先天性眼外肌纤维化综合征家系的眼外肌及眼运动神经影像学追踪研究  

Longitudinal study on imaging features of extraocular muscles and ocular motor nerves in Chinese families with congenital fibrosis of extraocular muscles

作　　者：吴丽[1] 周炼红[1] 刘昌盛[2] 查云飞[2] 叶美红[1] 王炯[1] 

机构地区：[1]武汉大学人民医院眼科,湖北省武汉市430060 [2]武汉大学人民医院放射科,湖北省武汉市430060

出　　处：《眼科新进展》2009年第10期759-763,共5页Recent Advances in Ophthalmology

基　　金：湖北省科技攻关项目基金资助(编号:2006AA301B58)~~

摘　　要：目的研究先天性眼外肌纤维化综合征家系发病者眼外肌及眼运动神经病变的特征及是否呈进行性发展,以探讨其发病机制。方法对收集的2个先天性眼外肌纤维化综合征家系共15例(30眼)成人发病者(男5例,女10例,平均年龄35.6岁)进行高分辨率MRI扫描,随机抽取16名(32眼)正常成人(男女各8例,平均年龄34.5岁)作为对照,比较两组眼外肌及眼运动神经(动眼神经和外展神经)影像学特征。2a后对该15例发病者再次进行高分辨率MRI扫描,并与2a前MRI结果进行比较。结果2a前发病者5条眼外肌体积(外直肌、内直肌、上直肌、下直肌、上斜肌)均较正常对照组小(P均<0.05),尤以上直肌明显[2a前发病者上直肌体积为(103.63±16.32)mm3,而正常对照组则为(316.69±15.61)mm3,差异有显著统计学意义(P=0.001)]。眼运动神经(动眼神经、外展神经)的颅内段截面积分别为(0.51±0.08)mm2、(0.55±0.07)mm2,而正常对照组分别为(1.30±0.21)mm2、(1.30±0.13)mm2,两组相比差异均有显著统计学意义(P=0.000、0.000)。2a后MRI结果显示发病者眼外肌体积和眼运动神经的颅内段截面积[动眼神经及外展神经颅内段截面积分别为(0.49±0.09)mm2、(0.55±0.07)mm2]较2a前无明显改变(P均>0.05)。结论先天性眼外肌纤维化综合征的病因为神经源性,由眼运动神经发育缺陷继发眼外肌萎缩所致,成人以后病变呈非进展性。Objective To investigate the imaging features of extraocular muscles and ocular motor nerves for congenital fibrosis of extraocular muscles（CFEOM） in two Chinese families and the pathological changes in progressive way or not,and to explore the pathogenesis of CFEOM.Methods High resolution magnetic resonance imaging（MRI） scanning was taken in 15 adult patients（30 eyes;5 male patients,10 female patients;averaged in 35.6 years old） from two Chinese families with CFEOM,and 16 normal adults（32 eyes;8 male patients,8 female patients;averaged in 34.5 years old） were randomly collected as control group.The imaging features of extraocular muscles and ocular motor nerves（oculomotor nerves and abducent nerves） were compared between the two groups.The high resolution MRI scanning was taken again at 15 patients 2 years later,and compared with those 2 years ago.Results Two years ago,the volumes of 5 extraocular muscles（lateral rectus muscle,medial rectus muscle,inferior rectus muscle,superior rectus muscle,superior oblique muscle） was significantly smaller than those of control group（all P〈0.05）,especially for superior rectus muscle that the volume was（103.63±16.32） mm3 2 years ago and（316.69±15.61） mm3 in control group（P=0.001）.Cross-sectional areas of intracranial segments of ocular motor nerves（oculomotor nerves and abducent nerves） were（0.51±0.08）mm3 and（0.55±0.07）mm3 2 years ago,and（1.30±0.21）mm3 and（1.30±0.13）mm3 in control group（both P=0.000）.Two years later,the volume of extraocular muscles and the cross-sectional areas of intracranial segments of ocular motor nerves,were not significant compared with those 2 years ago（all P〉0.05）.Cross-sectional areas of intracranial segments was（0.49±0.09）mm3 for oculomotor nerves and（0.55±0.07）mm3 for abducent nerves.Conclusions CFEOM is due to neuropathy,and caused by atrophy of extraocular muscles secondary to hypoplasia of ocular motor nerves.But the pathological changes are non-progressive w

关 键 词：眼外肌纤维化综合征 MRI 眼外肌 眼运动神经 

分 类 号：R777.4[医药卫生—眼科]