Adopted children with cleft lip or palate, or both, require special needs cleft surgery

In recent years adoption of children with cleft lip, with or without cleft palate (CLP), and other birth defects has become more common. The aim of the present study was to describe the characteristics and initial care and treatment of adopted children with CLP. A total of 25 children were referred to our department between 2008 and 2010, 7 (28%) of whom had bilateral CLP and 16 (64%) had unilateral CLP. Two children had atypical clefts. Twenty of the patients (80%) had been operated on with a lip plasty in China before adoption. Most patients (n = 14) was seen by the cleft team within two months of arrival, and 13 were operated on within a month of the first visit at our department. In total, 22 primary palatoplasties, 6 lip plasties, and 1 lip adhesion were done. There were 5 fistulas (14%) three months after the palatoplasty. On arrival, 11 (44%) of the children were carriers of methicillin-resistant Staphylococcus aureus (MRSA). Adoption of children with CLP creates new challenges for the cleft teams, as we no longer have control over the overall treatment plan as regards preoperative and surgical treatment and timing of the operations. The patients are also often carriers of resistant bacteria, which create nursing challenges. In cases where the child is older than a year and has not been operated on, we advocate that the palatoplasty, or combined lip plasty and palatoplasty, is always given priority so that speech development is not compromised.     

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.     

General intellectual capacity of young men with cleft lip with or without cleft palate and cleft palate alone.

We report on the general intellectual capacity of men around 17-19 years of age and with cleft lip either with or without cleft palate (CLP) or cleft palate alone. Data were obtained from the Swedish National Service enrollment register for the years 1991-1997, about 335 individuals with CLP and 88 with cleft palate alone, who were compared with a control group consisting of 272 879 people. Those with CLP showed no significant differences compared with the control group. The group with cleft palate alone had significantly lower general intellectual scores than the control group.     

Physical characteristics of young men with cleft lip,with or without cleft palate,and cleft palate only

We report the weight, stature, body mass index (BMI), and muscular strength of men about 19 years old who have cleft lip, either with or without cleft palate (CLP), or cleft palate only (CP). Data were obtained from the Swedish National Service enrolment register for the years 1991–97, and concerned 335 men with CLP and 88 with CP, who were compared with a control group of 272 879 men. The data showed that those with CLP and CP were significantly lighter than controls. Their stature in the CLP group was similar to that in controls, but those with CP significantly shorter. These findings imply that men with CLP had a significantly lower BMI whereas men with CP had a similar BMI compared with controls. Those with CLP did not differ as regards to muscular strength, but those with CP were significantly weaker than controls.     

Physical characteristics of young men with cleft lip, with or without cleft palate, and cleft palate only.

We report the weight, stature, body mass index (BMI), and muscular strength of men about 19 years old who have cleft lip, either with or without cleft palate (CLP), or cleft palate only (CP). Data were obtained from the Swedish National Service enrolment register for the years 1991 -97, and concerned 335 men with CLP and 88 with CP, who were compared with a control group of 272 879 men. The data showed that those with CLP and CP were significantly lighter than controls. Their stature in the CLP group was similar to that in controls, but those with CP significantly shorter. These findings imply that men with CLP had a significantly lower BMI whereas men with CP had a similar BMI compared with controls. Those with CLP did not differ as regards to muscular strength, but those with CP were significantly weaker than controls.     

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.     

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.     

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).     

TGF-α基因TaqⅠ RFLPs与国人非综合征性唇腭裂关系的研究

目的　探讨国人非综合征性唇腭裂与转化生长因子 α(transforminggrowthfactoralpha ,TGF α)基因多态性的关系。方法　应用聚合酶链反应 限制性片段长度多态性核苷酸分型技术 ,以TaqⅠ限制性内切酶消化PCR扩增产物 ,对 10 7例非综合征性唇裂或唇腭裂 (nonsyndromiccleftlipwithorwithoutcleftpalate,NSCL P)患者、136例正常人的TGF α TaqⅠ等位基因进行分析。结果　NSCL P患者的C2等位基因频率与正常对照组明显增高 ,差异有显著性意义 (P <0 0 5 ) ;有家族史的患者的TGF α TaqⅠ基因型与无家族史患者比较 ,差异有显著性意义 (P <0 0 5 )。结论　TGF α基因多态性与国人的非综合征性唇腭裂的发生有关 ;有先天性唇腭裂家族史可能与TGF α TaqⅠ少见变异体相互关联。     

On the pathogenesis of cleft palate in the Pierre Robin syndrome

In a series of Pierre Robin syndrome (PRS) and isolated cleft palate patients (ICP) both U- and V-formed clefts were observed with equal frequency, but the PRS clefts were in average slightly wider. There were totally submucous clefts among the PRS patients. There was no statistical difference between the groups in the prevalence of clefts in the relatives of the patients. The incidence of (genetically influenced) conical elevations in the lower lip was lowest in the noncleft subjects, high in ICP and highest in PRS children. The incidence of hypodontia, which acceptedly is genetically influenced, was also highest in the PRS group. Thus the foetal malposition with the tongue between the palatal shelves does not seem to play any decisive role in the pathogenesis of most PRS clefts. The cause for the PRS is more likely a genetically influenced growth disturbance in the maxilla and the mandible, which due to the organogenetic differences leads to diverging end results, micrognathia and cleft.     

Oral clefts in the Sultanate of Oman

This is a retrospective study of the epidemiology of congenital clefts of the lip and palate in babies of Omani nationals born in the Sultanate of Oman between 1989 and 1995. Babies of non-Omani immigrants were excluded from the study. The data were collected from two sources. The first was the Centralized Plastic Surgery Service of Khoula Hospital at Muscat, the capital city of Oman between 1989 and 1995, which registers all oral cleft cases in the country. These data revealed the overall incidence of oral clefts (OC) to be 1.5 per 1000 live births, with incidences of 0.62 per 1000 for combined lip and palate (CLP) cleft, 0.34 per 1000 for cleft lip (CL), and 0.54 per 1000 for isolated cleft palate (CP). The second data source was national delivery records from maternity sections of all hospitals in the country from 1989 to 1995, which include oral clefts as congenital anomalies identified at birth. These statistics were similar to those from the first source for CL/CLP but underestimated the number of isolated cleft palates (CP). The histories of patients attending the Plastic Surgery Clinic of Khoula Hospital during the last year of the study period could be examined in detail. A study of the 177 OC cases registered in 1995 alone revealed associated congenital anomalies in 38.4%. A higher inbreeding coefficient of 0.0294 was noted among oral cleft cases than in the general population, which was calculated at 0.0198. Familial clefts accounted for 23% of all cases. Greater parental age and birth order were found to be associated with increased risk of oral cleft. High temperatures during the tropical desert summers in Oman (when temperatures reach 48°C) do not seem related to the incidence of oral clefts.     

A six-year follow-up study of 155 children with cleft lip and palate

A total of 155 consecutive cases from three "Six-year-old follow-up clinics" have been reviewed to assess the speech results in boys and girls with cleft lip and palate belonging to Group II (complete labio-palatal clefts) and Group III (varying degrees of posterior median clefts of the palate). Clinical examination and assessment of speech was supplemented by X-ray-video assessment and the findings showed a good degree of correlation. The speech results between Group II and III and between the sexes are compared. Finally the extent of bone formation in the alveolar cleft was studied radiologically in 61 children with Group II clefts. Bone formation was present in 43 children, in 38 of whom a tooth had migrated into bone.     

General body growth in children with clefts of the lip, palate, and craniofacial structure

This paper discusses general body growth in children with craniofacial clefts. Body growth is important in such patients because morphology reflects the cumulation of metabolism over time. The same hormones that direct general body growth also govern the ontogeny of the head and face. Body growth varies in children with different types of clefts. We found no average differences from US norms for those with isolated clefts of the lip alone or those with bilateral clefts of the lip and palate. Children with unilateral clefts of the lip and palate and with isolated cleft palate were significantly shorter than their unaffected peers. Males with these defects were also thinner than normal based on average standard deviation scores for body mass indices. Both unilateral and bilateral clefts of the lip and palate predominated in males, while isolated cleft lip was more frequent in females. Our results indicate that congenital metabolic variation contributes to the development of orofacial clefting and influences postnatal development in certain types of cleft. Accordingly, cleft type is important to growth prognosis, and growth status is relevant to optimization of therapy in orofacial cleft patients.     

Social adjustment in French adults from who had undergone standardised treatment of complete unilateral cleft lip and palate

France has a population of about 60 million peoples and each ten years data about the standard of living are collected by the central bureau of statistics, we considered the collection of data on 5000 households in 1999 in our geographical area would afford a unique opportunity to compare the equivalent status of French adults with repaired cleft of the lip and palate (CLP). Aspects of social adjustment were investigated in a sample of 82 French adults 18-35 years old with repaired complete unilateral cleft of the lip and palate (CLP). All subjects received a standardized regimen of care from the Burgundy cleft palate team of Dijon. The investigation, based on response to a questionnaire, partly replicated a national survey of social and economic life in the population (Standard of living survey Burgundy, INSEE France 1999), so that adults with complete clefts could be compared with a large control sample of the same age. The control group was constituted by subjects between 18 and 35 years in the standard of living survey Burgundy 1999, INSEE France, they were taken from a regional probability sample of households. This report covers education, employment, and marriage. The significant difference between groups was assessed by: Student's t-test or analysis of variance for continuous variables and chi2 test for categorical variables. The results demonstrated that there are significant differences in educational attainment and employment between adults with cleft of the lip and palate and other people. Fewer with cleft of the lip and palate marry, and when they marry they do so later in life, scholarship history showed significant delay in the cleft of the lip and palate group, independence regarding housing was lower in the cleft of the lip and palate group. If cleft of the lip and palate adults functioned within normal limits with regard to employment. However, levels of income were substantively lower than control groups. It would appear that cleft subjects experience some limitation in their ability to secure vocational and economic rewards from society. As a conclusion we can say regarding our results that the cleft of the lip and palate group, even with the smallest degree of malformation (unilateral without associated malformation), showed a significant delay in the independence process.     

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.     

Foetal surgery and cleft lip and palate: current status and new perspectives.

Now-a-days, high-resolution ultrasound allows an accurate and relatively early diagnosis of congenital malformations. In a limited number of such conditions foetal surgery may be lifesaving. However, premature labour has been the major drawback for open foetal surgery. Recently, improvement of video-endoscopic technology has boosted the development of operative techniques for feto-endoscopic surgery, which has been demonstrated to be less invasive than the open approach. Main clinical application of fetoscopic procedures today is the treatment of feto-foetal transfusion syndrome. Although still in development, feto-endoscopic surgery seems to offer new hope for surgical foetal therapy not only in cases of life threatening conditions. Experimental intrauterine correction of cleft lip and palate (CLP) has been lately performed using the feto-endoscopic approach. This procedure offers two major advantages: first, scarless foetal wound healing and bone healing without callus formation, which would also allow a better/normal maxillary growth, and second, significant decrease of foetal and maternal morbidity. Herein, we report the current status of experimental and clinical foetal surgery and propose possible directions for continuing research to make intrauterine procedures safer. Furthermore, we discuss current knowledge and new perspectives of experimental foetal cleft lip and palate repair, which in the future may lead to such excellent results in the operative treatment of clefts, that less or no secondary corrections and therapies, such as orthodontic, dental, logopedic, etc. would be needed. Only if these conditions can be fulfilled, will we be able to improve substantially our therapy for the human foetus with a cleft lip and palate. In spite of all efforts, however, it must be considered that it may not ever be possible to find the optimal treatment method for this or other craniofacial malformations.     

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.     

Current status of presurgical infant orthopaedic treatment for cleft lip and palate patients: A critical review

Rehabilitation of cleft lip and palate (CLP) patients is a challenge for all the concerned members of the cleft team, and various treatment modalities have been attempted to obtain aesthetic results. Presurgical infant orthopaedics (PSIO) was introduced to reshape alveolar and nasal segments prior to surgical repair of cleft lip. However, literature reports lot of controversy regarding the use of PSIO in patients with CLP. Evaluation of long-term results of PSIO can provide scientific evidence on the efficacy and usefulness of PSIO in CLP patients. The aim was to assess the scientific evidence on the efficiency of PSIO appliances in patients with CLP and to critically analyse the current status of PSIO. A PubMed search was performed using the terms PSIO, presurgical nasoalveolar moulding and its long-term results and related articles were selected for the review. The documented studies report no beneficial effect of PSIO on maxillary arch dimensions, facial aesthetics and in the subsequent development of dentition and occlusion in CLP patients. Nasal moulding seems to be more beneficial and effective in unilateral cleft lip and palate patients with better long-term results.KEY WORDS: Bilateral cleft lip and palate, long-term results, nasoalveolar moulding, presurgical infant orthopaedics, unilateral cleft lip and palate     

Prenatal compared with postnatal cleft diagnosis: What do the parents think?

Abstract Since the 1980s, prenatal detection of cleft lip with or without cleft palate (CLP) is possible with a transabdominal ultrasound. The improvement of prenatal detection has resulted in an increased demand for prenatal information and counselling. The aim of this study was to, by means of a questionnaire, evaluate how families, who received the diagnosis prenatally and postnatally, respectively, think about the prenatal and postnatal consultation. Patients referred prenatally or postnatally to the department, due to CLP, between January 2006 and September 2010 were included in the study. Thirty-six of the families (95%) answered the questionnaire concerning prenatal counselling. A total of 46 parents (82%) answered the questionnaire concerning postnatal counselling. In the prenatal group, half of the patients were first informed by the ultrasound unit, whereas 16% were initially informed by the cleft team. More of the families that were informed by the cleft team were content with the information received than the families who were informed by the ultrasound unit. The majority of the families were satisfied with the structure of the consultation. Most of the patients in the postnatal group were happy with the postnatal consultation. Most of the parents in the postnatal group would have wanted to know about the diagnosis prenatally. The great majority of the parents was happy with the consultation with the cleft team and thought that they received enough information. However, the information received at the referring units could be improved. Parents seem to want information on the cleft diagnosis and its treatment as soon as possible.     

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.