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.     

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.     

Furlow反向双Z法腭裂修复术后腭瘘发生率的研究

目的：研究腭裂Furlow术后瘘的发生率以及影响因素。方法：对53例腭裂修复术后瘘的发生率和可能影响瘘发生的因素如性别、年龄、腭裂类型、裂隙的程度进行回顾性研究。结果：总瘘孔率为18.86%（10/53）,瘘孔率最高为硬腭前部（64%）,其次为软硬腭交界处及硬腭部（分别为18%）,瘘孔率最低为软腭区域（0%）（P〈0.05）。男性与女性患者瘘的发生率无统计学上差异（P〉0.05）。单侧完全性腭裂组（UCCLP,24%）和双侧完全性腭裂组（BCCLP,36.7%）瘘发生率高于硬软腭裂组（HSCP,0%）和软腭裂组（SCP,0%）,但无统计学上差异（P〉0.05）,完全性腭裂组瘘的发生率高于不完全性腭裂组（P〈0.05）。结论：腭裂术后腭瘘的发生与腭裂的类型有关,Furlow术式修复腭裂可减少软腭、软硬腭交界处的腭瘘发生率。但相对的有增加完全性腭裂硬腭前部腭瘘发生率的可能。     

兰氏和双反Z法腭裂修复术后患儿语音清晰度对比

目的比较兰氏和双反Z法腭裂修复术后患儿的语音清晰度,寻找重建腭咽闭合的最佳手术方法。方法选取2009年至2013年在我院口腔颌面外科就诊的先天性软腭裂患儿69例,其中行双反Z法腭裂修复术35例(双反Z法组),行兰氏法腭裂修复术34例(兰氏法组)。患儿3.5岁后随访,由3名语音师进行单盲性审听,比较两组患儿术后语音清晰度。结果语音测评结果显示,双反Z法组患儿术后患者语音清晰度平均达到88.72%±6.05%,明显高于兰氏法组的71.31%±3.46%,语音改善明显。结论双反Z法能够充分缩小咽腔、延长软腭,重建软腭肌肉结构,更有利于恢复良好的腭咽闭合功能。     

唇裂和腭裂的麻醉

唇裂的形成是因胚胎发育时中胚叶从神经嵴到头部和面部的增厚支膜复盖不全,移行迟缓或缺如所致。腭裂是因胎儿发育期间裂缝内舌异位,导致腭难以融合。胚胎性腭分为初腭和继发腭二阶段。初腭发育于妊娠4～7周,由门齿骨(四个前齿及其牙槽)、鼻中膈和鼻小柱组成。继发腭发育于妊娠7～12周,包括硬腭和软腭。唇、腭裂按程度可分为:1.不全性腭裂;2.完全性腭裂;3.不全性腭裂及唇裂;4.单侧完全性唇腭裂;5.双侧完全性唇腭     

腭裂术后复裂原因分析及护理

腭裂是口腔颌面部常见的先天畸形,主要治疗方法为手术修补,而防止术后复裂是修补术成功的关键。我院1986～1990年共收治腭裂病人81侧,有15例发生术后复裂,复裂率为18.64%,现将复裂原因分析如下: 一、一般资料15例术后复裂者中,男12例,女3例;年龄10岁以下5例,11～15岁9例,16岁以上1例;软硬腭交界处穿孔性复裂7例,软腭复裂3例,悬雍垂复裂4例,完全性复裂1例;术后伤口感染5例,恶心、呕吐2例,感冒、咳嗽2例,饮食不当3例,呼吸困难、吸痰不当、大声喊叫哭闹3例。二、腭裂术后复型原因分析资料表明,腭裂术后复裂除与患儿年龄、腭裂     

Sommerlad-Furlow法在腭裂整复术中的应用

目的:采用Sommerlad-Furlow法对腭裂患者进行腭裂唇整复术,并对其手术方式及疗效进行了探讨。方法:腭裂患者67例,其中单侧完全性腭裂29例(左侧13例,右侧16例),不完全性腭裂38例;67例患者手术年龄为1~9岁,平均2.5岁。手术方法是先采用鼻咽侧壁松弛切口以此有效地缩小咽侧壁间的腭咽距离,再以Sommerlad法进行腭帆提肌的解剖复位和重建术,最后运用Furlow法即反向双Z成形术进行有效的进行软腭延长即Sommerlad-Furlow法。结果:本文中所有手术病例均无局部出血、感染、出血、复裂、穿孔。有4例局部创口糜烂;术后随访时间1~3个月,所有患者均无复裂及穿孔。结论:采用Sommerlad-Furlow法进行腭裂整复术可以在术中有效地进行腭帆提肌的解剖复位与重建,从而保证了各种腭裂鼻腔裂隙的完整封闭,极大地减少了腭裂术后复裂及穿孔的发生率并且最大限度地减少传统的硬腭松弛切口应用的概率。由于术中有效地延长软腭,从而为提高临床上腭裂整复术的腭咽闭合率以及今后患者的语音训练提高了很好的基础。至于该方法对腭裂患者远期临床疗效以及术后语音评估的影响需进一步观察研究。     

腭裂硬腭植骨

腭裂患儿由于先天性腭部畸形,口鼻腔相通导致语音含糊不清。腭裂修复术的目的是关闭腭部裂隙,恢复解剖完整性,延长软腭,为接近正常的腭咽闭合创造条件。多年来为了达到腭咽闭合,许多学者在手术方式上不断改进和创新,也包括了对腭裂骨缺损区植骨的研究。自1901年Ｅｉｓｅｌｓｂｅｒｇ[1]首次提出牙槽突植骨以来,由齿槽嵴裂引起的鼻唇部及牙列畸形已逐渐引起重视。腭裂齿槽嵴裂植骨术已成为综合序列治疗的一部分,在我国已逐步开展。1997年在上海召开的会议上报告了300例齿槽嵴裂植骨术的经验总结[2],代表性地说明了植骨的重要意义及推广价值,…     

单侧唇裂鼻畸形的临床研究进展

唇裂是口腔颌面部常见的先天性畸形,其发病率约为新生儿的0.18%,唇裂Ⅰ期修复手术多在患儿出生后的3个月进行[1].唇裂可分为单侧(完全性或不完全性)唇裂、双侧(完全性、不完全性、混合性)唇裂、正中裂(极少见)和隐裂(常为单侧或双侧唇裂中的某一侧)[2].     

早期腭裂修复术后瘘的发生率研究

目的 探讨婴儿期腭裂手术后瘘发生的规律。方法 对1995年7月～2001年9月期间施行的2～12个月龄的531例腭裂修复术后瘘发生率进行回顾性分析。结果 全组腭裂术后瘘发生率为4.9%，双侧完全性腭裂术后瘘发生率（7.8%），明显高于不完全性腭裂（4.4%），单侧完全性腭裂（4.3%,P＜0.01），后两组之间无显著性差别（P＞0.05）；术后瘘发生率与年龄，及本组所采用的不同术式无关（P＞0.05）；其中硬腭部瘘占完全性腭裂术后瘘率70%，较其它部位差异有非常显著性意义（P＜0.01）。结论 婴儿期腭裂手术并不会增加瘘发生率，对于双侧完全性腭裂，推荐做术前正畸治疗。     

Congenital heart disease and associated malformations in children with cleft lip and palate in Pakistan.

Children with cleft lip and palate often have other associated malformations. The reported incidence and types of associated malformations vary between different studies. There is a great paucity of literature on the subject from the region in general and none from Pakistan at all. The purpose of this study was to assess the frequency of associated malformations, particularly congenital heart disease, in children with cleft lip and palate presenting to the Aga Khan University (AKU) and Murshid Hospital (MH). From 1st October 1999 to 31st March 2002, all children with cleft lip and palate who presented to AKU and MH were prospectively enrolled in the study group. Socio-demographic characteristics and a number of other variables were documented. All children underwent a thorough clinical examination and an echocardiogram as part of the study protocol. 123 children formed the study group. Thirty-five (29%) of these children were found to have associated malformations. The most common of these was congenital heart disease, which accounted for 51% of all associated malformations. Thirty percent of cleft palate children had associated anomalies while 27% of cleft lip, with or without cleft palate, children had associated anomalies. There was a significant association between children born of a consanguineous marriage and the risk of associated malformations (p-value: 0.001). Consanguinity was present in 74% of children with associated anomalies as compared to 40% of children with no associated anomaly. Dysmorphic features and the presence of associated anomalies were also significantly associated (p-value: 0.009). Dysmorphic features were present in 46% of children with anomalies as compared to 21% of children with no associated anomaly. Fifty percent of children with associated anomalies had a low birth weight compared to 34% of children with no anomalies, but the difference was not statistically significant.The presence of consanguinity in a child with dysmorphic features should raise the suspicion of an associated anomaly. The likelihood of this being a cardiac defect is high and should be ruled out with a thorough clinical examination, supplemented with an echocardiogram in certain cases.     

Palatal surface area measurement: comparisons among different cleft types

The purpose of this study was to use three-dimensional imaging methods to measure the palatal surface of unrepaired cleft patients. The surface area of the palate was defined and measured on three-dimensional computed tomography images of dental plaster models in four different groups of cleft patients at 3 months of age. There were 30 unilateral complete cleft lips and palates (UCLP), 27 bilateral complete cleft lips and palates (BCLP), 23 isolated cleft palates of incomplete form (CP), and 19 unilateral cleft lips without cleft palates (UCL). These patients were nonsyndromic, unoperated, and without other major deformities. The dental casts were scanned, and the computed tomography data were transferred to an imaging laboratory for processing and reconstruction of three-dimensional images. Surface area of the palate was delineated, which was defined as within the alveolar crest and the line connecting both tuberosities. In UCLP and BCLP, the edge of cleft formed the medial boundary of the area for each palatal shelf, and the palatal surface area was the combination of both palatal shelves and the premaxillary area in BCLP group. The surface area was measured. Repeated definition and measurement tasks were performed for calculation of errors. The imaging data management and measurement were performed using the Analyze program (Biomedical Imaging Resource, Mayo Foundation, MN). In addition, linear distances were measured between the canine points on the alveolar crest (line C) and the tuberosity points (line T). The measurements were compared among the different groups. Analysis of variance and multiple comparisons were used for statistical analyses. The results showed that the mean error between repeated area definitions and measurements in this study was 1.86%. The bilateral complete cleft lip and palate (BCLP) and unilateral complete cleft lip and palate (UCLP) groups had significantly smaller palatal surface area than the unilateral cleft lip without cleft palate (UCL) and isolated cleft palate of incomplete form (CP) groups. There was no significant difference between the BCLP and UCLP groups. Line C and line T distances were significantly longer in BCLP and UCLP groups than in UCL and CP groups. The findings suggest that compared with UCL and CP patients, there is an intrinsic tissue deficiency in the palate/maxilla of BCLP and UCLP patients.     

Otitis media and feeding with breast milk of children with cleft palate.

The purpose of the present study was to analyse the incidence of acute and secretory otitis media (OM), and feeding with breast milk, and the use of a grommet in children with a cleft palate (CP/CLP) or cleft lip (CL), compared with controls. A total of 84 children between 6 and 10 years of age were studied. The CP/CLP group consisted of 48 children with an isolated cleft palate (n = 28), or a cleft lip and palate (n = 20). The CL group consisted of 15 children with an isolated cleft lip. The controls were 21 children without clefts. Children with CP/CLP had acute OM significantly more often than children without clefts (43/48 compared with 10/21), and secretory OM (40/48 compared with 4/21), despite the use of grommets. CP/CLP children were breast fed for a mean of 2.8 months (range 0-13), compared with 3.6 months (0-12) for CL, and 7.5 (0-24) months for controls. There was a significant correlation during the first 18 months of life between longer duration of feeding with breast milk and a lower incidence of acute and secretory OM in the three study groups combined. The incidence of otitis media was not affected by care in a day centre, having a sibling attending a day care centre, or by the family's medical history. Despite cleft repair and early treatment with grommets, both secretory and acute OM are common among children with cleft palate, presumably as a result of their eustachian tube dysfunction. The present study suggests that premature cessation of feeding with breast milk may contribute to an increased incidence of acute and secretory OM.     

Isolated cleft palate requires different surgical protocols depending on cleft type

A staged protocol for isolated cleft palate (CPO), comprising the early repair of the soft palate at 6 months and delayed repair of the eventual cleft in the hard palate until 4 years, designed to improve maxillary growth, was introduced. CPO is frequently associated with additional congenital conditions. The study evaluates this surgical protocol for clefts in the soft palate (CPS) and for clefts in the hard and soft palate (CPH), with or without additional malformation, regarding primary and secondary surgical interventions needed for cleft closure and for correction of velopharyngeal insufficiency until 10 years of age. Of 94 consecutive children with CPO, divided into four groups with (+) or without (?) additional malformations (CPS?+?or CPS???and CPH?+?or CPH?), hard palate repair was required in 53%, performed with small local flaps in 21% and with bilateral mucoperiosteal flaps in 32%. The total incidence of soft palate re-repair was 2% and the fistula repair of the hard palate was 5%. The total incidence of secondary velopharyngeal surgery was 17% until 10 years, varying from 0% for CPS???and 15% for CPH?, to 28% for CPS?+?and 30% for CPH+. The described staged protocol for repair of CPO is found to be safe in terms of perioperative surgical results, with comparatively low need for secondary interventions. Furthermore, the study indicates that the presence of a cleft in the hard palate and/or additional conditions have a negative impact on the development of the velopharyngeal function.     

Maxillary growth of adult patients with unoperated cleft: answers to the debates.

Maxillary growth of adult patients with unoperated cleft has long been studied, but results varied between different studies. The objectives of this study were to determine the growth potential of adult patients with unoperated clefts compared to the normal population and to differentiate the growth potential among types of clefts. METHODS: Subjects were from the same ethnic group, were more than 16 years of age with non-syndromic cleft and no associated anomalies. The types of cleft included unilateral complete cleft lip and palate (UCLP), bilateral complete cleft lip and palate (BCLP), unilateral cleft lip (UCL) and isolated cleft palate (CP). The sella-nasion-A point (SNA) angle from the cephalometry was measured, and a dental cast study using a three-dimensional imaging system was performed. RESULTS: SNA measurements showed significant differences among different groups, the BCLP group having larger values and the CP group having smaller values when compared with normal values. Dental cast analysis measuring palatal surface area showed a significantly smaller area in patients with cleft as compared to normal controls, but no difference among the different types of cleft. From the linear measurement it was found that the interdental distance was significantly more narrow in the anterior part up to the first premolar region as compared to the control group, especially in the complete cleft groups (UCLP and BCLP). Dentoalveolar arch was also deeper and longer in these complete cleft groups. CONCLUSION: There is an intrinsic tissue deficiency in all groups of patients with cleft; however, the sagittal development is still comparable to that of a normal population. Tissue deficiency mostly occurs in the anterior part. There is no difference in terms of the deficiencies among the different groups of cleft.     

Speech after repair of isolated cleft palate and cleft lip and palate.

The speech of children with isolated cleft palate (CP) repaired by one surgeon has been compared with the speech of children with some form of unilateral cleft lip and palate (CLP) repaired by the same surgeon. All palate repairs included an intravelar veloplasty. We identified 57 children (5--12 years old) with cleft palates repaired in infancy, of which three patients with other medical problems were excluded. Of the 54 patients, 44 (81%) attended for review (27 CP, 17 CLP). Video recordings were analysed by two speech and language therapists, using the Cleft Audit Protocol for Speech. The CP patients had no evidence of permanent fistulas. Final speech outcomes were similar for CP and CLP patients. Intelligibility was normal in 10 (37%) CP and nine (53%) CLP patients. Mild consistent hypernasality was present in five (18.5%) CP and four (23.5%) CLP patients. No patients had moderate or severe hypernasality or nasal emission. Mild consistent hyponasality was present in five (18.5%) CP and five (29%) CLP patients. Moderate consistent hyponasality was present in one (4%) CP patient. Dysphonia was present in eight (30%) CP and seven (41%) CLP patients. Cleft-type characteristics were noted in 11 (41%) CP and nine (53%) CLP patients. No CLP patients but 10 (37%) CP patients had required a pharyngoplasty (P=0.004, Fisher's exact test). Possible reasons for this (age, cleft type, surgeon and surgery) are discussed.