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目的 探讨提上睑肌缩短术联合 Mustarde 双 Z 形切除术矫正儿童小睑裂综合征(blepharo-phimosis-ptosis-epicanthus inversus syndrome,BPES)的疗效.方法 2015 年 3 月—2017 年 6 月,采用提上睑肌缩短术联合 Mustarde 双 Z 形切除术一期矫正 26 例儿童双眼 BPES.其中,男 16 例,女 10 例;年龄 4～14 岁,平均7 岁.均有典型的小睑裂四联征.7 例伴有鼻梁低平,20 例伴有弱视和斜视.睑裂长度(19.5±4.5)mm,睑裂宽度(2.5±1.6)mm,内眦间距(42.1±6.5)mm,提上睑肌肌力(5.5±1.3)mm.结果 术后切口均Ⅰ期愈合.23 例获随访,随访时间 2～12 个月,平均 10 个月.其中 2 例矫正不足、3 例过度矫正,6 例眼睑线弧度欠佳,均无眼睑内外翻和角膜炎发生.其余患儿均成功矫正上睑下垂和内眦赘皮,眼睑线弧度自然.术后 7 d 测量睑裂长度(27.2±1.9)mm,睑裂宽度(12.5±1.3)mm,内眦间距(29.4±2.6)mm;以上指标均较术前显著改善,差异有统计学意义(t=0.127,P=0.042;t=0.341,P=0.029;t=0.258,P=0.038).术后无因睑裂宽度、长度回退导致的成角畸形发生.结论 提上睑肌缩短术联合 Mustarde 双 Z 形切除术能达到一期矫正儿童 BPES 目的,可获得较好临床疗效.“,”Objective To investigate the effectiveness of levator muscle resection combined with Mustarde’s double Z-plasty to correct blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). Methods Be-tween March 2015 and June 2017, one-stage operation of levator muscle resection combined with Mustarde’s double Z-plasty were performed on 26 children with bilateral BPES. There were 16 boys and 10 girls with an average age of 7 years (range, 4-14 years). All patients marked the four typical signs of BPES. There were 7 cases accompanied with a low nasal bridge, and 20 cases with amblyopia and strabismus. The length of eye fissure was (19.5±4.5) mm, the width of eye fissure was (2.5±1.6) mm, the diameter of inner canthus was (42.1±6.5) mm, and the muscular strength of levator palpebrae superioris was (5.5±1.3) mm. Results All the incisions healed by first intention. Twenty-three patients were followed up 2-12 months, with an average of 10 months. Among which, 2 cases were less corrected, 3 cases were over corrected, 6 cases had poor curvature of the eyelid. No eyelid internal and external pronation or keratitis occurred. Amelioration of blepharoptosis and epicanthus was achieved in the other patients, and the double eyelid fold was naturally smooth. At 7 days after operation, the length of eye fissure was (27.2±1.9) mm, the width of eye fissure was (12.5±1.3) mm, and diameter of inner canthus was (29.4±2.6) mm, which were superior to preoperative values (t=0.127, P=0.042; t=0.341, P=0.029; t=0.258, P=0.038). There was no angular deformity caused by the width and length regressions of eye fissures. Conclusion The levator muscle resection combined with Mustarde’s double Z-plasty can effectively correct BPES and obtain good effectiveness.