Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware, USA.
Curr Opin Ophthalmol. 2019 Sep;30(5):301-305. doi: 10.1097/ICU.0000000000000597.
Sturge-Weber syndrome (SWS) is a rare, congenital disease which frequently involves the eye. It is important that ophthalmologists recognize this syndrome and are aware of its range of ocular manifestations. The aim of this article is to present our understanding of the pathogenesis and clinical manifestations of this syndrome and provide updated information on the treatment of SWS glaucoma and choroidal hemangioma.
SWS glaucoma usually fails medical management. Surgical options include angle procedures, filtering procedures, device placement, and combination procedures. Combination procedures have become popular in this population due to the single procedure failure rate of angle surgery and the complications associated with device implantation. Choroidal hemangioma is best treated by photodynamic therapy.
Lifelong monitoring for ocular complications related to SWS is essential. There is a need for consensus guidelines on care and surveillance of patients with SWS to provide the best care for these patients.
Sturge-Weber 综合征(SWS)是一种罕见的先天性疾病,常累及眼部。眼科医生识别该综合征并了解其眼部表现范围非常重要。本文旨在介绍我们对该综合征发病机制和临床表现的理解,并提供 SWS 青光眼和脉络膜血管瘤治疗的最新信息。
SWS 青光眼通常对药物治疗无效。手术选择包括房角手术、滤过手术、设备植入和联合手术。由于角手术单一手术失败率以及与设备植入相关的并发症,联合手术在该人群中越来越受欢迎。光动力疗法是治疗脉络膜血管瘤的最佳方法。
终生监测与 SWS 相关的眼部并发症至关重要。需要制定关于 SWS 患者护理和监测的共识指南,为这些患者提供最佳护理。