gms | German Medical Science

Purpose: This study investigates the clinical and surgical management of bilateral ocular Sturge-Weber syndrome (SWS), focusing on intraoperative challenges, postoperative outcomes, and the impact of multimodal interventions on long-term visual prognosis. Bilateral ocular involvement in SWS is rare (10–20% of cases), presenting significant diagnostic and treatment challenges, especially in managing severe glaucoma. Early and comprehensive interventions are crucial to managing aggressive glaucoma and preventing progressive visual impairment.

Methods: A case series that was conducted on patients with bilateral ocular SWS treated at a tertiary ophthalmic surgery centre over a 10-year period. Surgical procedures included trabeculectomy, glaucoma drainage devices (GDDs), and cyclophotocoagulation. Data on intraocular pressure (IOP), visual acuity, surgical complications, and repeat interventions were collected.

Results: Patients commonly presented with severe, refractory glaucoma. While trabeculectomy initially controlled IOP, 60% of eyes required GDD implantation within two years. Intraoperative challenges included vascular fragility, increasing the risk of suprachoroidal haemorrhage. Postoperatively, 75% of eyes maintained stable vision, while 25% required additional procedures due to complications. Multimodal interventions were essential to preserve vision and function.

Conclusions: Bilateral ocular SWS presents unique surgical challenges, especially due to vascular anomalies and glaucoma progression. Early surgical intervention, including trabeculectomy and GDDs, along with multidisciplinary care, is vital for preserving vision and minimizing complications. A personalized, comprehensive approach is necessary for optimal long-term outcomes.