先天性小睑裂综合征临床手术疗效分析

Analysis of surgical treatment for congenital blepharophimosis syndrome

目的探讨先天性小睑裂综合征的手术治疗效果。方法回顾性分析先天性小睑裂综合征患儿23例（46眼），年龄2～11岁，均伴有其他眼疾或全身疾病。所有患儿同期行双眼内眦赘皮矫正＋额肌腱膜悬吊术。内眦赘皮矫正采用的术式有4种，其中Y—V成形术2例、Stallars“Z”成形术7例、Speath术式5例及经典的Mastands内眦成形术9例。上睑下垂矫正采用额肌腱膜悬吊术。术后随访1～24个月，观察睑裂长度、睑裂高度、内眦间距及眉睑距等参数变化。术后1、6、18个月分别检查三棱镜斜视度。采用配对t检验对手术前后患者的睑裂长度、睑裂高度、内眦间距及眉睑距等进行比较。结果23例患儿均顺利完成手术。术后睑裂长度及高度均有明显提高，内眦间距及眉睑距明显缩小。术后1个月，睑裂长度由术前的（18．5±1．1）mm增长至（23．4±1．2）mm，睑裂高度由术前的（2．1±1．2）mm增高至（6．7±0．9）mm，内眦间距由术前的（3512±1．4）mm减小至（31．1±1．1）mm，眉睑距由术前的（13．6±1.3）mm减小至（9．8±1．2）mm，与术前比较，差异均有统计学意义（t分别为4．985、10．832、2．143和4．171，P均〈0．05）。术后1个月，仅见暴露性角膜炎1例，未见其他并发症。术后6、18个月的随访数据与术后1个月比较，差异均无统计学意义（P〉0．05），表明手术疗效在术后1个月时保持稳定。结论对于先天性小脸裂综合征，早期双跟周期行内眦赘皮矫正联合额肌腱膜悬吊术是比较理想的治疗手段．

Objective To investigate the outcome of the surgical treatment for congenital blepharophimosis syndrome. Methods This was a retrospective case study to analyze a treatment approach for congenital blepharophimosis syndrome and its clinical efficacy. A bilateral correction of the epicanthus and blepharoptosis was performed simultaneously. Suitable methods were adopted to correct the epicanthus： Y-V, Stallars ＂Z＂, Speath and classic Mastands. Twenty-three patients （46 eyes） were involved in the study, ranging in age from 2 to 11 years. All patients suffered from eye or systemic disease. There were cases of strabismus, refractive error, amblyopia, inherited and systemic disease. The study analyzed effectiveness by comparing the preoperative and postoperative status of palpebral fissure length, palpebral fissure width, inner eanthic diameter and eyebrow-eyelid diameter using a paired t-test. Patients were followed up for 1 to 2d months after the operation. Results There was an obvious improvement in palpebral fissure length and palpebral fissure width in all patients, and an obvious decrease in the inner canthic diameter and eyebrow-eyelid diameter. Comparisons one month after the operation showed that palpebral fissure length changed from （18.5±1.1）mm to （23.4±1.2）mm, palpebral fissure width changed from （2.1±1.2）mm to （6.7±0.9）mm, inner canthic diameter changed from （35.2±1.4）mm to （31.1±1.1）mm, and eyebrow-eyelid diameter changed from （13.6±1.3）mm to （9.8±1.2）mm. The differences were statistically significant （P〈0.05）. There was only one case with complication of exposure keratitis after the operation. Conclusion For congenital blepharophimosis syndrome with refractive errors and amblyopia, it is better to perform a bilateral correction of the epicanthus and blepharoptosis simultaneously, which is a more ideal approach than other treatments.