Episcleral hemangioma distribution patterns could be an indicator of trabeculotomy prognosis in young SWS patients

To explore the relationships between episcleral hemangioma distribution patterns and trabeculotomy prognosis in young Sturge-Weber syndrome (SWS) patients.

Sturge-Weber syndrome-induced glaucoma patients less than 4 years of age who underwent trabeculotomy in our Ophthalmology Department from February 2016 to June 2017 were included. Every patient could be divided into simple episcleral vascular abnormal network (SEVAN) or multiple episcleral vascular abnormal network (MEVAN) groups according to their episcleral hemangioma patterns. The intraocular pressure (IOP) was recorded during follow-up until the last visit.

Fifty eyes (forty-six patients) of SWS were included. Mean age of surgery was 12.6 ± 15.1 months (range 1-47 months). Twenty-six eyes were in the SEVAN group, while 24 eyes were in the MEVAN group. There were no significant differences between the two groups in demographic data before surgery (p > 0.05). IOPs at 12 months (p = 0.013) and 24 months (p = 0.002) were significantly different between the two groups. At the 24-month follow-up, the cumulative proportions of trabeculotomy success in the SEVAN and MEVAN groups were 89.7% and 36.0%, respectively. Patients with MEVAN and larger preoperative corneal diameter (per 0.5 mm) were more likely to experience failed trabeculotomy (hazards ratio [HR], 7.997 [95% CI, 1.640-38.996], p = 0.010; 1.853 [95% CI, 1.128-3.042], p = 0.015).

Hemangiomas exhibited different distribution patterns between SEVAN and MEVAN. Trabeculotomy had a poorer prognosis in young SWS patients with MEVAN than in those with SEVAN. In addition to anterior chamber angle anomalies, vascular factors may contribute to the aetiology of SWS early-onset glaucoma.