Objective: To investigate the clinical characteristics of Sturge-Weber syndrome (SWS) in the patients with Port-wine stain (PWS). Methods: A total of 279 PWS patients, 164 males, 115 females with a median age of first visit 17.00 (4.75, 56.00) months. Most of the PWS patients were referred to the Ophthalmology Department for screening eye problems when the cutaneous angiomas involved the ophthalmic division of the trigeminal nerve distribution. The intraocular pressure (IOP), cup to disk ratio (C/D), corneal condition and other essential measurements were examined to screen glaucoma or choroidal hemangioma. The differences of age, gender and vascular ectasia in the ipsilateral eyes were compared among PWS and SWS patients with chi-square test. The differences about the first visit time, IOP, C/D and corneal diameters were evaluated with independent-sample T test or nonparametric test followed by Mann-Whitney U test. Results: A total number of 66 out of 279 PWS patients (23.7%) were confirmed as SWS with glaucoma. The IOP of the ipsilateral eye with vascular ectasia in PWS and SWS was 13.00 mmHg (1 mmHg=0.133 kPa) (IQR: 9.75, 17.00) and 23.00 mmHg (20.00, 32.00), respectively (Z=-8.212, P<0.001); the IOP differences between the ipsilateral and contralateral eye in PWS and SWS was 1mmHg (0, 2) and 7 mmHg (3, 11) respectively; the C/D in the ipsilateral eye and the contralateral eye was 0.30 (0.30, 0.35) and 0.7 (0.6, 0.8) respectively in SWS cases with secondary glaucoma. Conclusions: There is a high proportion of SWS with glaucoma in ophthalmic division affected PWS patients. Fundus examinations were necessary for this type of patients. (Chin J Ophthalmol, 2017, 53:753-757).
目的： 探索葡萄酒色斑（PWS）患者中Sturge-Weber综合征（SWS）继发性青光眼患者的临床特征。 方法： 回顾性系列病例研究。共纳入2011年9月至2016年2月来上海交通大学医学院附属第九人民医院眼科就诊的连续PWS患者279例，其中男性164例，女性115例，初诊时中位数月龄17.00（4.75，56.00）。大部分患者为整复外科转诊至眼科，转诊指征为沿三叉神经眼支分布的血管畸形。对所有就诊患者进行眼压测量、杯盘比测量、角膜观察、测量及其他必要的检查以筛查SWS造成的青光眼及其他眼部异常。年龄、性别、血管畸形受累眼别等频数分布情况进行卡方检验，正态分布数据采用t检验，非正态分布数据采用非参数检验。 结果： 在279例PWS患者中共筛查出66例（23.7%）SWS继发性青光眼患者。PWS不伴随青光眼患者血管畸形同侧眼的眼压和杯盘比分别为13.00 mmHg（9.75，17.00）（1 mmHg=0.133 kPa）和0.30（0.30，0.35），SWS继发性青光眼患者则分别23.00 mmHg（20.00，32.00）和0.7（0.6，0.8），差异均有统计学意义（Z=-8.212，P<0.001；Z=-10.272，P<0.001）。在SWS继发性青光眼患者中，血管畸形累及侧眼与对侧眼的眼压差为7 mmHg（3，11）与PWS不伴随青光眼患者双眼眼压之差1 mmHg（0，2）相比差异具有统计学意义（Z=-6.761，P<0.001）。 结论： 在血管畸形累及三叉神经眼支的PWS患者中具有较高的青光眼比例，针对这类患者需要常规进行眼底检查，对于婴幼儿患者，镇静下的眼底检查同样是非常必要的。（中华眼科杂志，2017，53：753-757）.
Keywords: Glaucoma; Intraocular pressure; Port-wine stain; Sturge-weber syndrome; Vascular malformations.