Duane眼球后退综合征临床和组织病理学研究  

Study on clinical manifestation and histopathology of Duanes retraction syndrome

作　　者：邓大明[1] 李永平[1] 麦光焕[1] 林建贤[1] 杨少梅[1] 

机构地区：[1]中山医科大学中山眼科中心,510060

出　　处：《中国实用眼科杂志》2000年第7期396-398,共3页Chinese Journal of Practical Ophthalmology

摘　　要：研究 Duane眼球后退综合征的临床表现及眼外肌的组织病理改变 ,探讨其发病机理。方法 :研究 41例 Duane眼球后退综合征的临床表现 ,手术病例 (4例 )术中观察眼外肌的形态学并取内、外直肌和上斜肌标本作光镜和电镜检查。结果 :41例患者中 ,男 2 7例 ,女 14例 ,年龄 1～ 2 5岁 ;双眼发病 10例 ,单眼发病 31例 ;所有病例均有典型 Duane眼球后退综合征的表现 ,伴有内斜视 11例 ,外斜视 9例 ,上斜视 1例 ,12例还有内转位时的眼球迅速上转或下转的垂直运动异常。光镜观察显示眼外肌中可见大量的胶原纤维组织 ,未见到正常的横纹肌结构 ,残留的肌纤维变性、坏死 ,并见肥大细胞浸润 ;电镜下见胶原纤维粗细不均 ,部分肿胀、变性 ,残留的肌细胞肌膜和肌质溶解 ,肌浆网扩张呈空泡状。结论 :眼外肌的变性、胶原纤维化和肌细胞变性、坏死、自溶是 Duane眼球后退综合征的主要病理改变 。Objective:To investigate the clinical manifestation,histopathologic changes and pathogenic mechanism of Duanes retraction syndrome(DRS).Methods:The clinical features of 41 in 4 cases with surgical treatment were investigated,and the specimens of medial rectus,lateral rectus and superior oblique muscles were taken light and electrical microscopy.Results:These were 27 males and 14 females in 41 cases,age was from 1 to 25 years old.10 cases appeared monocular,and 31 cases were binocular.All cases had typical clinical manifestation of DRS,and there were 11 cases with esotropia,9 cases with exotropia and 1 case with hypertropia,12 cases with upshoot or downshoot of the eye in adduction.The light microscopy showed there were lots of colloid fibers and few normal striomuscular tissues in the extraocular muscles.The degeneration and necrosis of residual muscular fibers and mast cells were found in the tissues.The electrical microscopy found that the sizes of colloid fibers were unequal and some tissues were turgescent and denatured.The myolemma and myoplasms of residual myocytes were lytic.Sarcoplasmic reticulum dilated like vacuoles.Conclusion:The degeneration,colloid fibrosis of extraocular muscles,the degeneration necrosis and autolysis of myocytes are the prominent histopathologic changes of DRS.Immune mechanism may play a role in the pathogenesis of DRS.

关 键 词：临床表现 病理学 DUANE眼球后退综合征 

分 类 号：R771.102[医药卫生—眼科]