Craniofacial cephalometric morphology in 6-year-old children with isolated cleft lip,isolated submucous cleft palate,and combined cleft lip and submucous cleft palate

One hundred and twenty-one cleft children (67 with isolated cleft lip (CL), 32 with isolated submucous cleft palate (SMCP), and 22 with combined cleft lip and submucous cleft palate (CL + SMCP)) were compared retrospectively from lateral cephalograms taken at a mean age of 6.2 years (range 5.5–7.9). None of the children had had their palates repaired or been operated on to treat velopharyngeal insufficiency (VPI). The children with CL + SMCP and CL had similar morphology. They had greater maxillary length, greater maxillary and mandibular prominence, less vertical growth pattern, less deep nasopharyngeal airways, and thinner upper lips than those with isolated SMCP. After 6 years of age one patient with CL + SMCP, none with CL, and 16 with SMCP needed operations for VPI. This small series suggests that children with CL + SMCP and SMCP have different morphology. Although CL + SMCP is a combination of two types of clefts, it seems to be associated with similar morphology to CL.     

Craniofacial cephalometric morphology in 6-year-old children with isolated cleft lip, isolated submucous cleft palate, and combined cleft lip and submucous cleft palate.

One hundred and twenty-one cleft children (67 with isolated cleft lip (CL), 32 with isolated submucous cleft palate (SMCP), and 22 with combined cleft lip and submucous cleft palate (CL + SMCP)) were compared retrospectively from lateral cephalograms taken at a mean age of 6.2 years (range 5.5-7.9). None of the children had had their palates repaired or been operated on to treat velopharyngeal insufficiency (VPI). The children with CL + SMCP and CL had similar morphology. They had greater maxillary length, greater maxillary and mandibular prominence, less vertical growth pattern, less deep nasopharyngeal airways, and thinner upper lips than those with isolated SMCP. After 6 years of age one patient with CL + SMCP, none with CL, and 16 with SMCP needed operations for VPI. This small series suggests that children with CL + SMCP and SMCP have different morphology. Although CL + SMCP is a combination of two types of clefts, it seems to be associated with similar morphology to CL.     

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     

Cleft lip and palate patients prior to delayed closure of the hard palate: evaluation of maxillary morphology and the effect of early stimulation on pre-school speech

Speech and maxillary development were analysed in two groups of patients with unilateral cleft lip and palate; both groups had early jaw orthopaedic treatment and a surgical regimen that included two-stage lip surgery (mean ages of 2 and 19 months) and soft palate repair (8 months). Closure of the hard palate was postponed until the children were 8 to 10 years of age. The first group comprised 10 consecutive patients who were analysed at 5 and 7 years of age, and the second group seven patients who were studied at the age of 5. Both groups were thus investigated before the repair of the cleft in the hard palate. In addition to surgical and jaw orthopaedic treatment, the second group of patients received early stimulation of lip and tongue tip movements. Our results indicated that hypernasality was less a problem than was retracted palatal or velar articulation of dental consonants. These deviations tended to be reduced, however, after early stimulation. There seemed to be no clear association between the size of the residual cleft in the hard palate and the extent of speech development. The average size of the residual cleft in our patients was comparatively small, and decreased further during follow up. We conclude that preschool children with unilateral cleft lip and palate may develop good speech, in spite of the residual cleft, if they use an intraoral plate and are given extra lip and tongue tip stimulation, together with early speech therapy if necessary.     

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).     

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.     

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.     

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.     

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.     

Unrepaired clefts in the hard palate: speech deficits at the ages of 5 and 7 years and their relationship to size of the cleft.

The purpose of the study was to investigate the relationship between the size of oronasal openings in the hard palate and speech deficits. Audiotape recordings and plaster casts were taken according to standard procedures at 5 and 7 years of age from 22 consecutive children born with complete unilateral cleft lip and palate treated at Sahlgrenska University Hospital, G?teborg, Sweden. The soft palate had been repaired before the age of 12 months, whereas the cleft in the hard palate was left unrepaired, to be closed later. Perceptual judgements of nine speech variables at 5 and 7 years of age were correlated with measures of the area of the residual cleft in the hard palate. "Retracted oral articulation" (to velar place) found in nine of the 22 children correlated significantly with the area of the cleft at the age of 5 years but not later. The establishment of this particular speech error seems to be related to the size of an oronasal opening.     

Psychological issues in cleft lip and palate

The treatment of cleft lip and palate extends over the infancy, childhood, and adolescence of individuals who are born with this condition. The children and their families contend with multiple surgeries throughout these years. Depending on the severity of the cleft, the families may also need to cope with speech therapy, ear infections, learning disabilities,and various orthodontic treatments. The end result of these treatments and interventions should be a child, teen, or adult who is an appropriately contributing member of society at each stage of development. The psychological support needed to enable a child to meet that goal should be provided by family, school, the surgeon, and other members of the cleft treatment team. These adults should demonstrate a belief in the child's ability to cope with the challenges of cleft lip and palate treatment and should focus the child on the efficacy he will gain from having had the experience of growing up with a cleft lip and palate.     

The effect of form and dimension on the management of the maxillary arch in unilateral cleft lip and palate conditions

Over the past 28 years, a series of studies at the West Midlands Regional Plastic Unit has compared the maxillary arches at birth and 4 months of 30 normal children and two groups of children with unilateral cleft lip and palate, one of which had presurgical maxillary orthopaedic treatment and the other which did not. Other studies have compared the area of palatal mucosa at birth with the overall size of the arch at 5 years of age and have also assessed the long term effect of simultaneous lip and palate repair and presurgical treatment on the profile, occlusion and speech of the older patient. The paper summarises the findings of the investigations and considers how they have contributed to an understanding of the unilateral cleft lip and palate problem particularly with regard to the long term management of such cases and the provision of a suitable treatment protocol.     

Treatment of cleft lip and palate: long-term results]

Since 1958, 900 children with cleft lip and palate were operated on at the University Hospital Brugmann in Brussels. Until 1981, the children were operated at 6 months for the lip and at 18 months for the palate with two flap palatoplasties. Since 1982, R. Malek's technique and sequence were adopted. The results after 5 years follow-up are analyzed between two groups of 50 and 49 children operated with the two techniques. Growth of the maxillary arch and phonation are well improved in the second group, but no improvement was observed in terms of hearing.     

用医学美学的理念指导唇腭裂患儿的临床护理

目的:总结以医学美学的理念指导先天性唇腭裂患儿临床护理的经验。方法:本组患儿309例,男169例,女140例;年龄:1月~18岁,体重1.5~66Kg;单纯唇裂79例,唇裂伴腭裂181例,单纯腭裂49例。所有患者均行手术整复。主要的护理内容有:从医学美学的角度进行唇腭裂相关知识的宣教;帮助患儿及父母的心理达到平衡状态;以医学美学的理念指导常规护理操作和舒适护理;美化病房环境;出院的美学指导。结果:309例唇腭裂患儿的护理均取得了较满意的效果,唇裂患儿术后容貌明显美观;仅8例腭裂患儿发生腭瘘或复裂,其余患儿创口愈合良好,悬雍垂成形,语音明显改善。结论:以医学美学的理念指导临床护理,有助于提高唇腭裂患儿的医护质量和患者满意度,并有利于提高护理人员的美学修养。     

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.     

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.     

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.     

Maxillary dental arch and occlusion in patients with unilateral cleft lip and palate treated with different delays in closure of the hard palate after early velar repair.

We wanted to find out if growth of the maxilla in 26 patients with unilateral cleft lip and palate (UCLP) was adversely affected by having the residual cleft of the hard palate repaired earlier than had been done previously in a 2-stage palatal closure protocol. The ages at repair of the hard palate of the present patients ranged from 38 to 89 months. Dental casts from ages about 3 years (before any repair of the hard palate), 5, 7, and 10 years of age were analysed. The results indicated that earlier repair of the cleft in the hard palate did not influence maxillary growth differently from the later repair.     

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.     

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.