先天性眼外肌纤维化临床特征分析

目的 对住院行手术治疗的先天性眼外肌纤维化(CFEOM)病历进行临床总结,以加深对此病的认识.方法 回顾性病例分析.对40例(75只眼)CFEOM患者的病历资料进行回顾性总结和分析,包括性别、手术年龄、屈光状态、视力、分型、斜视度、眼部和全身合并症、家族史和手术方法.结果 在40例患者中,男26例,女14例,性别比约为2∶1;手术年龄2～36岁;在有屈光记录的60眼中,单纯远视7只眼,占11.67％;单纯近视2只眼,占3.33％;散光51只眼,占85.00％;在有矫正视力记录(不包括高度近视及视神经病变者)的56只眼中,弱视占94.64％.CFEOM 1型29例,占72.5％;CFEOM 3型10例,占25.0％;CFEOM 2型1例,占2.5％.有家族史者10例,占25％.手术方式:根据斜视方向及肌肉纤维化情况选择肌肉后徙、后徙悬吊或断腱减弱及相应结膜后徙术,相应的拮抗肌若被动牵拉试验没有明显限制,可考虑该肌的加强术;62只眼术后斜视改善,3只眼术后斜视无变化,2只眼垂直斜视改善水平斜视过矫.结论 CFEOM属于少见的先天性颅神经支配异常性疾病(CCDDs),在本资料中,散光性屈光不正者比例较大,弱视发生率较高,CFEOM 1型居多,多数患者手术可使斜视明显改善.

A clinical analysis of 40 cases with congenital fibrosis of extraocular muscles

Objective To analyse the clinical manifestations and the effects of surgical management on Congenital fibrosis of extraocular muscloes (CFEOM).Methods Forty cases with CFEOM were retrospectively summarized.The follows were recorded and analysed,including sex,surgical age,visual acuity,refrective condition,phenotype,the diopter,the complications of eye and other position of the body,familial history,surgical methods.Results In the 60 eyes having refrective record,7 eyes were simple hyperopia ( 11.67％ ),2 eyes were simple myopia (3.33％) ;51 eyes is astigmatism (85.00％) ;Amblyopia were 94.64％.29 cases were CFEOM1 (72.5％ ) ;10 cases were CFEOM 3(25.0％ ) ;1 case was CFEOM 2(2.5％ ).10 cases had familial history(25％ ).The methods of surgery include recession,hang-back or tenotomy of the rectus muscles.If forced duction of the antagonist was negative,we can considered strengthening it.The clinical manifestations can be improved in 62 eyes After surgery.Conclusion CFEOM is one of rare CCDDs,the astigmatism of refrective errors was among 51 eyes( 85％ ),the incidence of amblyopia and CFEOM1 were high.The strabismic angles can be decreased in most patients through surgery.