Vu Daniel M, Gjerde Harald, Elhusseiny Abdelrahman M, Oke Isdin, VanderVeen Deborah K
Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts; Department of Ophthalmology, Massachusetts Eye and Ear, Harvard Medical School, Boston, Massachusetts.
Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts; BC Children's Hospital and University of British Columbia, Vancouver, British Columbia, Canada.
Ophthalmol Glaucoma. 2025 Mar-Apr;8(2):181-187. doi: 10.1016/j.ogla.2024.10.007. Epub 2024 Oct 22.
To identify which features of Sturge-Weber syndrome (SWS) were most associated with glaucoma onset, severity, and treatment failure at a tertiary care center.
Retrospective cross-sectional study.
Children who had SWS with and without glaucoma.
Electronic health records were reviewed for all children with SWS presenting between 2014 and 2020. Examination and imaging findings from dermatology, neurology, and ophthalmology were collected. Logistic regression was used to identify factors associated with glaucoma-related outcomes.
Primary outcomes included glaucoma development, progression to surgery, and treatment failure. Failure was defined as having a final intraocular pressure >21 mmHg, devastating complication, or ≤20/200 vision.
Twenty-three of 44 SWS patients (52.3%) developed glaucoma, and 6 of 23 patients (26.1%) had both eyes affected. Sixteen of 29 eyes (55.2%) required surgery, and 29.6% overall met our failure criteria (mean follow-up: 5.1 ± 4.3 years). Glaucoma diagnosis was associated with bilateral port-wine birthmarks (PWBs; odds ratio [OR] 5.9; 95% confidence interval [CI] 1.3-43.2), PWB with any lower eyelid involvement (OR 9.7, 95% CI 2.6-44.5), and choroidal hemangiomas (OR 3.8, 95% CI 1.1-13.8), but was not associated with upper eyelid or leptomeningeal angiomas, seizures, prior hemispherectomy, or pulsed-dye laser. Eyes that progressed to surgery were more likely to have PWB affecting the lower eyelid (OR 33.7, 95% CI 4.5-728.0). No clinical or demographic factors were associated with treatment failure. In most cases, angle surgery failed (72.7%) but was a temporizing measure before subconjunctival filtering surgery.
Lower eyelid and choroidal angiomas were associated with glaucoma diagnosis, suggesting a spatial relationship with SWS findings. However, leptomeningeal angiomas were not associated, possibly because these are further from the eye.
FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
确定在一家三级医疗中心，斯特奇-韦伯综合征（SWS）的哪些特征与青光眼的发病、严重程度及治疗失败最为相关。
回顾性横断面研究。
患有和未患有青光眼的SWS儿童。
对2014年至2020年间就诊的所有SWS儿童的电子健康记录进行回顾。收集皮肤科、神经科和眼科的检查及影像学检查结果。采用逻辑回归分析确定与青光眼相关结局相关的因素。
主要结局包括青光眼的发生、进展至手术治疗以及治疗失败。治疗失败定义为最终眼压>21 mmHg、出现严重并发症或视力≤20/200。
44例SWS患者中有23例（52.3%）发生青光眼，23例患者中有6例（26.1%）双眼受累。29只眼中有16只（55.2%）需要手术治疗，总体有29.6%符合我们的失败标准（平均随访时间：5.1±4.3年）。青光眼诊断与双侧葡萄酒色斑（PWBs；优势比[OR]5.9；95%置信区间[CI]1.3 - 43.2）、累及任何下眼睑的PWB（OR 9.7，95% CI 2.6 - 44.5）以及脉络膜血管瘤（OR 3.8，95% CI 1.1 - 13.8）相关，但与上眼睑或软脑膜血管瘤、癫痫发作、既往大脑半球切除术或脉冲染料激光治疗无关。进展至手术治疗的眼睛更可能有影响下眼睑的PWB（OR 33.7，95% CI 4.5 - 728.0）。没有临床或人口统计学因素与治疗失败相关。在大多数情况下，房角手术失败（72.7%），但它是结膜下滤过手术前的一种临时措施。
下眼睑和脉络膜血管瘤与青光眼诊断相关，提示与SWS表现存在空间关系。然而，软脑膜血管瘤与之无关，可能是因为它们距离眼睛较远。
在本文末尾的脚注和披露中可能会发现专有或商业披露信息。
