Neonatal cleft lip and cleft palate repair

Facial clefting is one of the most common birth defects and having a child born with this defect can be a devastating experience for parents and family members. Until recently, repair of cleft lip and palate deformities was deferred until the infant had reached 10 weeks of age, 10 pounds in weight, and a hemoglobin level of 10 gm per 100 mL. This criteria eliminated neonates 28 days of age or younger from the possibility of early repair. Waiting until these criteria were achieved often caused problems with parentinfant bonding and infant growth and development. Advances in neonatology and pediatric anesthesia now have made it possible to perform cleft surgery during the neonatal period. This article discusses the anatomy and embryology of cleft deformities and how cleft repair surgery now can be performed on the neonate. AORN J 75 (March 2002) 490-499.     

Tongue-palate contact patterns for Japanese speakers with and without cleft lip and palate

Abstract

Purpose: Visual feedback therapy, using electropalatography (EPG), has been used to remedy residual articulation errors associated with cleft palate. The tongue-palate contact patterns of typical speakers without cleft palate are usually used as a model. However, it is questionable whether these model patterns are adequate for clients with repaired cleft palate, as their dento-palatal morphology is different from non-cleft speakers. The objective of this study was to investigate the differences in tongue-palate contact patterns between typical speakers with and without cleft palate.

Method: EPG data were recorded for alveolar consonants in 15 participants with repaired unilateral cleft lip and palate (UCLP) whose speech was perceptually assessed as typical Japanese. The cumulative templates for each consonant were generated from the maximum contact frame; quantitative analyses (centre of gravity [CoG], variability index) were performed. Fifteen typical Japanese speakers without cleft served as a control group.

Result: EPG patterns for each consonant were generally similar between groups. The CoG value of the UCLP group was significantly lower only for /s/. The average variability index was higher for every consonant but the comparisons did not reach significance.

Conclusion: The typical tongue-palate contact patterns can be used as a model of visual feedback therapy.     

Genetics of cleft lip and palate

Cleft lip with or without cleft palate (CL/CP) is one of the most common structural birth defects, with treatment including multiple surgeries, speech therapy, and dental and orthodontic treatments over the first 18 years of life. As a result of the treatment interventions, pediatric nurses may be required to care for these children beginning in infancy through adolescence. Providing care for these patients and families can include educating patients and parents about the genetics of CL/CP, as well as meeting their immediate medical needs. A basic overview of normal lip and palate development, classification of clefts, pathophysiology of CL/CP, incidence, inheritance, genetic and environmental causes, genetic counseling, prenatal diagnosis, fetal surgery, and nursing implications is provided to educate pediatric nurses about the basics of the genetics of CL/CP.     

Cleft lip and cleft palate.

The birth of a child with a cleft lip or a cleft palate, or both, can be traumatic to the family. Although referral to a multidisciplinary team experienced in craniofacial abnormalities is essential, the family physician can reduce the impact on the family by providing antenatal diagnosis and continued care of the entire family after diagnosis, during initial feeding and bonding difficulties and throughout the many years of surgical and speech therapy.     

中国山西省部分地区人群肌节同源型结构域1基因与非综合征性唇腭裂的关联性

背景:有研究认为肌节同源型结构域1基因突变可能与非综合征性唇腭裂发生有关,然而针对中国人群的相关研究则少有报道.目的:探讨肌节同源型结构域1基因编码区外显子后端和3'端部分非编码区的基因突变与中国山西部分人群非综合征性唇腭裂的关联性.方法:采用随机化方法收集2007-05/2008-01山西医科大学第一医院口腔科收治的130例非综合征性唇腭裂患者口腔裂隙处黏膜和130例正常人口腔黏膜,提取肌节同源型结构域1基因测序进行病理对照研究.结果与结论:非综合征性唇腭裂组中肌节同源型结构域1基因的表达水平低于对照组(P<0.01),说明在非综合征性唇腭裂组中,肌节同源型结构域1基因表达水平呈现出下调趋势;实验对两组中肌节同源型结构域1基因表达产物进行基因测序,Logistic回归分析结果显示(ANAT904)β=-3.74,(GG/GAA1389)β=-22.18,在被引入的变量中,进入方程的危险因素位点是AA/AT904和GG/GAA1389,说明在非综合征性唇腭裂患者中呈下调表达趋势的肌节同源型结构域1基因变异位点可能是AA/AT904和GG/GAA1389.由此推断肌节同源型结构域1基因编码区外显子后端和3'端部分非编码区的基因突变与中国山西部分地区非综合征性唇腭裂的发生具有关联性.     

胎儿唇腭裂的超声诊断及影响因素

唇腭裂为最常见的先天畸形，发病率平均为0．1％，男多于女”。唇腭裂畸形影响患者面容、生后吸吮、吞咽、发音、呼吸等功能，从优生优育的角度来说，给患者及其家庭造成精神和心理上的负担，所以应将胎儿鼻唇部作为超声常规检查内容之一。近年来超声诊断胎儿唇腭裂已有陆续报道。本文着重探讨唇腭裂诊断的影响因素。     

唇腭裂患儿术后并发症发生的影响因素分析

目的分析唇腭裂患儿术后并发症发生的影响因素分析。方法对386例唇腭裂患儿进行回顾性资料分析,其中术后出现创口裂开出血或并发感染共49例,对患儿的年龄、性别、体质量、喂养方式、手术时间、唇腭裂类型、血红蛋白、母亲年龄、母亲受教育程度等相关因素进行单因素Logistic回归分析,选择有统计学意义的因素进行多因素Logistic回归分析。结果单因素回归分析提示,患儿的年龄、性别、体质量、血红蛋白对唇腭裂患儿术后并发症无显著性影响(P>0.05),而喂养方式、手术时间、唇腭裂类型、母亲年龄、母亲受教育程度会影响唇腭裂患儿术后并发症的发生(P<0.05)。多因素Logistic回归分析发现,喂养方式(OR=5.322,95%CI为1.356~21.321)、手术时间(OR=1.167,95%CI为1.029~1.531)、母亲受教育程度(OR=1.568,95%CI为1.353~2.589)均能影响唇腭裂患儿术后并发症的发生。结论采用汤匙或注射器喂养方式、手术时间短、母亲受教育程度高能减少唇腭裂患儿术后并发症的发生。     

二维超声诊断胎儿唇裂的探讨

目的探讨二维超声产前诊断胎儿唇裂的价值。方法应用二维超声对2457例中、晚期孕妇的胎儿行颜面部检查。结果产前诊断胎儿唇裂5例,均经分娩证实,唇裂检出率100％。结论二维超声是筛选及诊断胎儿唇裂的重要手段。     

改良喂养方式对唇裂腭裂新生儿喂养的效果

目的探讨改良喂养方式对唇裂腭裂新生儿的喂养效果。方法将40例唇裂腭裂新生儿随机分为对照组和实验组各20例,对照组使用普通汤匙常规喂养,实验组采用改良喂养方式喂养。结果实验组新生儿发生呛咳与呕奶、窒息和吸入性肺炎三项并发症的概率明显低于对照组,差异具有统计学意义（P〈0．05）,其家长对于护理工作的认可情况也明显好于对照组,差异具有统计学意义（P〈0．05）。结论改良喂养方式能减少唇裂腭裂新生儿喂养并发症的发生,提高家长满意度,随访结果好,值得临床推广。     

Implementation of standardized medical photography for cleft lip and palate audit

Much has been published relating to the treatment and surgical outcome of cleft lip and palate disorders. Clinical audit is one of the most important tools for assessing the quality of care provided, with medical photography an invaluable component of this process. The Clinical Standards Advisory Group Report 1998 recommended that cleft lip and palate patients should be audited when 0 (under 1 year of age), and then at the ages of 5, 10, 15 and 20 years. For both audit and research purposes, medical photographs need to be accurate and of a consistently high standard. This paper describes the development of a standardized photographic protocol for cleft patients of audit age, to the benefit of both the multidisciplinary team and the patients.     

延续护理在唇腭裂患者中的应用

目的探讨对唇腭裂患者实施延续护理服务的方法与效果。方法成立延续护理小组,建立随访制度;制订个性化健康教育及出院后的自我护理计划;采取电话随访和基于网络平台的健康教育方式开展院外延续护理。结果患者门诊复查率及序列治疗完成率提高(P0.01),患者及医生对护理工作的满意度提高(P0.01或P0.05)。结论开展延续护理是唇腭裂序列治疗的重要组成部分,保证了治疗的连续性和针对性,有利于患者康复。     

先天性小儿唇腭裂的围手术期护理

目的探讨小儿先天性唇腭裂有效的围手术期护理措施。方法对我院2010年1月至2011年12月收治的43例先天性唇腭裂患儿给予围手术期护理。结果患儿伤12I愈合时间为6,7d,平均6．6d,术后对患儿家长进行护理满意度调查发现,患儿家长总满意度为100％。结论护理干预对先天性小儿唇腭裂手术的疗效具有重要意义,围手术期综合护理措施能够取得较好的临床效果,提高患儿家长的满意度。     

单侧完全性唇腭裂患者下颌骨对称性分析

目的 探讨单侧完全性唇腭裂患者下颌骨两侧垂直向对称性。方法 52名单侧完全性唇腭裂患者作为观察组,52名同龄正常牙合人群作为对照组,两组病例拍摄全景片,对下颌关节高度（CH）,升支高度（RH）及升支关节高度总和（CH+RH）进行测量和分析,所得数据行配对样本T检验和独立样本T检验。结果 单侧完全性唇腭裂患者下颌骨健患侧垂直高度中CH,RH,CH+RH均存在统计学差异(P<0.05),对照组左右侧各项高度测量指标均无统计学差异。组间分析关节高度（CH）及关节升支高度（CH+RH）存在统计学差异(P<0.05)。结论 单侧完全性唇腭裂患者下颌骨健患侧垂直向不对称,且其与正常牙合人群比较存在差异。正常牙合人群下颌骨垂直向左右侧对称。     

Exploring parental reactions to the diagnosis of cleft lip and palate

The birth of an infant with a facial cleft evokes a range of emotions and reactions in parents. During acceptance of a child's disability, parents are said to progress through several stages similar to those responses reported in the bereavement process. This paper describes the process of adapting in terms of personal construct theory, and reports a pilot of the repertory grid technique as a therapeutic tool to elicit parental reactions following diagnosis of cleft lip and palate. Two mothers compared other significant life events to their reactions to the diagnosis of cleft lip and palate. This pilot supported the use of the repertory grid technique as a means to facilitate exploration of parental responses. Comparisons to the bereavement process were evident, although individual reactions or perceptions may differ from those expected. Recommendations are made for a wider research study within cleft lip and palate.     

唇腭裂的超声筛查及诊断价值

目的：研究常规鼻唇冠状切面加特殊切面在胎儿唇腭裂畸形筛查中的应用价值.材料与方法：对11688例,孕24 ～ 30周胎儿唇腭部采用常规鼻唇冠状切面加特殊切面（经唇部冠状面、经双眼标准横断面、经鼻腔标准横断面、经上牙槽突标准横断面、经舌标准横断面、经下牙槽突标准横断面和经鼻正中矢状面）行产前超声筛查,并与出生儿颜面部检查结果进行对照分析,总结鼻唇冠状切面加特殊切面超声扫查法的诊断符合率.结果：11688例胎儿共查出59例阳性患者,阳性率0.50％（59/11688）,单纯唇裂13例,占阳性病例的22.03％ （13/59）,唇裂合并腭裂的46例,占阳性病例的77.97％（46/59）,左侧唇腭裂38例,占64.4％ （38/59）,右侧唇腭裂10例,占16.9％（10/59）,正中唇裂2例占3.39％（2/59）,双侧唇腭裂9例,占12.25％（9/59）.结论：超声诊断胎儿唇腭裂畸形通过观察鼻唇部冠状切面加特殊切面（上牙槽突标准横断面、下牙槽突标准横断面、鼻腔横断面、双眼标准横断面、舌标准横断面、胎儿头颅矢状面）及配合一定手法,可明显提高其诊断率.