Difficult laryngoscopy in cleft lip and palate surgery

We studied prospectively 800 paediatric patients undergoing repair of cleft lip and palate to determine the predictors of difficult laryngoscopy. The incidence of difficult laryngoscopy (Cormack and Lehane grade III and IV) was 2.95% in patients with unilateral cleft lip, 45.76% in bilateral cleft lip and 34.61% in patients with retrognathia. Tracheal intubation was successful in 99% of patients in whom laryngoscopy was difficult. There was a significant association between age and laryngoscopic view (P < 0.01).      

Assessing blood loss in cleft lip and palate surgery

A method of continuously assessing peroperative blood loss is presented. A calibrated trap which collects all the blood loss is placed in the suction tubing. Bipolar diathermy and suction maintain a bloodless wound. A test using 20 ml of fresh venous blood showed that approximately 5 ml remained clotted in the tubing and 15 ml entered the trap. The method is particularly applicable to cleft lip and palate surgery.     

Van der Woude syndrome (lip pits, cleft lip and cleft palate syndrome)

Three patients with lip pits among 701 cases (0.43%) with clefts were treated in Beijing Stomatological Hospital in last five years. Genetic analysis of cases in 5 families with Van der Woude syndrome (Lip pits, cleft lip and cleft palate) in China was studied. We report on three unrelated families from the Beijing area, other three families from the Hebei, Heilongjang, Gansu province, in which the autosomal dominant gene for Van der Woude syndrome is segregating. All three or one of the clinical features may occur in an affected individual who carries the gene. Two probands had ankyloglossia. Six members were missing upper lateral incisors. One proband was missing second premolars. Six members were cross-bite. Two probands had congenital fistula of the mouth angle.     

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.     

Neonatal surgery of cleft lip and palate: juridical aspects

Neonatal surgery of cleft lip and palate is a common practice fro many surgical teams. The modifications of the French Law in 2002 on patient's rights, the way judges decide how the law should be applied, and the modifications of the rules for health centres need an analysis of the juridical aspects of this surgery. First the bases of the Law are presented, then how the surgeon responsibility can be engaged is learned.     

鼻-牙槽术前矫治器在唇腭裂治疗中的应用及进展

作为唇腭裂的一种辅助治疗手段,McNeil提出的现代术前正畸已有50多年的历史。随着研究的逐步深入和方法的不断改进,术前正畸已取得了很大的进展,并成为唇腭裂序     

右美托咪定用于小儿唇腭裂手术麻醉的临床观察

目的 探讨右美托咪定用于小儿唇腭裂手术麻醉中的临床效果,为小儿唇腭裂手术麻醉安全用药提供依据.方法 先天性唇腭裂修复术患儿40例,随机分为右美托咪定组(D组)和对照组(C组).两组患儿均采用紧闭面罩8％七氟烷吸入麻醉诱导,D组同时静脉泵注右美托咪定1 μg/kg,C组给予同等容量生理盐水,均于10 min给完.两组患儿均采用气管插管静吸复合全身麻醉方法,术中维持C组采用异丙酚-七氟烷复合麻醉;D组采用右美托咪定-异丙酚-七氟烷复合麻醉,观察并记录不同时点患儿心率、平均动脉压、脉搏血氧饱和度、呼气末二氧化碳分压并进行躁动评分,记录两组呼吸抑制及躁动发生率.结果 两组患儿的手术时间、麻醉时间及苏醒时间差异无统计学意义(P＞0.05).D组患儿在气管插管即刻(T2)、术中20 min(T3)、术毕(T4)及拔管(T5)时平均动脉压和心率均明显低于C组(P＜0.05).D组躁动发生率为11.1％,明显低于C组36.4％(P＜0.05).D组无一例发生呼吸抑制.结论 右美托咪定用于小儿唇腭裂修复术安全有效,术中血流动力学平稳,术后恢复期安静、舒适,能够减少围术期并发症的发生.     

Digits in the mouth; a peculiar chapter in cleft lip and palate surgery

In 1901 von Eiselsberg reported closing a fistula of the hard palate using the patient's little finger. The case is reviewed here and set in the context of its time.     

Separate clefts of the lip and the palate. A variant of cleft lip and palate

Separate clefts of the lip and of the palate (CL-CP) may belong to the same etiological class as the cleft lip with or without cleft palate CL(P), or a child may have two separate anomalies, CL and CP. This theory was tested in Finnish cleft patients. Among 2471 cleft cases, there were 66 CL-CP (2.7%). Adequate medical records were available for 62 children: 45 boys (73%) and 17 girls (27%). Familial occurrence was recorded in 6 cases (10%). Of the cleft cases among the near relatives, 5 were CL(P) and one CP. The prevalence of hypodontia was 37% among 38 subjects studied, as compared with 8.2% in the CL-, 29.8% in the CP- and 48.1% in the CLP controls. Conical elevations of the lower lip were observed in none, as compared with 0.8% of the CL(P)- and 39% of the CP controls. It was therefore assumed that the CL-CP belongs to the same etiological class as the CL(P).     

Frequency of homologous blood transfusion in patients undergoing cleft lip and palate surgery

Aim:

The study aims to determine the frequency of homologous blood transfusion in patientsundergoing cleft lip and palate surgery at the Lagos University Teaching Hospital, Nigeria.

Setting and Design:

A prospective study of transfusion rate in cleft surgery conducted at the Lagos University Teaching Hospital, Nigeria.

Material and Methods:

One hundred consecutive patients who required cleft lip and palate surgery were recruited into the study. Data collected included age, sex and weight of patients, type of cleft defects, type of surgery done, preoperative haematocrit, duration of surgery, amount of blood loss during surgery, the number of units of blood cross-matched and those used. Each patient was made to donate a unit of homologous blood prior to surgery.

Results:

There were 52 females and 48 males with a mean age of 64.4 ± 101.1 months (range, 3-420 months). The most common cleft defect was isolated cleft palate (45%) followed by unilateral cleft lip (28%). Cleft palate repair was the most common procedure (45%) followed by unilateral cleft lip repair (41%). The mean estimated blood loss was 95.8 ± 144.9 ml (range, 2-800ml). Ten (10%) patients (CL=2; CP=5, BCL=1; CLP=2) were transfused but only two of these were deemed appropriate based on percentage blood volume loss. The mean blood transfused was 131.5 ± 135.4ml (range, 35-500ml). Six (60%) of those transfused had a preoperative PCV of < 30%. Only 4.9% of patients who had unilateral cleft lip surgery were transfused as compared with 50% for CLP surgery, 11% for CP surgery, and 10% for bilateral cleft lip surgery.

Conclusions:

The frequency of blood transfusion in cleft lip and palate surgery was 10% with a cross-match: transfusion ratio of 10 and transfusion index of 0.1. A "type and screen" policy is advocated for cleft lip and palate surgery.     

米库氯铵与顺苯磺酸阿曲库铵在婴幼儿唇腭裂手术中的应用比较

目的观察和比较米库氯铵和顺苯磺酸阿曲库铵用于婴幼儿唇腭裂手术中的肌松效应和安全性。方法 2012年9月至2013年9月择期行唇腭裂手术治疗的患儿60例,年龄6个月至3岁,随机分为两组,每组30例。两组均给予咪达唑仑0.1mg/kg、丙泊酚2mg/kg、瑞芬太尼1μg/kg及米库氯铵0.2mg/kg(M组)或顺苯磺酸阿曲库铵0.15mg/kg(C组)行麻醉诱导后气管插管。采用TOF-Guard加速度肌松监测仪连续监测尺神经四个成串刺激(TOF)。观察并记录肌松起效时间、临床作用时间和恢复指数,进行插管评级并观察不良反应情况。结果两组的插管条件评级差异无统计学意义。M组肌松起效时间、肌松临床作用时间以及恢复指数明显短于C组(P0.05);M组不良反应发生率明显低于C组(P0.05)。结论米库氯铵用于婴幼儿唇腭裂手术时起效快、肌松效应时间短,恢复较快。     

Management of cleft lip and palate in Brazil

We aimed to evaluate the treatment protocols for cleft lip and palate that are used in Brazil, to compare them with the ones proposed from elsewhere, and to discuss the official data about admission for treatment of cleft lip or palate, or both, in Brazil. We also assessed the importance of integrated action of different specialities to treat this condition. A questionnaire related to attendance protocols was developed and sent out to all Brazilian Plastic Surgery Services connected to the Brazilian Society of Plastic Surgery, and to other units involved in such treatment. We also studied the data produced by the Brazilian Department of Health about the operations done during the past five years. Many protocols were identified, but despite much controversy in many areas, a consensus was reached about the surgical techniques, the age group most suitable to be operated on, and there was total agreement about the need for multidisciplinary management. According to the State Department of Health, the number of operations done in 1995 was 9696, and this had increased to 21?022 in 1999. The state of São Paulo had done 17?636 (84%) of all procedures in 1999.     

Current concepts in the embryology and genetics of cleft lip and cleft palate

Many mechanisms underlying normal and abnormal craniofacial embryogenesis are well understood. The genetic factors that provoke abnormal development and result in orofacial clefts are not clear, but much progress has occurred in our understanding. Genes or chromosomal rearrangements on many chromosomes can lead to syndromes that include orofacial clefts. This diversity in the mechanisms that can lead to syndromic clefts highlights the fact that the processes leading to the development of the oral cavity and face are complex and sensitive to disturbances at multiple timepoints or within multiple genetic domains. As for nonsyndromic clefting, large-scale family studies are consistent with one or a few loci exerting major effects on phenotypic expression, although no single gene has been identified as a "necessary" locus for development of nonsyndromic clefts. Rather, the emerging consensus is that the genetic etiology of nonsyndromic clefting is complex, with several loci showing significant results in at least some studies. Some of these loci may be genes for susceptibility to environmental factors, some may be modifying loci, and some may be "necessary" loci. Mutations in genes that are now known to control early development are logical candidate genes for future studies of nonsyndromic orofacial clefting. Continued genetic analyses and developmental studies are crucial for eventual understanding of the complex etiology of these common congenital anomalies.