先天性眼外肌纤维化综合征的手术治疗

Surgical management of patients with congenital fibrosis of the extraocular muscles

目的探讨治疗先天性眼外肌纤维化综合征的手术方法和疗效。方法术前行被动牵拉试验，垂直斜视眼均行垂直直肌悬吊和（或）断腱术，伴水平斜视眼同时行水平直肌悬吊和（或）断腱术，退后缝合球结膜，二期行上睑下垂矫正术。结果共23例病人，22例术后眼位和头位均有改善，1例术后斜视无变化，其中8例二期行上睑下垂矫正术，术后头位和外观明显改善。结论先天性眼外肌纤维化综合征手术治疗是有效的。由于患者临床表现各不相同，对每个患者应进行个性化治疗。

Objective To evaluate the method and effect of the surgery for congenital fibrosis of the extraocular muscles（CFEOM）, Methods A careful forced duction test was done before the surgery. The patients with vertical strabismus were corrected by vertical muscle suspension or tendotomy and conjunctiva recession, combined with horizontal muscle suspension or tendotomy in some cases with horizontal strabismus simultaneously, Ptosis was repaired by levator resection or brow suspension in the second procedure. Results Among 23 cases, 22 cases had improved in strabismus and/or head postin after surgery, but strabismus hasn＇t change in one case; 8 cases had marked improvement of head position and appearance after ptosis was repaired in the second procedure. Conclusion Surgery is effective at improving CFEOM. Because of the wide clinical variation in strabismus between patients, the surgical approach should be individualized to each patient.