小睑裂综合征的联合手术方法

目的探讨小睑裂综合征的联合手术方法。方法小睑裂综合征l3例26眼,采用三联手术,即上睑提肌缩短术、上睑提肌缩短加横韧带悬吊术、额肌腱膜悬吊术或额肌悬吊术治疗上睑下垂;Spaeth双“Z”成形术和“Y-V”成形术治疗内眦赘皮;并进行外眦开大术以扩大睑裂,3项手术一次完成。结果术后外观有明显改善,睑裂长度和高度显著增加,内眦间距显著缩短。术前睑裂长度19~24mm,平均(21.0±1.4)mm,睑裂高度:1~4mm,平均(2.3±1.1)mm,内眦间距35~40mm,平均(37.0±1.6)mm,术后睑裂长度25~30mm,平均(27.4±1.3)mm。睑裂高度:5~8mm,平均(6.2±0.9)mm。内眦间距30~35mm,平均(32.7±1.3)mm,各相应组两两比较差异均有统计学意义(P=0.000)。结论三联手术治疗小睑裂综合征取得良好效果。     

小睑裂综合征三家系报告

小睑裂综合征是一种先天性遗传病,其表现为双眼上睑下垂、睑裂窄小、倒向内眦赘皮,内眦远距,常合并眼外异常。在我国群体发病率为0.17‰[1]。现将笔者所遇小睑裂综合征3家系21例报告如下。家系Ⅰ先证者女18岁因出生后双睁眼困难而入院,否认眼部红痛、外伤史。右眼裸眼视力0.3,不能矫正;左眼裸眼视力0.5,矫正视力0.8(-1.50DS-2.00DC×130°)。睑裂宽度:双眼均为1mm,睑裂长度:右18mm,左19mm。倒向内眦赘皮。内眦间距45mm。提上睑肌肌力0mm。眼球无震颤。双眼球结膜无充血、角膜透明、前房深度正常,瞳孔直径3mm,对光反射灵敏,晶状体及玻璃体…     

小睑裂综合征16例的临床观察和治疗

目的探讨小睑裂综合征的临床特点，手术效果及遗传学研究进展。方法回顾性分析我院１９９２年１１月至１９９７年３月间收治的小睑裂综合征１６例。结果其中４例同时行内外眦开大和上睑下垂矫正术，１２例先行内外眦开大术，２周或３个月后行上睑下垂矫正术；７例有完整的散瞳验光结果，６例双眼有不同程度的弱视；随访时间３个月～５年４个月。结论小睑裂综合征病人屈光不正和弱视的发生率远高于正常人群，应尽早手术治疗，然而术后功能及美容效果尚不满意     

先天性小睑裂综合征家系分析及手术治疗

0引言先天性小睑裂综合征(congenital blepharophimosis syndrome),又称睑裂狭小-上睑下垂-倒向型内眦赘皮综合征(blepharophimosis-ptosis-epicanthus inversus syndrome,BPES)和小睑裂畸形,是一种少见的常染色体显性遗传病。临床表现为睑裂狭小,上睑下垂,反向内眦赘皮,内眦间距增宽。本文对一组先天性小睑裂综合征病例进行家系分析,全部采用同期手术治疗方法,有效地改善了患者外观,取得满意效果。1临床资料本组患者为2008/2010年在我院眼科病房收治的20例小睑裂综合征,其中男10例,女10例,计40眼。     

先天性小睑裂综合征临床手术疗效分析

目的探讨先天性小睑裂综合征的手术治疗效果。方法回顾性分析先天性小睑裂综合征患儿23例（46眼）,年龄2～11岁,均伴有其他眼疾或全身疾病。所有患儿同期行双眼内眦赘皮矫正＋额肌腱膜悬吊术。内眦赘皮矫正采用的术式有4种,其中Y—V成形术2例、Stallars“Z”成形术7例、Speath术式5例及经典的Mastands内眦成形术9例。上睑下垂矫正采用额肌腱膜悬吊术。术后随访1～24个月,观察睑裂长度、睑裂高度、内眦间距及眉睑距等参数变化。术后1、6、18个月分别检查三棱镜斜视度。采用配对t检验对手术前后患者的睑裂长度、睑裂高度、内眦间距及眉睑距等进行比较。结果23例患儿均顺利完成手术。术后睑裂长度及高度均有明显提高,内眦间距及眉睑距明显缩小。术后1个月,睑裂长度由术前的（18．5±1．1）mm增长至（23．4±1．2）mm,睑裂高度由术前的（2．1±1．2）mm增高至（6．7±0．9）mm,内眦间距由术前的（3512±1．4）mm减小至（31．1±1．1）mm,眉睑距由术前的（13．6±1.3）mm减小至（9．8±1．2）mm,与术前比较,差异均有统计学意义（t分别为4．985、10．832、2．143和4．171,P均〈0．05）。术后1个月,仅见暴露性角膜炎1例,未见其他并发症。术后6、18个月的随访数据与术后1个月比较,差异均无统计学意义（P〉0．05）,表明手术疗效在术后1个月时保持稳定。结论对于先天性小脸裂综合征,早期双跟周期行内眦赘皮矫正联合额肌腱膜悬吊术是比较理想的治疗手段．     

改良Y-V成形及额肌瓣悬吊治疗小睑裂综合征

目的 探讨小睑裂综合征的临床特点及手术效果。方法 回顾性分析我院2002年3月至2006年7月间收治的小睑裂综合征15例。结果术后随访3～36个月,Y-V成形及额肌瓣悬吊Ⅰ期手术治疗的15例患者,术后睑裂长度平均增长3～5mm;睑裂宽度增大3～6mm;双眼内眦间距缩短7～12mm。结论 改良Y-V成形及额肌瓣悬吊Ⅰ期手术治疗小睑裂综合征患者效果肯定。小睑裂综合征患者应尽早手术治疗。     

家族性先天性睑裂狭小综合征

0引言先天性睑裂狭小综合征（congenital blepharophimosis syndrome）是一种先天异常,又称睑裂狭小-上睑下垂-倒向型内眦赘皮综合征（blepharophimosis-ptosis-epicanthus inversus syndrome,BPES）[1],以睑裂狭小为特征,     

分期手术治疗先天性小睑裂综合征

目的观察分期手术(内眦成形术、外眦成形术、异体筋膜悬吊术)治疗先天性小睑裂综合征的临床疗效.方法对31例62眼先天性小睑裂综合征患者进行分期手术治疗.比较术前、术后睑裂长度、宽度、内眦间距与上睑缘的位置.结果睑裂平均长度由术前19.5 mm增至术后28.1 mm,睑裂平均宽度由术前3.4 mm增至术后8.6mm,平均内眦间距由术前35.6 mr减至术后25.3 mm,上睑缘位置由术前位于瞳孔上、下缘间提高至角膜上缘附近.1例发生暴露性角膜炎.结论分期手术治疗先天性小睑裂综合征能使睑裂长度和宽度接近或达到正常,有效地改善了外观.     

先天性睑裂狭小综合征的遗传学研究进展

先天性睑裂狭小综合征，即睑裂狭小-上睑下垂-逆向内眦赘皮综合征（Blepharophimosis-ptosis-epicanthus inversus syndrome，BPES）为少见的常染色体显性遗传疾病，偶有散发病例。临床特点为睑裂水平径和垂直径明显变小，同时伴有上睑下垂和逆向内眦赘皮等。BPES可分为两型，Ⅰ型女性患者因卵巢功能早衰（premature ovarian failure，POF）而不育，但男性生育功能正常。Ⅱ型男女患者因只累及眼睑故均可生育。     

先天性小眼球小睑裂畸形的矫治

目的:探讨先天性小眼球小睑裂畸形的矫治方法。方法:对11例11眼先天性小眼球小睑裂畸形行结膜瓣全角膜覆盖、外眦开大、结膜囊成形联合术并进行回顾分析。结果:在11例患者中,9例于术后4wk顺利安装预选好的义眼;2例角膜表面结膜瓣愈合不良,治疗痊愈后于术后6wk安装义眼。随访2～21mo,4例更换较大型号义眼,全部病例均能轻松安装义眼,无睑裂回缩,角膜覆盖完好,安装义眼后无明显刺激症状,外观较术前明显改善。结论:采用结膜瓣全角膜覆盖,外眦开大联合结膜囊成形术治疗先天性小眼球畸形,具有外观满意,创伤小,并发症及痛苦少,治疗费用低廉等优点,是一种值得推广的方法。     

三联术治疗先天性睑裂狭小综合征

目的探讨先天性睑裂狭小综合征的手术方法。方法13例行内眦成形、外眦成形和上睑下垂矫正三联手术。结果术后全部13例(26眼)睑裂宽度≥6mm，重睑线美观自然，眼睑闭合良好。结论三联术治疗先天性睑裂狭小综合征术式简便，效果确切。     

先天性上睑下垂的手术矫正

目的报告先天性上睑下垂手术矫正的效果,并对其疗效进行评价。方法对234例(308眼)先天性上睑下垂病人,均采用前路提上睑肌缩短术,重度上睑下垂采用提上睑肌超常量缩短术。结果术后平视睑裂高度6～9mm者280眼(90.9%),上睑缘弧度及双重睑形成良好,双侧眼窝深度对称。197眼重度上睑下垂行提上睑肌超常量缩短术后均有不同程度的睑裂闭合不全,术后1～3月恢复眼睑闭合功能,10眼结膜脱垂。结论该术式是治疗先天性上睑下垂比较理想且符合解剖生理的可行术式。目的报告先天性上睑下垂手术矫正的效果,并对其疗效进行评价。方法对234例(308眼)先天性上睑下垂病人,均采用前路提上睑肌缩短术,重度上睑下垂采用提上睑肌超常量缩短术。结果术后平视睑裂高度6～9mm者280眼(90.9%),上睑缘弧度及双重睑形成良好,双侧眼窝深度对称。197眼重度上睑下垂行提上睑肌超常量缩短术后均有不同程度的睑裂闭合不全,术后1～3月恢复眼睑闭合功能,10眼结膜脱垂。结论该术式是治疗先天性上睑下垂比较理想且符合解剖生理的可行术式。     

儿童先天性上睑下垂手术方法探讨

目的观察不同的手术方式矫治儿童先天性上睑下垂的治疗效果。方法对56例（100眼）儿童先天性上睑下垂采用上睑皮肤单切口,分3组：第1组利用额骨膜缝线式吊术,治疗年龄在3月～1．5岁的患儿。第2组利用上睑提肌缩短术治疗上睑提肌肌力为4—8mm的患儿。第3组利用额肌瓣悬吊术治疗上睑提肌肌力为0～6mm的患儿。结果随访6月～2a,额肌瓣组病例全达满意效果,而上睑提肌组12眼,矫正不足,额骨膜缝线悬吊组4眼上睑垂睑线消失。结论额肌瓣悬吊术为矫治儿童先天性上睑下垂的最佳手术方式。     

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

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. Mustardé 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). Mustardé 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. Mustardé 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.     

网织带额肌悬吊术一期矫正儿童小睑裂综合征

目的:探讨网织带额肌悬吊术联合内眦韧带缩短、内眦成型术一期矫治儿童小睑裂综合征的手术效果。方法:对14例小睑裂综合征患儿行网织带额肌悬吊术联合内眦韧带缩短、Y-V内眦成型术,术后随访1～24mo。结果:小睑裂综合征患儿14例术后垂直睑裂5～9(平均6.67)mm;水平睑裂25～30(平均28.92)mm,双眼内眦距离30～35(平均30.57)mm。矫治效果满意,无严重并发症发生。结论:网织带额肌悬吊术联合内眦韧带缩短、内眦成型术一期矫治儿童小睑裂综合征是可行的。     

Blepharophimosis: a recommendation for early surgery in patients with severe ptosis

Purpose: To determine the optimal age for surgical correction of blepharophimosis. Associated features and their effects on incidence of amblyopia were also investigated. Methods: The study was a retrospective case series of 28 patients with blepharophimosis, ptosis and epicanthus inversus syndrome presenting to a tertiary referral eyelid, lacrimal and orbital clinic. Results: Amblyopia was present in 39% of patients. Patients with coexistent strabismus had a 64% incidence of amblyopia compared to 24% for those without strabismus. Hypermetropia was present in 43% of patients and 7% were myopic. Significant astigmatism was found in 40% of patients, but these factors did not increase the risk of amblyopia. Patients with severe ptosis had lower rates of amblyopia than those with moderate ptosis but had their ptosis corrected at a median age of 2 years compared to 5 years for those with moderate ptosis. There was an 18% incidence of nasolacrimal drainage problems. A good to excellent cosmetic outcome was achieved in 86% of patients. A positive family history was noted in 75% of patients, usually with paternal inheritance. Conclusions: Patients with blepharophimosis have a high rate of amblyopia. Co‐existent strabismus doubles the risk of amblyopia. Ptosis alone causes mild to moderate amblyopia only. Patients with severe ptosis should have their ptosis corrected before 3 years of age, and all other patients should undergo surgery before 5 years of age.     

Marcus Gunn张口瞬目综合征手术方式探讨

目的探讨Marcus Gunn张口瞬目综合征不同手术方式和治疗效果。方法Marcus Gunn张口瞬目综合征6例(6眼)采用上睑提肌水化分离及节段切除联合额肌瓣悬吊术。结果6例随访6月～2年,双眼睑裂高度对称,弧度自然,颌动瞬目联合性动作消失。结论采用上睑提肌水化分离及节段切除联合额肌瓣悬吊,是治疗Marcus Gunn张口瞬目综合征的较好术式。