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Blepharophimosis-ptosis-epicanthus inversus syndrome: objective analysis of surgical outcome in patients from a single unit.

机译:支气管增生-上睑下垂-picpichuths综合征:客观分析单个患者的手术结局。

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BACKGROUND: The aim was to objectively assess surgical outcome in blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). METHOD: Retrospective review of 14 consecutive patients with BPES. Mustarde double z plasty (at about age 4) and autogenous fascia lata Crawford brow suspension (9-12 months later) were used in all. One consultant performed all operations. Patient photographs were scanned and magnified for analysis. Preoperative and postoperative intercanthal distance, vertical palpebral aperture and cosmetic outcome were measured. Values were converted into ratios. RESULTS: Bilateral (mildly asymmetrical) ptosis and symmetrical epicanthic folds were found in all preoperatively. Seventy-one per cent had severe folds. Preoperative intercanthal distance ratio range was 1.26-1.60 (non-BPES=1.0). Median reduction post surgery was 26% (P=0.001, Wilcoxon's signed ranks test). Patients with largest preoperative ratio displayed greatest correction (P=0.029). Mustarde technique completely abolished all epicanthic folds and unmasked prominent caruncles in all. Fifty per cent had mild scarring postoperatively in the medial canthal region. Median change in marginal reflex distance ratio (right)=22.2% (P=0.012), left=18.2% (P=0.008), Wilcoxon's signed ranks tests. Postoperatively all had formed eyelid creases with improved upper lid position symmetry. CONCLUSIONS: This is the first known study objectively assessing surgical outcome in BPES. Mustarde double z plasty and Crawford autogenous fascia lata brow suspension are an effective surgical combination in the treatment of this syndrome, with overall cosmetic benefit.
机译:摘要背景:目的是客观评估睑缘部上睑下垂-上睑下垂-picpichuths inversus综合征(BPES)的手术效果。方法:回顾性分析连续14例BPES患者。全部使用Mustarde double z成形术(大约4岁)和自体筋膜lat Crawford眉骨悬吊术(9-12个月后)。一名顾问执行了所有操作。扫描患者照片并放大以进行分析。测量术前和术后椎间距离,垂直睑裂和美容效果。将值转换为比率。结果:术前均发现双侧(轻度不对称)上睑下垂和对称上皮褶皱。 71%的人有严重的皱褶。术前can间距离比范围为1.26-1.60(非BPES = 1.0)。手术后中位数减少率为26%(P = 0.001,Wilcoxon的秩和检验)。术前比率最大的患者表现出最大的矫正(P = 0.029)。芥末酱技术完全消除了所有上表皮褶皱,并完全消除了所有掩盖的突出的unc。百分之五十的术后在内侧can区有轻度的疤痕。 Wilcoxon的符号秩检验,边缘反射距离比的中位数变化(右)= 22.2%(P = 0.012),左= 18.2%(P = 0.008)。术后所有人均形成了眼睑折痕,上睑位置对称性得到改善。结论:这是第一个客观评估BPES手术结果的已知研究。芥末双z成形术和克劳福德自体筋膜大腿骨悬吊术是治疗该综合征的有效手术组合,具有整体美容效果。

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