先天性唇、腭裂对患儿呼吸系统顺应性的影响

目的 探讨先天性辰、腭裂对患儿呼吸系统顺应性（ＣＴ）的影响。方法 唇裂或腭裂病儿２４０例（观察组）,非唇腭裂病儿６０例（对照组）均为择期手术患,按年龄段：１～１２月、１～３岁、４～７岁、８～１２岁,各分为５个亚组。全麻诱导气管内插管后,行机械通气,保持ＰＥＴＣＯ２４～４．６ｋＰａ,用Ｄａｔｅｘ Ｕｌｔｉｍａ监测仪测定ＣＴ。结果 与非唇裂同一年龄组ＣＴ值相比,唇裂各年龄组、腭裂１～１２月及１～３岁组     

Cleft foot and ectrodactyly–ectodermic dysplasia–cleft lip/palate syndrome: Review of the literature and report of two new cases

Ectrodactyly–ectodermic dysplasia–cleft lip/palate syndrome is a rare congenital anomaly of inherited origin and varying clinical features. It includes clefting in hands and feet, cleft lip and palate, and defects in ectodermic derived tissues (skin, hair, nails). Defects in other locations are less frequent.Management of this type of patient is difficult, and there have been few reports in the medical literature about their treatment, whether from the orthopaedic or the surgical point of view. The purpose of this communication is to review current knowledge of this syndrome, and to describe two new cases treated conservatively in our clinic.     

Craniofacial cephalometric morphology in 6-year-old children with isolated cleft lip,isolated submucous cleft palate,and combined cleft lip and submucous cleft palate

One hundred and twenty-one cleft children (67 with isolated cleft lip (CL), 32 with isolated submucous cleft palate (SMCP), and 22 with combined cleft lip and submucous cleft palate (CL + SMCP)) were compared retrospectively from lateral cephalograms taken at a mean age of 6.2 years (range 5.5–7.9). None of the children had had their palates repaired or been operated on to treat velopharyngeal insufficiency (VPI). The children with CL + SMCP and CL had similar morphology. They had greater maxillary length, greater maxillary and mandibular prominence, less vertical growth pattern, less deep nasopharyngeal airways, and thinner upper lips than those with isolated SMCP. After 6 years of age one patient with CL + SMCP, none with CL, and 16 with SMCP needed operations for VPI. This small series suggests that children with CL + SMCP and SMCP have different morphology. Although CL + SMCP is a combination of two types of clefts, it seems to be associated with similar morphology to CL.     

Two cases of cleft lip and palate with craniosynostosis (oxycephaly and scaphocephaly)

Summary Although syndromic cleft lip and palate is occasionally associated with other anomalies, even with craniosynostosis, non-syndromic cleft lip and palate is rarely seen with craniosynostosis. The morphogenesis of these two anomalies seems different, and combined cases are interesting to report. Two rare cases of the association of non-syndromic cleft lip and palate and craniosynostosis (one oxycephaly and the other scaphocephaly) which were operated on with a follow-up of 10 years and 2 years are described.     

婴儿唇腭裂同期修复468例临床分析

目的探讨唇腭裂在婴儿期同期修复的可行性及优缺点。方法回顾性分析我科近十年来同期修复婴儿期唇腭裂468例,对术后3年以上患者进行语音评价。结果468例婴儿均完成唇腭裂同期修复,术后出现并发症173例,死亡1例。其中94例出现不同程度呼吸困难,74例出现腭部缝合口裂开(63例为悬雍垂裂开),2例唇部切口感染,1例因术中缺氧出现脑瘫,2例出现恶性高热(其中1例死亡),1例拔出碘仿纱条后大出血。术后3年以上有278例,随访168例,发音优115例,良48例,差5例。结论婴儿期同时修复唇腭裂虽具有术后语音效果好及其他优点,但呼吸困难及悬雍垂裂开发生率高,良好的麻醉对保证手术成功至关重要。     

Unilateral cleft lip and palate: Simultaneous early repair of the nose, anterior palate and lip

Unilateral cleft lip and palate is a defect involving the lip, nose and maxilla. These structures are inter-related, and simultaneous early correction of all the aspects of the defect is necessary to obtain a satisfactory result that will be maintained with growth. The surgical technique combining various procedures is presented and compared with previously published reports.     

双侧唇裂或唇腭裂修复后口哨畸形的分级与修复

目的 对双侧唇裂或唇腭裂修复后口哨畸形的严重程度进行分级。方法 将我院136例双侧唇裂或双侧唇腭裂术后上唇口哨畸形患者作为研究对象，观察上颌前门齿牙冠及相应牙龈暴露的程度将口哨畸形分为Ⅳ级。Ⅰ级： 牙冠显露1／2；Ⅱ级： 牙冠全显露或 牙冠全显露伴 显露近中纵行牙冠1／2—2／3；Ⅲ级： 牙冠全显露和附丽龈显露1／2或 牙冠全显露伴附丽龈显露1／2及 牙冠显露2／3以上；Ⅳ级： 牙冠及相应牙龈全显露伴 近中牙冠纵显露2／3以上。口哨畸形严重程度与双侧唇裂或唇腭裂修复术式的关系一并进行分析。结果 口哨畸形Ⅰ级者60例，占44．2％；Ⅱ级者47例，占34．5％；Ⅲ级者16例，占11．8％；Ⅳ级者13例，占9．5％。结果 显示Ⅰ级和Ⅱ级口哨畸形明显多于Ⅲ级与Ⅳ级，前者为后者的3．7倍。结论 对双侧唇裂或唇腭裂修复后口哨畸形的分级具有良好的临床参考价值，为修复上唇口哨畸形选择修复方法提供了客观依据。     

Unrepaired Clefts in the Hard Palate: Speech Deficits At the Ages of 5 and 7 Years and Their Relationship to Size of the Cleft

The purpose of the study was to investigate the relationship between the size of oronasal openings in the hard palate and speech deficits. Audiotape recordings and plaster casts were taken according to standard procedures at 5 and 7 years of age from 22 consecutive children born with complete unilateral cleft lip and palate treated at Sahlgrenska University Hospital, Göteborg, Sweden. The soft palate had been repaired before the age of 12 months, whereas the cleft in the hard palate was left unrepaired, to be closed later. Perceptual judgements of nine speech variables at 5 and 7 years of age were correlated with measures of the area of the residual cleft in the hard palate. "Retracted oral articulation" (to velar place) found in nine of the 22 children correlated significantly with the area of the cleft at the age of 5 years but not later. The establishment of this particular speech error seems to be related to the size of an oronasal opening.     

大鼠多指(趾)、并指(趾)、分裂手(足)畸形模型的建立

目的 建立稳定的肢体畸形的大鼠模型.方法 采用抗肿瘤药物白消安,在wistar孕鼠孕第12天(GD12)按25 mg/kg给药,观察胎鼠的畸形类型及畸形率.结果 胎鼠的畸形类型以多指(趾)、并指(趾)、分裂手(足)为主,其中多指(趾)最常见.另外,还可出现缺指(趾)、无指(趾)、掌(跖)骨缺失及骨化不全、胫(腓)骨缺失及骨化不全.结论 成功建立畸形类型稳定的wistar大鼠多指(趾)、并指(趾)、分裂手(足)畸形动物模型,有利于进一步分析研究肢体畸形的发生机制.     

单侧完全性唇腭裂鼻畸形的早期修复

目的　修复单侧完全性唇腭裂的同期 ,修整明显的鼻翼畸形。方法　以同心圆内外弧不等长的几何学原理 ,分析单侧完全性唇腭裂修复后出现典型的患侧鼻翼“S”畸形现象的形成机理 ,设计鼻前庭皮肤“T”形切口 ,三角形切除 ,“T”形缝合方法 ,以有效地修整鼻翼畸形。结果　 1997年以来 ,13例采用此法修复后 ,鼻翼“S”畸形消除 ,鼻孔外形明显改善。结论　单侧完全性唇腭裂修复唇裂后 ,鼻翼前庭侧皮肤过剩 ,切除多余皮肤 ,运用几何原理设计修复手术 ,便可实现鼻翼畸形的改善。     

唇腭裂患者上颌骨发育的研究进展

唇腭裂患者常伴有不同程度的上颌后缩,自身发育不足、功能性及医源性的因素均能引起上颌骨发育受限。我们对唇腭裂患者的颅底形态、上颌骨形态、上颌骨与颅底的关系,以及引起上颌后缩的原因进行综述。     

Physical characteristics of young men with cleft lip,with or without cleft palate,and cleft palate only

We report the weight, stature, body mass index (BMI), and muscular strength of men about 19 years old who have cleft lip, either with or without cleft palate (CLP), or cleft palate only (CP). Data were obtained from the Swedish National Service enrolment register for the years 1991–97, and concerned 335 men with CLP and 88 with CP, who were compared with a control group of 272 879 men. The data showed that those with CLP and CP were significantly lighter than controls. Their stature in the CLP group was similar to that in controls, but those with CP significantly shorter. These findings imply that men with CLP had a significantly lower BMI whereas men with CP had a similar BMI compared with controls. Those with CLP did not differ as regards to muscular strength, but those with CP were significantly weaker than controls.     

腭裂术后腭裂隙内自发骨桥形成及其影响因素

目的 进一步观察腭裂修复术后腭裂隙骨性愈合状况，统计并讨论影响骨性愈合的因素。方法 对52例完全性腭裂术后患者进行头颅冠状CT扫描，观察腭裂骨性裂隙的愈合情况并测量腭裂隙内骨桥形成的位置、形态及质量，对测量结果进行统计，分析其影响因素。结果 52例受试者中的37例(7l％)在腭裂隙处存在有不同程度的术后腭骨板骨桥形成。经统计发现，在形成骨桥的患者中男女比例接近；单侧与双侧腭裂者骨桥形成情况差异无显著性意义；在不同手术年龄的患者中，4～7岁接受腭裂修复术者骨桥形成最为明显；所形成的腭裂隙内骨桥相对集中于双尖牙区及磨牙区前份。结论 腭裂术后骨性腭裂隙内明显有新生骨桥形成，影响其形成的因素与手术年龄有关，与性别及腭裂的类型无明显关系。     

干细胞在唇腭裂患者牙槽突裂中应用的研究进展

干细胞具有自我更新能力及多向分化潜能,已被广泛应用于多个领域。相对于唇腭裂患者牙槽突裂的常规手术治疗方法,干细胞具有来源广泛、取材方便、免疫原性低等优点,整形外科医生已逐渐关注唇腭裂患者牙槽突裂干细胞治疗的研究。该文对胚胎干细胞、人间充质干细胞、颌面部来源干细胞在唇腭裂患者牙槽突裂中应用的研究进展进行了综述。     

单纯腭裂患儿心脏畸形的发生率与腭裂程度的关系

目的 研究单纯腭裂患儿合并先天性心脏病的特点,探讨腭裂的程度与心脏畸形发生率的关系.方法 收集2008年8月至2009年12月收治的416例单纯腭裂患儿的临床资料,对所有患儿进行心脏超声检查;将患儿分为完全性腭裂和不完全性腭裂组,每组再分为单侧和双侧亚组,不完全腭裂组进一步分为悬雍垂裂、软腭裂、软腭及部分硬腭裂3个亚组,统计各组患儿合并心脏畸形的例数和类型,计数资料用百分构成比表示,采用SPSS 13.0版软件包进行统计学处理,组间比较采用x2检验.结果 416例中共检出46例先天性心脏病,占11.1%;不完全性腭裂组心脏畸形检出率为9.9%(38/384),完全性腭裂组检出率为25%(8/32),明显高于不完全性腭裂组(F=6.852,P<0.05);在合并的心脏畸形中,房间隔缺损占52.2%(24/46),是最常见的心脏畸形.结论 与不完全性腭裂相比,完全性腭裂具有较高的发生心脏畸形的风险,应常规进行心脏超声检查,腭裂程度可作为腭裂合并先天性心脏病的预测因子之一.

Abstract：

Objective To analyze the relationship between cleft severity and incidence of associated heart defect in children with isolated cleft palate(CP), as well as the characteristics of the heart defect. Methods From Aug 2008 and Dec 2009, a total 416 children with CP underwent echocardiogram, and were divided into complete and incomplete CP groups. Then each group was further classified as unilateral or bilateral groups. Incomplete CP was subdivided into submucous cleft palate, soft palate cleft, hard and soft palate cleft. The associated heart defects were recorded and analyzed in each group. The data were analyzed statistically using SPSS 13.0. Chi-square test was used to compare the incidence between groups. Results In the series of 416 patients, 46 (11.1%) children were found to have an associated congenital heart disease. The incidence of heart defect was 9.9% (38/384) in the incomplete cleft group, and 25% (8/32) in the complete cleft group, showing a significant difference between the two groups (F = 6. 852, P < 0.05). Atrial septal defect was the most common heart defect,which accounted for 52.2% (24/46) of all associated heart malformations. Conclusions Compared to incomplete cleft palate, complete cleft palate has a higher risk of heart defect. Cleft severity may be a predictor for congential heart diseases in cleft palate. Routine echocardiogram should be considered in CP patients.     

唇腭裂的牙槽突裂植骨修复术

牙槽突植骨术是唇腭裂序列治疗的一个组成部分，对于恢复上颌牙弓的完整性，保证上颌牙齿的正常萌出，促进上颌骨的垂直向生长及颌骨的稳定性，矫正患侧鼻底塌陷畸形及修复唇侧口鼻腔瘘均有重大意义。总结了保证手术成功的关键，并认为植骨术前后应接受正畸治疗。８～１１岁Ｘ线片示尖牙根形成２／３为最佳手术时间。     

唇腭裂的牙槽突裂植骨修复术

牙槽突植骨术是唇腭裂序列治疗的一个组成部分,对于恢复上颌牙弓的完整性,保证上颌牙齿的正常萌出,促进上颌骨的垂直向生长及颌骨的稳定性,矫正患侧鼻底塌陷畸形及修复唇侧口鼻腔瘘均有重大意义。总结了保证手术成功的关键,并认为植骨术前后应接受正畸治疗。8～11岁 X 线片示尖牙根形成2/3为最佳手术时间。