Case Report: management of refractory glaucoma secondary to Sturge-Weber syndrome associated with ocular melanocytosis.

BACKGROUND

Glaucoma associated with Sturge-Weber syndrome (SWS) is widely regarded as one of the most challenging types of secondary glaucoma, with the lowest surgical success rates. In this report, we present a successful management of refractory glaucoma secondary to Sturge-Weber syndrome accompanied by ocular melanocytosis.

CASE REPORT: A 28-years-old female with a history of right eye glaucoma since childhood presented with poorly controlled intraocular pressure (IOP) despite multiple surgical interventions, including two trabeculectomies and one glaucoma drainage device implantation. She exhibited ipsilateral facial cutaneous port-wine stains and bluish-purple scleral pigmentation consistent with ocular melanocytosis (OM). Examination revealed a low corneal endothelial cell count and significant optic nerve atrophy (cup-to-disk ratio of 0.9) in the right eye. The patient underwent combined trabeculotomy- CO2 laser-assisted sclerectomy surgery (CLASS), which successfully reduced the IOP to below 10 mmHg with no complications and preserved visual acuity (VA).

CONCLUSION

This case demonstrates the efficacy of combined trabeculotomy-CLASS in refractory glaucoma associated with SWS and ocular melanocytosis, offering a promising surgical alternative for complex cases.