双上转肌麻痹的手术治疗

Surgical treatment of congenital ocular muscle palsy characterized with double elevator dysfunction

目的探讨双上转肌麻痹的有效手术治疗方法。方法对近5年来收治的6例双上转肌麻痹患者进行回顾性分析,对其临床特征、手术方式及效果进行总结。结果6例患者中男性2例,女性4例,年龄8～20岁,均为自幼患病,单眼受累,麻痹眼存在假性上睑下垂,垂直斜度25～50△,2例合并有15～20△外斜视,2例存在代偿头位,下颏上举。3例行下直肌退后联合上直肌缩短,3例行内外直肌移位术,其中2例3个月后二期行下直肌退后术,合并存在的水平斜视同时矫正。术后眼位正,代偿头位及假性上睑下垂改善。结论先天性双上转肌麻痹是临床较为少见的一种眼球运动障碍性疾病,可能是神经肌肉发育不良所致。根据麻痹特征选择手术方式,通常可获得良好的矫正效果。

Objective To investigate the clinical features of congenital ocular elevator muscle palsy and to evaluate the efficacy of surgical treatment. Methods A retrospective study were made in 6 patients with congenital double elevator palsy of ocular muscles, the clinical characters, surgical treatments and outcomes were investigated. Results Among the 6 patients, 2 male and 4 female, aged 8-20. When fixated with the nonparetic eye, patients showed a 25-50 hypotropia and pseudoblepharoptosis in the primary position ; Two of 6 patients had exotropia of 15-20; Two patients had a chin-up abnormal head position. For surgical treatment, three patients were undergone inferior rectus recession and superior rectus resection. The other three patients were undergone transposition of horizontal rectus, 2 of them were undergone a recession of inferior rectus three months later. The existed extropia were corrected at the same time. Strabismus were corrected in all patients, and the psuedoptosis and abnormal head position disappeared. Conclusion Congenital double elevator dysfunction is a unusual dysfunction of ocular motility, which may be due to the hypogenesis of nerve and extraocular muscle. Satisfied results could be obtained due to appropriated surgical treatment.