先天性Brown综合征11例分析  

Analysis of 11 patients with Congenital Brown Syndrome

作　　者：孙艳群[1] 陈霞[1] 曲晓蕾[1] 付令利[1] 

机构地区：[1]天津医科大学眼科临床学院天津市眼科医院天津市眼科研究所,300020

出　　处：《中国实用眼科杂志》2014年第6期754-757,共4页Chinese Journal of Practical Ophthalmology

摘　　要：目的 探讨先天性Brown综合征的病因、临床特征、手术方式及术后效果.方法 回顾性分析2003年4月至2013年6月在天津市眼科医院收治的11例先天性Brown综合征患者的临床资料,术后平均随诊6.7个月,对其临床特征、手术方式和术后疗效进行分析总结.结果 11例患者,年龄3.5～29岁,平均9.02岁;双眼受累1例,单眼受累10例;6例有代偿头位.术前原在位眼底照相10例无内旋,1例轻度内旋.被动牵拉试验,11例患者内上转均有不同程度限制.按临床特征分级,轻度3例、中度6例、重度2例.8例行上斜肌断腱术或部分切除术,2例上斜肌后徙术,1例上斜肌延长术;合并水平斜视者行常规水平斜视矫正术,手术平均矫正垂直斜视度11.50△.术后眼球内上转受限均明显改善,代偿头位消失或改善.结论 先天性Brown综合征患者比较少见,需结合特征性临床表现及上斜肌被动牵拉试验做出正确诊断.手术是主要治疗方法,需根据术前详细检查结果及术中牵拉试验制定合理的个性化手术方案.Objective To investigate the etiologies,clinical characteristics,surgery methods and the effect of the surgery of Congenital Brown Syndrome.Methods Eleven medical records of Congenital Brown Syndrome patients in Tianjin Eye Hospital from April 2003 to June 2013 were reviewed,with a mean follow-up of 6.7 months,of The clinical characteristics,surgeries and the postoperatively effects were analyzed and summarized.Results Mean age of the 11 patients was 9.02 years （ranged from 3.5～29 years）.Only one had bilateral Congenital Brown Syndrome,10 cases had unilateral,6 cases had abnormal head posture.Fundus torsion before surgery showed no intorsion of 10 cases,and one minimal intorsion.All patients proved restricted in forced duction test.All the patients were divided into 3 grades depending on the clinical characteristics,with 3 in mild,6 in moderate and 2 in severe.A superior oblique muscle （SO） tenotomy or a SO tenectomy was performed in 8 patients,a SO recession in 2 patients and a SO expander in 1 patient.Those who combined with horizontal strabismus also underwent relevant horizontal surgery.Mean vertical deviation which was corrected was 11.50△,with improvement of elevation in adduction and resolution of abnormal head posture after surgery.Conclusions Congenital Brown Syndrome is rare.Right diagnosis according to the special clinical characteristics and forced duction test can be made.Surgery is main therapies.The individual surgery program for each patient depending on the detailed preoperative examination and intraoperative forced ductions should be made.

关 键 词：先天性Brown综合征 牵拉试验 斜视手术 

分 类 号：R777.41[医药卫生—眼科]