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CASE REPORT article

Front. Med.

Sec. Ophthalmology

This article is part of the Research TopicProgress and Perspectives in Surgical Management of GlaucomaView all 4 articles

Case Report: Management of Refractory Glaucoma Secondary to Sturge-Weber Syndrome associated with Ocular Melanocytosis

Provisionally accepted
Yan  ZhouYan ZhouGangwei  ChengGangwei Cheng*Erqian  WangErqian WangYang  ZhangYang ZhangRongping  DaiRongping DaiAiling  BianAiling BianZaihong  WengZaihong Weng
  • Peking Union Medical College Hospital (CAMS), Beijing, China

The final, formatted version of the article will be published soon.

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-year-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-disc 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.

Keywords: Refractory Glaucoma 1, Sturge-Weber syndrome 2, ocular melanocytosis 3, class 4, trabeculotomy 5

Received: 02 Apr 2025; Accepted: 14 Nov 2025.

Copyright: © 2025 Zhou, Cheng, Wang, Zhang, Dai, Bian and Weng. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Gangwei Cheng, chenggw@pumch.cn

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