Mobius综合征长期手术效果观察  

Longterm surgery outcome of MiJbius syndrome

作　　者：岑洁[1] 亢晓丽[1] 于军 董凌燕[1] 韦严[1] 赵堪兴[2] 

机构地区：[1]上海交通大学医学院附属新华医院眼科,200092 [2]天津市眼科医院天津医科大学眼科临床学院天津市眼科研究所天津市眼科学与视觉科学重点实验室

出　　处：《中华眼科杂志》2017年第12期897-902,共6页Chinese Journal of Ophthalmology

基　　金：上海市卫生和计划生育委员会重点项目（201440037）

摘　　要：目的探讨Mfibius综合征的临床特征及手术治疗的长期效果。方法回顾性系列病例研究。收集2009至2014年于上海交通大学医学院附属新华医院眼科确诊为MObius综合征患者7例，其中男性5例，女性2例，初次就诊年龄平均（28．9±27．9）个月，所有患者均行头颅MRI检查、斜视角检查、眼球运动检查和全身麻醉后被动牵拉试验。7例第一眼位内斜视患者早期接受内斜视矫正术，观察术前与术后眼位、斜视度数、眼球运动的变化。对手术前后斜视角度的比较采用配对t检验。结果所有患者头颅MRI均显示单侧或双侧外展神经和面神经缺如或发育不良。7例患儿首次手术均行超常量内直肌后徙术，每只眼平均（6．17±1．47）mm，术后6个月之内斜视度数回退。术前原在位内斜〉-40°患者（3例）欠矫明显，需要二次手术。术前及末次随访原在位平均斜视度数分别为＋35．00°±16．58°和＋2．14°±5．67°，差异有统计学意义（t=6．040，P〈0．01）。眼球运动：患眼术前和术后均不能外转，内转术后较术前稍差。结论MObius综合征属于先天性颅神经支配异常性疾病的一种，由于外展神经先天缺如，因此手术方法首选内直肌减弱术，长期观察术后第一眼位情况良好。Objective To investigate clinical features and the long-term surgery results of Mobius syndrome patients. Methods This investigation presents a retrospective study of Mobius syndrome in 7 children we found since 2009. All 7 patients had MRI scan, ocular alignment in primary position, the limitation of versions and ductions and forced duction under general anesthesia. Early surgery is done to the 7 esotropia children. The pre-operative and post-operative outcomes, including the ocular alignment, deviation measurements and ocular rotations, were evaluated and compared. Results MRI showed absence of uni- or bilateral CN6 and CN7 in all 7 patients. All 7 patients underwent extra large recession of medial rectus at the first surgery, （6.17±1.47） mm/eye. Variation of ocular deviation in the primary position within 6 months postoperatively in all patients, demonstrating that strabismus surgical stabilization needs time. Three patients with esotropia deviation〉40° preoperative were under corrected and needed the secondary operations. The average deviation in the primary position was ＋35.00°± 16.58° before surgery and ＋2.14°± 5.67°after surgery（t=6.040, P〈0.01）. The abduction in affected eye is limited both pre and postoperatively and a smaller limitation of adduction after surgery. Conclusions M6bius syndrome has been classified to congenital cranial dysinnervation disorders （CCDDs）. We recommend MR recession as the first surgical choice because of the lack of abduction ability. Longterm surgical results were considered satisfactory, improving patient self-esteem and the parent satisfaction.

关 键 词：MOBIUS综合征 颅神经疾病 展神经 先天畸形 眼外科手术 

分 类 号：R779.6[医药卫生—眼科]