 14 patients). METHODS:Patients in the prospective cohort received oral propranolol 2 mg/kg of body weight daily in 2 divided doses 1 week before surgery and continued for 6 weeks after surgery. There was no modification (e.g., posterior sclerotomy) in the existing surgical technique. The historical control group was identified from records and SWS diagnosis validated by chart review. MAIN OUTCOME MEASURES: The incidence and extent of postoperative choroidal effusion, additional procedures required compared with the control group, and adverse effects of the drug in the prospective cohort. RESULTS: Average follow-up was 25.7±12.1 months (95% confidence interval, 19.3-32.1 months). The intraocular pressure reduced from 25.2±9.7 mmHg at presentation to 16.25±6.2 mmHg, 14.6±4.5 mmHg, 13.7±6.4 mmHg, and 16.5±8.0 mmHg at 1 week, 1 month, 3 months, and 1 year after surgery, respectively. In the perioperative propranolol group, no patient demonstrated sight-threatening choroidal effusion within the vascular arcades. In the 2 patients with bilateral disease, both eyes of each patient showed peripheral choroidal effusion, which settled with medical treatment. Surgery was a repeat procedure in 3 of the 4 eyes. There were no adverse effects of propranolol in any patient. In the control group, 5 of 12 eyes showed peripheral choroidal effusion after primary glaucoma surgery, whereas 5 of 6 eyes that underwent repeat surgery failed demonstrated sight-threatening choroidal effusion requiring surgical intervention. CONCLUSIONS: Oral propranolol seems to be an effective method to minimize the development of sight-threatening choroidal effusion after glaucoma surgery in SWS.