The clinical observation of difficult laryngoscopy and difficult intubation in infants with cleft lip and palate

BACKGROUND: The aims of this study were to evaluate the incidence of difficult laryngoscopy in infants with cleft lip and palate and to observe its relationships with age, sites, and degrees of deformities. METHODS: A total of 985 infants aged 1 month to 3 years, undergoing repair of cleft lip and palate were included in this study. The infants suffering from unilateral cleft lip, simple cleft palate, and combined bilateral cleft lip and palate were 465, 421, and 79 respectively. They were divided into three groups according to age; 1-6 months group, 6-12 months group and 1-3 years group. RESULTS: The total incidence of difficult laryngoscopy was 4.77%. The incidence of difficult laryngoscopy was closely related to age, sites and degrees of deformities, and micrognathia. The incidence of difficult laryngoscopy was 7.06% in 1-6 months group, 2.90% in 6-12 months group, and 3.13% in 1-3 years group, and was greatest for infants with combined bilateral cleft lip and palate, less for those with left cleft lip and least for those with right cleft lip and simple cleft palate. The incidences of difficult laryngoscopy in infants with and without micrognathia were 50% and 3.83% respectively. The incidences of moderately difficult, difficult, and failed intubations were 1.02%, 0.91%, and 0.102% respectively. CONCLUSIONS: Infants with cleft lip and palate, left cleft lip and alveolus, combined bilateral cleft lip and palate, micrognathia, and age <6 months were the important risk factors for difficult laryngoscopy. Difficult intubation occurred mainly in infants with laryngoscopic views of grade III and IV.     

先天性唇腭裂患儿的围手术期护理

目的：先天性唇腭裂是口腔颌面部最常见的先天畸形,探讨如何加强对唇腭裂患儿的围手术期护理,以达到最理想的手术及术后康复效果。方法：统计我科2009年1月1日～2010年12月31日收治的120例唇腭裂患儿,总结围手术期护理经验。结果：所有患儿经过精心的护理、心理和康复治疗,均获得良好的治疗效果。结论：良好的围手术期护理以及心理护理是唇腭裂患儿手术成功及术后康复的重要保障。     

Anaesthesia for primary repair of cleft lip and cleft palate: a review of 244 procedures

The peri-operative course of 194 patients undergoing 244 procedures for primary repair of cleft lip and palate over an 8-year-period was studied. A marked increase in the extent of intra-operative monitoring was noted during this period. The Pierre Robin syndrome was the most common associated abnormality and was found in 17% of patients in this series. There were no deaths. A total of 101 procedures were undertaken in infants with cleft lip of whom 10% received an intra-operative blood transfusion. Post-operative opiate analgesics were administered following 97% of these procedures and profound respiratory depression was observed in three children. The use of lignocaine and noradrenaline did not significantly reduce the operative blood loss. A post-operative pyrexial illness was significantly associated with the presence of a positive pre-operative nasal and throat swab and this could be significantly reduced by pre-operative antibiotic treatment. A total of 143 children underwent repair of cleft palate and of these 16.8% received an operative blood transfusion. An elective tracheostomy was required in one patient because of unsuccessful attempts at endotracheal intubation. One patient developed a respiratory arrest after two doses of diamorphine peri-operatively. The use of lignocaine and noradrenaline significantly reduced the operative blood loss. The presence of a positive bacteriological nose or throat swab did not influence the development of a post-operative pyrexia which could however be significantly reduced by the use of pre-operative antibiotics.     

271例婴幼儿完全性唇腭裂一期修复及初步观察

目的 探索婴幼儿完全性唇腭裂一期修复的可行性，并对其效果进行初步观察。方法 对3－12个月婴儿安全性唇腭裂进行了一期修复，同时对24例裂隙宽大的患儿进行术前腭部矫治，对术后1－4年的116例患儿唇的外形及事音进行了初步评价。结果 271例婴幼儿完全性唇腭裂修复手术，术后除2例发生呼吸困难，6例腭部瘘孔形成及5例作品渗血外，全部愈合良好。研究发现19例单侧完全性唇腭裂术前腭部矫治后，齿槽部裂隙左右距离轿治前平均缩小6．1mm；前后距离轿较矫治前平均缩小6．6mm；唇外菜评价优良率达93．1％，语音评价优良率达94．8％。结论 婴幼儿完全性唇腭裂一期是完全的、可行的。术前腭部桥治可明显缩小齿槽部的裂隙，有利于宽大裂隙的修复。婴幼儿完全性唇腭裂一期修复可获良好唇外形及语音功能恢复。     

颌面部神经阻滞在唇腭裂修复术围手术期的应用进展

背景 唇腭裂在小儿先天性疾病中占很高比列,小儿唇腭裂修复术围手术期的疼痛对喂养和伤口愈合有很大影响. 内容 总结分析颌面部神经阻滞对唇腭裂患儿围手术期镇痛、术后苏醒、颌面部神经阻滞相关并发症以及对术后应用镇痛药物各方面的影响. 目的 通过对小儿唇腭裂修复术围手术期颌面部神经阻滞镇痛方式、应用药物研究现状的总结归纳,探讨颌面部神经阻滞用于唇腭裂修复术围手术期的镇痛效果和副作用,为唇腭裂修复术围手术期镇痛提供有效的依据,完善小儿镇痛. 趋向 颌面部神经阻滞是唇腭裂修复术围手术期较为理想的一种镇痛方式,超声的引进使小儿术后镇痛得到进一步完善.但目前仍缺乏一种绝对满意、安全的颌面部神经阻滞方法,相关内容仍需进一步临床研究.     

Failure to thrive in babies with cleft lip and palate.

We established the frequency of failure to thrive (FTT) in children undergoing primary cleft procedures by using growth charts and standard-deviation scores. Initially, 147 babies with cleft lip and/or palate undergoing 186 primary lip-and-palate repairs were studied between 1993 and 1996. Rates of FTT were categorised according to cleft type. There was an increasing rate of FTT from 32% for unilateral cleft lip and palate to 38% for bilateral cleft lip and palate to 49% for cleft palate. There was a high incidence of FTT in palatal clefts, especially if these were associated with a syndrome or anomaly (P= 0.001). The incidence of FTT with the Pierre Robin sequence was 100%. In view of the high rates of FTT, two changes were instituted: a feeding-support nurse was appointed to supervise and monitor patients at risk and all patients with the Pierre Robin sequence had supervised airway management. Thereafter, the incidence of FTT was prospectively studied in 68 babies undergoing 84 primary procedures between 1997 and 1999. There was a decrease in the incidence of FTT in comparison with the earlier cohort (9% for unilateral cleft lip and palate, 20% for bilateral cleft lip and palate, 26% for cleft palate). There was a significant decrease in the incidence of FTT in the group with the Pierre Robin sequence, from 100% to 40%. As a result of the provision of a feeding-support nurse and airway management of patients with the Pierre Robin sequence, the incidence of FTT was reduced and the audit loop closed.     

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。     

Malnutrition in cleft lip and palate children in Uganda

The objective of this study is to investigate the nutritional status of patients with cleft lip and/or palate when compared to non-cleft lip or palate patients. A retrospective analysis was carried out of all patients aged less than 1 year who were operated on in the Comprehensive Rehabilitation Services in Uganda hospital since opening in April 2009 to November 2010. The data was divided into three groups: cleft lip patients; cleft lip and palate patients and non-cleft patients. The WHO anthropometric calculator was used to calculate weight-for-age Z scores on each patient for the initial outpatient appointment and the operation. The demographic data and Z scores were compared using independent T tests. Three hundred and twenty-one patients were identified, 131 patients had cleft lip alone, 112 patients had cleft lip and palate and 78 patients had no cleft. The cleft lip and palate group had significantly lower Z scores for both the outpatient appointment and operation (i.e. were more malnourished) than either the cleft lip group or the non-cleft group. Malnutrition is a well-documented problem associated with cleft lip and palate. Our research confirms this malnutrition but also highlights the severity of the malnutrition. The patients with cleft lip and palate are unable to feed adequately and therefore need intervention. We operate on these patients once they reach a target weight of 3 kg and repair both lip and palate in one operation to enable patients to feed and improve their nutritional status.     

Length of the cleft in relation to the incidence of hypodontia of the second premolar and to inheritance of cleft lip and palate in children with isolated cleft palate.

We studied severity of the isolated cleft palate expressed as the length of the cleft in relation to hypodontia in the second premolar regions and known inheritance of any type of cleft lip and palate. The material consisted of 47 children at 10 years of age born with non-syndromic isolated cleft palate of varying extent, who all had hypodontia of at least one second premolar, and had panoramic radiographs taken at 10 years of age. Information about length of cleft and inheritance of cleft lip and palate was collected from surgical files initiated at birth. The higher the number of missing second premolars, the more extended was the length of the cleft. More children had hypodontia of the second premolar in the mandible than in the maxilla. The family history had little influence on the length of the cleft.     

22q11 Deletion in children with cleft lip and palate--is routine screening justified?

OBJECTIVE: To ascertain the prevalence of 22q11 deletion in children with a diagnosis of cleft lip and/or palate that had been referred to the Cleft Lip & Palate Service, Newcastle-upon-Tyne. DESIGN: Retrospective analysis of results of 22q11 FISH testing performed in all such referrals. PARTICIPANTS: 191 children, of whom 13 had a bilateral cleft lip and palate, two had a median cleft, 77 had a cleft palate only, 44 had a unilateral cleft lip, 47 had a unilateral cleft lip and palate and eight had a submucous cleft palate. RESULTS: nine patients had a positive 22q11 FISH test. CONCLUSION: This represents a higher percentage than has been previously reported. All children with cleft lip and/or palate should routinely have a 22q11 FISH test in view of the implications of a diagnosis of velocardiofacial syndrome.     

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.     

Prenatal diagnosis of cleft lip and palate.

A prospective study was conducted over a period of 8 years to compare the severity of the cleft lip and palate detected by ultrasound scanning of the foetus with the severity at birth. In the UK all pregnant women undergo a routine perabdominal ultrasound scan at 20 weeks' gestation. There were approximately 250000 scans performed in our catchment area during the study period. A total of 270 children were born with cleft lip and/or palate, of which 130 had cleft lip with or without cleft palate. Out of these, 23 were positively diagnosed by the ultrasound scan. The specificity was 100% and the sensitivity was 17.5%. Only two diagnoses led to termination of the pregnancy.     

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     

The incidence of cleft lip and palate in the Western Cape

The incidence of cleft lip and palate in the Western Cape was studied using data from two cleft palate centres and from all plastic surgeons in practice in the area. A high incidence was found among coloureds, while the incidences among whites and blacks were similar to figures reported from other countries.     

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.     

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。     

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.     

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。     

Ambulatory surgery for cleft lip repair

Standard of care for cleft lip repair has included preadmission testing, surgical correction, and postoperative hospital care. Driven not by managed care economics but to speed the safe home care of infants by parents, the authors have gained experience in ambulatory cleft lip repair. In this retrospective study the authors evaluated the outcome of patients who underwent ambulatory cleft lip repair compared with those patients who were hospitalized after surgery. From 1989 to 1998, 24 cleft lip repairs in 24 patients performed by the senior author were evaluated. Two groups were treated. Group 1 (N = 11) consisted of ambulatory unilateral cleft lip repairs and group 2 (N = 13) consisted of inpatient unilateral cleft lip repairs. Important surgical factors considered were technique of cleft lip repair, performance of ancillary procedures, type of local anesthetic administered, and intravenous steroid administration. Time to first postoperative feeding and complications, including bleeding, spontaneous or traumatic wound dehiscence, and infection, were considered important outcome parameters. There were no differences in surgical technique or use of antibiotics and postoperative analgesics between the two groups. None of the patients in group 1 underwent ancillary procedures. Four patients underwent soft palate repair and 3 patients underwent insertion of myringotomy tubes among group 2 patients. The use of a 1:1 mixture of 1% lidocaine and 0.5% bupivacaine with epinephrine vs. 1% lidocaine with epinephrine as a local anesthetic and intravenous steroid administration was greater in group 1 (92%) than in group 2 (33%) patients. The average time to the first postoperative feeding was more than 1 hour sooner in the ambulatory group (p < 0.05) compared with the hospitalized group (excluding the 4 patients who underwent soft palate repair). There were no complications among patients with ambulatory cleft lip repair, and there were two cases of minor wound separation in patients who received postoperative hospital care. Although many variables factor into the outcome after cleft lip repair, these data support the safety and continued practice of ambulatory cleft lip repair.     

Clinical and epidemiologic studies of nonsyndromic cleft lip and palate in china: analysis of 4268 cases

The aim of our study was to review clinical and epidemiologic characteristic of the nonsyndromic cleft lip and cleft palate over a period of 10 years at West China Stomatological Hospital, Sichuan University. Four thousand two hundred sixty-eight nonsyndromic cleft lip and cleft palate cases were retrospectively analyzed according to the following variables: general information, cleft type, maternal age, familial history, as well as associated malformations, et al. In these cases, 1075 of which were cleft lip, 1985 were cleft lip with cleft palate; 1208 were isolated cleft palate. There were more men than women in the cleft lip and cleft lip with cleft palate group, while there were more women than men in the isolated cleft palate group. There were significantly more unilateral cleft lip cases than cleft lip on both sides; the cases of cleft lip on the left side were more than that of cleft lip on the right side. The cases with inherited history accounted for 6.68% of all the cases. Most infection cases occurred among the deleterious factors and the complications experienced during the first 3 months after the pregnancy. In all of the 14 twin cases, only 1 of the babies was affected. There were totally 152 cases with other associated malformations. Patients with cleft lip born in November to January were less than those born in the other three quarters of the year. Patients of A blood group composed a higher proportion than the control group. Our data may provide references for appropriate resource use, cleft lip and cleft palate prevention programs, and counseling programs with China-specific data.