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先天性布朗综合征患者的自发缓解

Spontaneous resolution in patients with congenital Brown syndrome.

作者信息

Dawson Emma, Barry John, Lee John

机构信息

Moorfields Eye Hospital, London, United Kingdom.

出版信息

J AAPOS. 2009 Apr;13(2):116-8. doi: 10.1016/j.jaapos.2008.09.007. Epub 2008 Dec 12.

Abstract

INTRODUCTION

Brown syndrome is characterized by a restriction of active and passive elevation in adduction. It can be congenital, acquired, intermittent, or constant. Spontaneous improvement has been reported in all types but is less common in congenital and constant cases.

METHODS

A retrospective review of patients seen between 1992 and 2002 was conducted at our tertiary referral center of all new referrals with a diagnosis of constant congenital Brown syndrome that was previously untreated.

RESULTS

Thirty-two patients were identified (22 female and 10 male patients), with an age range at referral of between 1 and 14 years. The right eye was affected in 14 patients, and 3 patients had bilateral Brown syndrome. Twenty-nine (91%) demonstrated binocular vision. Nineteen patients had an abnormal head posture. Twenty-four (75%) patients had some improvement in their ocular movements without any intervention. The best improvement was from a -3 limitation to full movement. Of 6 patients with a "click," 5 improved. Pain was a problem in 10 patients, 8 of whom improved. Diplopia was a symptom in 13 patients, of whom 11 improved. The final review ranged from 6 months to 9.5 years after the first visit. Five patients who had no spontaneous improvement underwent surgery and obtained improvement.

CONCLUSIONS

Spontaneous improvement occurred in 75% of patients. Our management is to be cautious with treatment and adopt an observational approach with these patients.

摘要

引言

布朗综合征的特征是在内收时主动和被动上抬受限。它可以是先天性、后天性、间歇性或持续性的。据报道,所有类型的布朗综合征都有自发改善的情况,但在先天性和持续性病例中较少见。

方法

在我们的三级转诊中心对1992年至2002年间所有新转诊的、诊断为未经治疗的持续性先天性布朗综合征患者进行回顾性研究。

结果

共确定32例患者(22例女性和10例男性),转诊时年龄在1至14岁之间。14例患者右眼受累,3例患者患有双侧布朗综合征。29例(91%)有双眼视觉。19例患者有异常头位。24例(75%)患者在未进行任何干预的情况下眼动有一定改善。最佳改善情况是从-3的受限程度到完全运动。6例有“咔哒”声的患者中,5例有所改善。10例患者存在疼痛问题,其中8例有所改善。13例患者有复视症状,其中11例有所改善。最后一次复查在首次就诊后6个月至9.5年之间。5例未自发改善的患者接受了手术并获得改善。

结论

75%的患者出现了自发改善。我们的处理方法是谨慎对待治疗,对这些患者采取观察性方法。

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