5459例唇腭裂患者临床资料分析

目的：对20年来唇腭裂的治疗及唇腭裂患者的构成情况进行回顾。方法：对5459例唇腭裂患者病历资料分类整理，进行统计学分析。结果：唇腭裂各类型性别比例差异有显著性意义，唇腭裂患者的首次手术年龄差异有显著性意义，其主要修复方式随年代的变化而不同。结论：唇腭裂患者中男性明显多于女性。自1980年以来，唇腭裂患者的首次手术年龄呈逐渐下降趋势，主要手术方法也有很大的变化。     

A revised classification of the cleft lip and palate

BACKGROUND:

Submucous cleft palate is characterized by muscular diastasis of the velum in the presence of intact mucosa with variable combinations of bifid uvula and hard palatal defect. Submucous cleft palate is indicated as a separate entity in most previous classifications but it has never been properly classified on an anatomical basis.

OBJECTIVES:

To revise the Smith-modified Kernahan ‘Y’ classification of cleft lip and palate deformities, and to describe the different anatomical subtypes of submucous cleft palate.

METHODS:

The present study was conducted in Hayatabad Medical Complex, Abasin Hospital and Aman Hospital Peshawar, Pakistan, from November 2010 to December 2011. All patients who presented to the outpatient departments with cleft lip and palate, with the exception of previously operated cases, were included. All cases were described according to the Smith-modified Kernahan ‘Y’ classification and the authors’ revised Smith-modified Kernahan ‘Y’ classification. All of the data were organized and analyzed using SPSS version 17 (IBM Corporation, USA).

RESULTS:

A total of 163 cases of cleft lip and palate deformities were studied, of which 59.5% were male and 40.5% were female. Smith modification of the Kernahan ‘Y’ classification completely described the cleft deformities in 93.9% of patients. However, while the Kernahan ‘Y’ classification represented the submucous cleft palate, it did not describe its different anatomical subtypes in 6.13% of patients. The revised Smith-modified Kernahan ‘Y’ classification completely described the cleft deformities of the entire study population, including the different submucous cleft palate patients.

DISCUSSION:

The Smith alphanumeric modification of the Kernahan ‘Y’ classification of cleft lip and palate came into existence after a long search and a series of modifications over the past century. This classification system describes the cleft region, site of the cleft, degree of the cleft, rare and asymmetrical clefts, and are computer database friendly. However, this classification did not describe the different anatomical subtypes of submucous cleft palate that have variable relationships with velopharyngeal insufficiency.

CONCLUSION:

The revised Smith-modified Kernahan ‘Y’ classification described in the present study can describe all types of cleft lip and palate deformities in addition to the different types of submucous cleft palate deformities.     

Report of a hemohiliac's cleft lip and palate repair — ten year follow-up

Summary The association of cleft lip and palate with hemophilia is rare. Recent advances in hematology have made it possible for hemophiliacs to have surgery without problems, using prior administration of anti-hemophilic globulin. The case reported was a hemophilia B patient with unilateral cleft lip and palate. When primary cheiloplasty was carried out at four months of age, there were serious bleeding problems during and after the operation. Palatoplasty was performed at age two and repair of secondary deformity of the cleft lip at age eight. On both occasions a prothrombin complex was administered, and no problem was experienced in either procedure. A ten year follow-up is presented.     

单侧唇裂继发畸形整复术的术式改良

目的 探讨改良术式修复单侧唇裂术后继发唇、鼻畸形的效果.方法 手术切几线与口鼻轮廓线相一致,片使鼻翼外侧脚整体旋转复位,重建鼻槛及鼻底,通过鼻腔的V-Y黏软骨瓣使鼻翼软骨上推,矫正鼻畸形.结果 自2000年以来,应用此方法对69例单侧唇裂继发唇、鼻畸形患者进行了修复,均取得较满意的效果,术后瘢痕线不明显.结论 轮廓线切口以及鼻翼软骨上推复位的方法符合唇、鼻的解剖特征,是一种较好的手术方法.     

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。     

Anatomical closure technique of the nasal floor for patients with complete unilateral cleft lip and palate

Abstract

We describe a new technique for the reconstruction of the nasal floor at the same time as cheiloplasty in patients with complete unilateral cleft lip and palate. We operated on patients aged between 3 and 36 months in public secondary and tertiary level institutions. None of these patients had had a previous operation for the correction of the cleft lip or palate. The operation required the design of two mucous flaps, one lateral and one medial to the defect, to reposition the tissues anatomically and repair the congenital deficiency. Three hundred and fifty-eight patients have been treated using this technique, most of whom (n = 233, 65%) were boys, and 288 (80%) presented with a right complete unilateral cleft. Postoperative evaluation showed that 22 patients (6%) had asymmetry of the nasal base equal to or less than 1 mm, 18 (5%) had nasovestibular fistulas, and 5 (1%) required revision. We conclude that this technique greatly reduces the number of asymmetrical nasal floors and the incidence of nasovestibular fistulas.     

唇腭裂病人成年后的综合美容

既使是近乎完美的唇腭裂早期治疗，随着人体面部器官的生长发育，仍有可能出现鼻部、唇部畸型，因此，唇腭裂后期治疗至关重要。通过对１６０２例早期及后期唇腭裂病人的治疗体会与总结，指出各类型唇腭裂病人成年后常见的各种面颌部畸形。阐述了适用于该类病人的手术前设计、治疗原则、治疗步骤及方法。针对唇腭裂病人成年后所出现的畸形往往是多方面的这一现象，结合口腔颌面外科、正畸修复科、整形美容外科等专科特点，提出了综合美容整形的设计思路及治疗要点     

红唇瓣推进修复双侧唇裂继发红唇缺损

目的 介绍一种双侧唇裂术后继发红唇缺损的修复方法。方法 于缺损红唇的两侧红白唇交界处或其稍上方按需要向其两侧各设计红唇瓣，缺损范围大都可将一侧红唇瓣设计至口角下唇处，按设计切开解剖游离以唇动脉为蒂的红唇轴型瓣，向缺损区推进修复缺损。结果 临床应用15例。缺损宽度1.0-1.2cm，所有唇瓣全部成活，术后唇外形满意。结论 以唇动脉为蒂的红唇瓣推进修复红唇缺损，方法简便，唇瓣血供可靠，疗效满意，可广泛用于修复各种原因引起的红唇适当大小的部分缺损。     

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.     

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     

Early postoperative complications in primary cleft lip and palate surgery

We studied the occurrence of early complications of cleft lip and palate surgery by the retrospective study of 132 consecutive cases (160 procedures) operated between three and six months of age. Three children presented with major complications within the immediate postoperative period. All were treated successfully. A local complication directly related to the operation occurred in 7.5% of the cases within the first six postoperative days. In 17%, a general complication not directly related to the operation was observed. The majority of these general complications were cross infections during the hospital stay. These observations led us to reduce the hospital stay to one day for cleft lip and five days for cleft palate and cleft lip and palate operations without compromise to safety but with a reduction of the morbidity and hence the treatment costs and of the child-family disturbance.     

牙槽嵴裂和唇裂继发唇鼻畸形的同期联合矫治

目的 探讨牙槽嵴裂和唇裂继发唇、鼻畸形矫治的方法. 方法 对唇、腭裂术后畸形患者同期行牙槽嵴裂和唇裂继发唇、鼻畸形联合矫正. 结果 2004年～2007年,于临床应用37例.33例牙槽受植床创口一期愈合,3例松质骨外露,经清除外露骨和冲洗换药后愈合.本组患者术后正面观唇部饱满,红唇两侧高度基本对称,干湿唇线连续;仰视位鼻翼基底部高度恢复良好,两侧基本对称,鼻孔方向一致,但患侧鼻孔仍稍小于健侧. 结论 同期联合矫治牙槽嵴裂和唇裂继发唇、鼻畸形效果良好.     

α转化生长因子基因BamHⅠ限制酶切片段长度多态性与广东地区人群非综合征性唇腭裂的关系

目的 探讨中国广东地区人群非综合性唇腭裂与α转化生长因子(transforming growth factor α,TGF-α)基因多态性的关系.方法 应用聚合酶链式反应-限制酶切片段长度多态性(PCR-RFLP)核苷酸分型技术,以BamH Ⅰ限制性内切酶消化PCR扩增产物,对107例非综合征性唇裂或唇腭裂(nonsyndromic cleft lip with or without cleft palate,NSCL/P)患者,136例正常人为对照组的TGF-α/BamH Ⅰ等位基因多态性进行分析.结果 NSCL/P患者的A1等位基因频率比正常对照组明显增高,差异有统计学意义(P＜0.05).NSCL/P患者中,有家族史与无家族史者等位基因频数,差异无统计学意义(P＞0.05);双侧NSCL/P患者与单侧NSCL/P患者的TGF-α/BamH Ⅰ基因型频率,差异无统计学意义(P＞0.05).结论 中国广东地区人群NSCL/P患者TGF-α基因中存在BamH Ⅰ多态性位点,TGF-α基因BamH Ⅰ位点与中国广东地区人群NSCL/P的发生有关.     

双侧唇裂修复术的改进

要双侧唇裂畸形多较复杂,修复术后常出现上唇过紧,唇红过薄等继发畸形。为矫正畸形,设计了在直线修复术的基础上增设鼻唇沟瓣及两个小三角瓣的手术方法,取得了较满意的临床效果。认为本方法可恢复前唇的正常功能,有利鼻的发育。     

双侧唇裂修复术的改进

双侧唇裂畸形多较复杂，修复术后常出现上唇过紧，唇红过薄等继发畸形。为矫正畸形，设计了在直线修复术的基础上增设鼻唇沟瓣及两个小三角瓣的手术方法，取得了较满意的临床效果。认为本方法可恢复前唇的正常功能，有利鼻的发育。     

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。     

异氟醚与氯胺酮麻醉诱导对婴儿唇腭裂修复术后苏醒的比较

目的比较异氟醚与氯胺酮麻醉诱导对婴儿唇腭裂术后苏醒的影响.方法对208例1～12月婴儿全麻下行唇腭裂修复术的病例进行分析,按照不同的麻醉诱导方法分为氯胺酮组(K组)和异氟醚组(Ⅰ组),比较两组术后自主呼吸恢复时间、拔管时间、清醒时间、送入恢复室的例数、恢复室停留时间以及苏醒期躁动发生率.结果Ⅰ组自主呼吸恢复时间、拔管时间、清醒时间、送入恢复室的例数以及恢复室停留时间均少于K组,而Ⅰ组苏醒期躁动的发生率高于K组.结论和氯胺酮诱导相比,异氟醚吸入诱导用于婴儿唇腭裂修复术有利于患儿术后的快速苏醒,但同时苏醒期躁动的发生率增加.     

A status report on management of cleft lip and palate in India

Introduction:

This national survey on the management of cleft lip and palate (CLP) in India is the first of its kind.

Objective:

To collect basic data on the management of patients with CLP in India for further evaluation.

Materials and Methods:

A proforma was designed and sent to all the surgeons treating CLP in India. It was publicized through internet, emails, post and through personal communication.

Subjects:

293 cleft surgeons representing 112 centers responded to the questionnaire. Most of the forms were filled up by personal interview.

Results:

The cleft workload of the participating centers is between 10 and 2000 surgeries annually. These centers collectively perform 32,500–34,700 primary and secondary cleft surgeries every year. The responses were analyzed using Microsoft excel and 112 as the sample size. Most surgeons are repairing cleft lip between 3-6 months and cleft palate between 6 months to 1 year. Millard and Tennison repairs form the mainstay of lip repair. Multiple techniques are used for palate repair. Presurgical orthopedics, lip adhesion, nasendoscopy, speech therapy, video-fluoroscopy and orthognathic surgery were not always available and in some cases not availed of even when available.

Conclusion:

Management of CLP differs in India. Primary surgical practices are almost similar to other studies. There is a lack of interdisciplinary approach in majority of the centers, and hence, there is a need for better interaction amongst the specialists. A more comprehensive study with an improved questionnaire would be desirable.     

毛发游离移植治疗唇裂修复术后上唇瘢痕

目的应用毛发游离移植胡须再造,治疗唇裂修复术后遗留的上唇瘢痕。方法切取枕部头皮,分割成含单一毛囊的皮条,供区缝合,应用崔式植毛器移植到上唇瘢痕。结果临床应用4例,单侧唇裂3例,双侧唇裂1例,移植毛发生长良好,效果满意。结论毛发游移植治疗唇裂修复术后上唇瘢痕是一种切实可行的方法。