CAJ | 학술논문

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.
목적 탐토선천성Brown종합정적병인、림상특정、수술방식급술후효과.방법 회고성분석2003년4월지2013년6월재천진시안과의원수치적11례선천성Brown종합정환자적림상자료,술후평균수진6.7개월,대기림상특정、수술방식화술후료효진행분석총결.결과 11례환자,년령3.5～29세,평균9.02세;쌍안수루1례,단안수루10례;6례유대상두위.술전원재위안저조상10례무내선,1례경도내선.피동견랍시험,11례환자내상전균유불동정도한제.안림상특정분급,경도3례、중도6례、중도2례.8례행상사기단건술혹부분절제술,2례상사기후사술,1례상사기연장술;합병수평사시자행상규수평사시교정술,수술평균교정수직사시도11.50△.술후안구내상전수한균명현개선,대상두위소실혹개선.결론 선천성Brown종합정환자비교소견,수결합특정성림상표현급상사기피동견랍시험주출정학진단.수술시주요치료방법,수근거술전상세검사결과급술중견랍시험제정합리적개성화수술방안.
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.