INTRODUCTION:Sturge-Weber syndrome (SWS) is a rare congenital disorder characterized by leptomeningeal angiomas, port-wine stains, and glaucoma, affecting up to 70 % of pediatric cases. Elevated episcleral venous pressure disrupts aqueous outflow, leading to a persistently high intraocular pressure (IOP). Conventional treatments, such as trabeculectomy and Ahmed valve implantation, often fail because of fibrosis, hypotony, and vascular anomalies. The PreserFlo MicroShunt, a minimally invasive glaucoma surgery device, offers controlled IOP reduction with fewer complications; however, its use in pediatric SWS remains unexplored.
CASE PRESENTATION:A 32-week-old female infant with SWS presented with tearing, photophobia, and elevated IOP (40 mmHg) in the right eye. Initial management included seizure control with levetiracetam. Examination under anesthesia revealed corneal edema, horizontal corneal enlargement, and significant axial length asymmetry. Given the risk of irreversible vision loss, an urgent PreserFlo implantation was performed.
DISCUSSION:Postoperative outcomes demonstrated a 42 % reduction in IOP, improved corneal clarity, and a stabilized axial length. Revision surgery successfully addressed iris obstruction of the device and restored aqueous flow. The device's minimally invasive nature and predictable outflow make it a promising alternative to traditional surgeries. However, pediatric ocular growth necessitates close monitoring to prevent complications, such as tube migration or obstruction.
CONCLUSION:This case highlights the first successful off-label use of PreserFlo in infants with SWS-associated glaucoma. Early intervention and careful postoperative management can help achieve sustained IOP control. However, further studies are needed to optimize its long-term efficacy in pediatric patients.