Objective To study the skills and efficacy one-stage operation skills for the treatment of congenital blepharophimosis syndrome. Methods From January 2004 to December 2011, 18 patients (36 sides) with congenital blepharophimosis syndrome were treated, including 11 males and 7 females, aged from 3 to 25 years (mean 13.6 years). They all had severe bilateral blepharoptosis. The palpebral muscle force dimension as well as vertical and transverse dimensions of the palpebral tissues were 0 - 3 mm, 2 - 4 mm and 14 - 22 mm, respectively. The intercanthal distance was 33 - 40 mm and the levator function was 0 - 3 mm. All patients underwent medial and lateral canthoplasty combined with blepharoptosis correction at one stage. Then they were followed up for 3 to 36 months (mean 24 months). Results All the 18 patients underwent medial and lateral canthoplasty combined with blepharoptosis correction at one stage. None of them had epicanthal fold. The postoperative transverse dimension and vertical dimension of the palpebral tissues were 25 - 32 mm and 6 - 9 mm, respectively. The intercanthal distance was 28 - 34 mm and the levator function was 4 - 6 mm; the supratarsal fold in the upper lid was natural. The follow-up of 3 - 36 months showed that none of the patients had complications of blepharelosis, ectropion, exposure keratitis, or cicatrization of obvious medal and lateral canthus, and were satisfied with the results. Conclusion One-stage surgical treatment of combined medial and lateral canthoplasty with blepharoptosis correction can achieve a good and reliable result for blepharophimosis syndrome with a shortened treatment time and lessened pain from surgery.%目的 探讨先天性睑裂狭小综合征一期手术技巧及手术效果.方法 2004年1月～2011年12月的先天性睑裂狭小综合征患者18例.其中男11例,女7例.年龄3～25岁,平均13.6岁.所有病例均具有逆向内眦赘皮,双跟重度上睑下垂,上睑提肌肌力0～3 mm,睑裂高度2～4 mm,睑裂长度14～22 mm,内眦间距33～40 mm,上睑动度1～3 mm.患者在局麻或全麻下采用内眦和外眦成形术联合上睑下垂矫正术一次性完成.术后随访3～36月,平均随访24月.结果 所有患者采用内眦和外眦成形术联合上睑下垂矫正术一次性完成.术后内眦赘皮消失,泪阜外露,睑裂长度25～32 mm,睑裂高度6～9 mm,内眦间距28～34 mm,上睑动度4～6 mm,重睑线自然美观.术后随访3～36月,效果良好.无睑内翻、睑外翻或暴露性角膜炎等并发症发生,内外眦无明显瘢痕形成.结论 内眦和外眦成形联合上睑下垂矫正术一次性手术矫正先天性睑裂狭小综合征,减少多次手术痛苦,手术效果确切可靠.
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