目的：通过对先天性小睑裂综合征分期手术的疗效及患者满意度的分析，为临床治疗先天性小睑裂综合征的手术方式的选择提供参考。

方法：回顾性分析在上海交通大学附属第九人民医院眼科进修学习时处置的先天性小睑裂综合征分期手术的患者23例46眼，其中患者6例12眼有家族遗传史，2例4眼斜视，1例2眼眼球震颤。平均在5±1.9岁行Ⅰ期手术，6±2.7岁行Ⅱ期手术，手术分为Ⅰ期“Y-V”成形术、Von-Ammon外眦成形术和Ⅱ期额肌瓣悬吊手术，术后随访观察12mo，评价与分析其疗效并行患者满意度评分。

结果：睑裂长度(PFL)从术前右眼17.83±3.1mm，左眼17.87±2.9mm延长至术后右眼23.59±1.7mm，左眼23.59±1.8mm； 睑裂高度(PFH)从术前右眼2.91±1.4mm，左眼2.91±1.2mm提高至术后右眼7.89±0.9mm，左眼7.89±0.7mm； 内眦赘皮的长度从术前18.7±2.1mm减少至术后7.8±1.8mm ； 内眦赘皮的宽度从术前3.9±0.8mm减少至术后1.3±0.8mm； 内眦间距(IICD)从术前37.70±3.3mm缩短至术后30.70±2.6mm； 角膜反射光点至上睑缘的距离(MRD1)从术前右眼-1.67±1.6mm，左眼-1.80±1.6mm均上升至术后右眼+3.35±0.7mm，左眼+3.35±0.6mm； IICD/PFL从术前2.1±0.2下降至术后1.3±0.4。满意度评分47±2.1(满分60)分，患者对手术疗效及术后美容表现出较高满意度。

结论：先天性小睑裂综合征患者通过分期手术即Ⅰ期“Y-V”成形术、Ammon外眦成形术和Ⅱ期额肌瓣悬吊手术，能取得满意的疗效。

METHODS: A retrospective analysis of blepharophimosis-ptosis-epicanthus inversus syndrome staging surgeries included 23 cases(46 eyes), in which 6 patients(12 eyes)had family history, 2 patients(4 eyes)of strabismus, 1 patients(2 eyes)of nystagmus. First-stage surgery of “Y-V” plasty and Ammon canthoplasty were performed at average age of 5±1.9; second-stage surgery of frontal muscle flap suspension were performed at average age of 6±2.7. Postoperative follow-up lasted for 12mo. We evaluated and analyzed the efficacy, asked the patients to give the score to the surgeries.

RESULTS: Palpebral fissure length(PFL)was extended from 17.83±3.1mm in the right eye and 17.87±2.9mm in the left eye to 23.59±1.7mm and 23.59±1.8mm after surgeries. The palpebral fissure height(PFH)was increased from 2.91±1.4mm in the right eye and 2.91±1.2mm in the left eye to 7.89±0.9mm in the right eye and 7.89±0.7mm in the left eye after surgeries. The length of epicanthus was reduced from 18.7±2.1mm to 7.8±1.8mm after surgeries. The width of epicanthus was reduced from 3.9±0.8mm before surgeries to 1.3±0.8mm after surgeries. The inner canthal spacing(IICD)was shortened from 37.70±3.3mm before surgeries to 30.70±2.6mm after surgeries. The distance from the upper eyelid margin of the corneal reflector to the upper eyelid(MRD1)increased from -1.67±1.6mm of the right eye before surgery, and -1.80±1.6mm of the left eye to the postoperative +3.35±0.7mm in the right eye and +3.35±0.6mm in the left eye. IICD/PFL decreased from 2.1±0.2 to 1.3±0.4 postoperatively. The satisfaction score was 47±2.1(full score was 60), and the patient was more satisfied with the curative effect and postoperative beauty.

CONCLUSION: The surgical treatments of blepharophimosis-ptosis-epicanthus inversus syndrome can be divided into the first-stage of “Y-V” plasty and Ammon lateral canthoplasty and the second-stage frontal muscle flap, which can get satisfaction of the patients.