Facial growth in patients with isolated cleft palate treated with minimal incision technique compared to a normal population-a cephalometric study at 10 and 16 years of age.

OBJECTIVE: To evaluate the result of a novel palatoplasty (minimal incision technique) for closure of an isolated cleft palate (ICP) at 10 and 16 years of age, concerning facial growth, compared to a normal population. SUBJECTS: Treated group: 55 non-syndromic Caucasian children born with an ICP between 1987 and 2001. The children were treated surgically with a one-stage palatoplasty at a mean age of 13 months. Control group: 110 Caucasian children with normal occlusion with no history of clefts or orthodontic intervention. METHOD: The treated children (25 boys, 30 girls) were matched (gender and age) with the children in the control group. A retrospective evaluation at 10 (mean 10.5) and 16 (mean 16.0) years of age was performed by analysis of lateral cephalograms. Fifteen variables were evaluated. Ninety-nine per cent confidence intervals were calculated. Two-way factorial ANOVA and mixed-model analysis were performed. RESULTS: Treated patients compared to the control group showed: at 10 years of age, smaller mandible (P = 0.001) and reduced posterior upper and total face heights (P ≤ 0.001); at 10 and 16 years of age, a retrognathic (P ≤ 0.001), smaller (P ≤ 0.006) and with an increased posterior inclination of the maxilla (P < 0.001), as well as a retrognathic mandible (P ≤ 0.006). CONCLUSION: The craniofacial morphology at 10 and 16 years of age in patients born with an ICP and treated with the minimal incision technique differs compared to the morphology of a normal control group born without a cleft. Retrognathic maxillas and a smaller and posteriorly inclined maxilla were found in the treated group.

Mean GOSLON Yardstick Scores After 3 Different Treatment Protocols-A Long-term Study of Patients With Unilateral Cleft Lip and Palate.

OBJECTIVES: (1) To evaluate dental arch relationships, with the Great Ormond Street, London and Oslo (GOSLON) Yardstick, of participants with Unilateral cleft lip and palate (UCLP) and treated with 1-stage palatal closure with 3 different surgical protocols (2) to compare the mean GOSLON ratings with other CLP centers. DESIGN: Retrospective study of medical charts and dental models. SETTING: Karolinska University Hospital, Stockholm, Sweden. PARTICIPANTS: Eighty-seven patients with UCLP operated with 1-stage palatal repair. Thirty-five were operated with Veau-Wardill-Kilner (VWK) technique 1975 to 1986, 31 with minimal incision technique (MIT) from 1987 to 1997, and 21 according to MIT with muscle reconstruction (MITmr) 1998 to 2004. INTERVENTIONS: Dental casts at ages 5 (n = 87), 7 to 8 (n = 27), 10 (n = 81), 16 (n = 61), and 19 (n = 35) years were rated by 10 assessors with the GOSLON Yardstick. Information of other interventions was retrieved from patients' charts. MAIN OUTCOME MEASURES: Mean GOSLON ratings. RESULTS: A total of 82% of the participants were rated as having excellent to satisfactory outcome. Weighted κ statistics for the 10 assessors was good for inter-rater agreement and good/very good for intra-rater agreement. CONCLUSIONS: The mean GOSLON score in the Stockholm overall material at age 10 was 2.67. The VWK technique resulted in a greater need of orthognathic surgery than the MIT ( P < .01). The MITmr did not produce better dental arch relationships than MIT at age 5 ( P < .05). The best dental arch relationships were found in the MIT group at 10 years, mean 2.58, which is not significantly different from other centers with excellent outcome except Gothenburg and Vienna.

Minimal incision palatoplasty with or without muscle reconstruction in patients with isolated cleft palate-a cephalometric study at 5 and 10 years.

OBJECTIVE: To compare differences in facial growth in patients with isolated clefts of the hard and/or soft palate treated with the minimal incision technique without (MI) or with muscle reconstruction (MMI). SUBJECTS AND METHOD: A consecutive series of 170 Caucasian children born with isolated cleft palate were studied. Individuals with other craniofacial malformations, apart from Pierre Robin sequence (PRS), were excluded. The patients were treated surgically with MI (n = 85) or MMI (n = 85) palatoplasty (mean age: 13 months) and divided further into two subgroups: clefts within the soft palate only (small cleft, n = 51) and within the hard and soft palate (big cleft, n = 119). A retrospective evaluation at 5 (mean 5.4) and 10 (mean 10.3) years was performed using lateral cephalograms. Twelve skeletal and one soft tissue measurement was evaluated. Both 95% and 99% confidence intervals were calculated, two-way ANOVA and mixed model analysis was performed including/excluding PRS. RESULTS: At 5 years, statistically significant increased inclination of the palatal plane in the big MMI cleft group (P < 0.01), increased posterior upper face height (P < 0.01), and longer mandibular length (P < 0.001) in the small MI cleft group was observed. At 10 years, statistically significant increased inclination of the palatal plane (P < 0.001), decreased posterior upper face height (P < 0.001), and longer palatal length (P < 0.01) was seen in the big MMI group. LIMITATIONS: Retrospective single centre study, limited sample size, three surgeons. CONCLUSION: Minor differences in craniofacial morphology were found between patients with isolated clefts treated with MI or MMI technique and between small and big cleft lengths.

Facial Growth at 5 and 10 Years After Veau-Wardill-Kilner Versus Minimal-Incision Technique Repair of Isolated Cleft Palate.

OBJECTIVE: To compare the Veau-Wardill-Kilner technique with the Minimal-Incision technique repair of isolated clefts of the hard and soft palate regarding facial growth at 5 and 10 years of age. DESIGN: Retrospective study of lateral cephalograms. SETTING: Hospital and university based. PATIENTS: A consecutive series of 145 Caucasian nonsyndromic children born with isolated cleft palate between 1980 and 1996 were studied. Children with Pierre Robin sequence (PRS) were included in the study. The patients were divided into 4 groups: 2 groups regarding surgical technique and 2 groups regarding cleft length. INTERVENTION: Veau-Wardill-Kilner or Minimal-Incision palatoplasty. MAIN OUTCOME MEASURES: Eleven skeletal and 1 soft tissue measurement were evaluated from lateral cephalograms taken at 5 and 10 years of age. RESULTS: Only minor differences in cephalometric morphology were found between the Veau-Wardill-Kilner technique group and the Minimal-Incision technique group. Similar results were found independent of cleft length or the inclusion of PRS in the sample. CONCLUSION: The craniofacial cephalometric morphology at 5 and 10 years of age in patients with isolated cleft palate is similar between the Veau-Wardill-Kilner and the Minimal-Incision technique group.

Minimal incision palatoplasty with or without muscle reconstruction in patients with isolated cleft palate: a cast and medical records analysis.

Objectives: To compare the minimal incision (MI) technique with the minimal incision including muscle reconstruction (MMI) technique regarding surgical complications and dentoalveolar status at 5 years of age. Subjects and method: A consecutive series of 202 Caucasian non-syndromic children (apart from Pierre Robin Sequence) born with isolated cleft palate between 1987 and 2007 and treated with MI (n = 78) or MMI (n = 102) palatoplasty at a mean age of 12.7 (SD = 1.43) months in Stockholm. Twenty-two patients did not fulfill the inclusion criteria. The patients were divided into two subgroups: clefts within the soft palate only (small cleft, n = 50) and clefts within the hard and soft palate (big cleft, n = 130). Dental relations, structure of the palatal mucosa, and height of the palatal vault at 5 (mean age 5.3, range: 4.4-6.9) years of age were studied using plaster models. Time for surgery, blood loss, complications in the immediate postoperative period, frequency of fistulas, and additional pharyngeal flap surgery were evaluated. Student's t-test, chi-square test and 95 per cent confidence intervals were calculated. Results: MMI compared to MI technique result in statistically significant increased operation time, less need for pharyngeal flap surgery, and to shallower palatal vault. Big clefts result in statistically significant increased operation time and need for pharyngeal flap surgery. Dental relations were the same in all groups. Limitations: Retrospective single centre study, limited sample size, more than one surgeon. Conclusions: The muscle reconstruction results in a reduced subsequent need for pharyngeal flap surgery, but to shallower palatal vault and demand for almost double operation time. The dental relations were the same in all groups.

The influence of the initial width of the cleft in patients with unilateral cleft lip and palate related to final treatment outcome in the maxilla at 17 years of age.

The aim of this study was to assess whether the initial cleft width in patients born with complete unilateral cleft lip and palate (UCLP), is correlated to final treatment outcome regarding maxillary growth. This report is a retrospective longitudinal cohort study of 45 consecutive non-syndromic individuals with UCLP, 19 from the Stockholm Cleft Team, and 26 from the Oslo Cleft Team. The treatment protocols in the two teams differed. The initial transversal width in infants was measured at three levels on study casts. The width was correlated to variables obtained from lateral cephalograms at 17 years of age, from rating of dental arch relationship and to treatment variables obtained from the medical records: existence of Simonart's band, missing maxillary teeth, duration of orthopaedic/orthodontic treatment, and the need for orthognathic surgery. The initial width of the middle part of the cleft and final maxillary inclination (NSL/NL) showed a weak correlation (P < 0.05); the wider the cleft, the less the inclination. No further correlations were found between the initial width and final outcome measurements studied. In the Oslo group where Simonart's band was present, the anterior and middle widths of the cleft were significantly smaller (P < 0.001). Additionally, the existence of Simonart's band had a significant effect on final maxillary inclination (P < 0.05), i.e. the maxillary inclination increased. Treatment outcome seems mainly to depend on the treatment protocol performed rather than the severity of the cleft. The width of the middle part of cleft may be associated with the final maxillary inclination.

Speech results after one-stage palatoplasty with or without muscle reconstruction for isolated cleft palate.

OBJECTIVE: To investigate speech outcome between children with isolated cleft palate undergoing palatoplasty with and without muscle reconstruction and to compare speech outcomes between cleft and non cleft children. The number of subsequent velopharyngeal flaps was compared with respect to surgical techniques and cleft extent. DESIGN: Cross-sectional retrospective study. PARTICIPANTS: One hundred four children aged 4 years, 0 months to 6 years, 0 months, 33 with isolated cleft of the soft palate, 53 with isolated cleft of the hard and soft palate, and 18 non cleft children. INTERVENTIONS: Two primary palate repair techniques: minimal incision technique (MIT) and minimal incision technique including muscle reconstruction (MIT(mr)). MAIN OUTCOME MEASURES: Perceptual judgment of seven speech parameters assessed on a five-point scale. RESULTS: No significant differences in speech outcomes were found between MIT and MIT(mr) surgery groups. The number of velopharyngeal flaps was significantly lower after MIT(mr) surgery compared to MIT surgery. The number of flaps was also significantly lower in children with cleft of the soft palate compared to children with cleft of the hard and soft palate. Children with cleft of the soft palate had significantly less glottal articulation and weak pressure consonants compared to children with cleft of the hard and soft palate. CONCLUSIONS: The MIT(mr) surgery technique was not significantly superior to the MIT technique regarding speech outcomes related to velopharyngeal competence, but had fewer velopharyngeal flaps, which is contradictory. Until a larger sample can be studied, we will continue to use MIT(mr) for primary palate repair.

Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 2. Surgical results.

BACKGROUND: Longstanding uncertainty surrounds the selection of surgical protocols for unilateral cleft lip and palate, and randomised trials have only rarely been performed. The Scandcleft Project consists of three trials commenced in 1997 involving ten centres in Denmark, Finland, Norway, Sweden, and the UK. Three groups of centres tested a newly-defined common technique for palatal repair (Arm A) against their local protocols (Arms B, C, D). Arm A was familiar to most of the surgeons in Trial 1, but not to the surgeons in the other Trials. AIM: To evaluate surgical events and complications of the 448 (293 boys, 155 girls) patients with complete unilateral cleft lip and palate (UCLP) enrolled in the three trials. METHOD: The three trials were carried out in parallel in adherence with a fully developed, ethically approved protocol. Operative time, bleeding, complications, and major dehiscence during and after both primary surgeries were recorded by the surgeon. Rates of fistula and surgery for velopharyngeal incompetence (VPI) were assessed until the youngest patient of the study had reached the age of 9 years. Pearson Chi-square statistical analysis was used to compare the outcomes. RESULTS: No significant differences in bleeding, infection, anaesthetic complications or length of hospital stay between the different arms were found for Trial 1. However, in Trials 2 and 3 there were more airway problems in Arm A than with the traditional local protocols (Arms C or D). In Trial 3 fistula and VPI surgery rates were also higher in Arm A. CONCLUSIONS: The results do not provide statistical evidence that any technique is better than others, but indicate that surgery was more problematic for surgeons who were still gaining experience with an unfamiliar surgical protocol. TRIAL REGISTRATION: ISRCTN29932826.

A Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 1. Planning and management.

BACKGROUND AND AIMS: Longstanding uncertainty surrounds the selection of surgical protocols for the closure of unilateral cleft lip and palate, and randomised trials have only rarely been performed. This paper is an introduction to three randomised trials of primary surgery for children born with complete unilateral cleft lip and palate (UCLP). It presents the protocol developed for the trials in CONSORT format, and describes the management structure that was developed to achieve the long-term engagement and commitment required to complete the project. METHOD: Ten established national or regional cleft centres participated. Lip and soft palate closure at 3-4 months, and hard palate closure at 12 months served as a common method in each trial. Trial 1 compared this with hard palate closure at 36 months. Trial 2 compared it with lip closure at 3-4 months and hard and soft palate closure at 12 months. Trial 3 compared it with lip and hard palate closure at 3-4 months and soft palate closure at 12 months. The primary outcomes were speech and dentofacial development, with a series of perioperative and longer-term secondary outcomes. RESULTS: Recruitment of 448 infants took place over a 9-year period, with 99.8% subsequent retention at 5 years. CONCLUSION: The series of reports that follow this introductory paper include comparisons at age 5 of surgical outcomes, speech outcomes, measures of dentofacial development and appearance, and parental satisfaction. The outcomes recorded and the numbers analysed for each outcome and time point are described in the series. TRIAL REGISTRATION: ISRCTN29932826.

Novel and de novo mutations of the IRF6 gene detected in patients with Van der Woude or popliteal pterygium syndrome.

The interferon regulatory factor 6 gene (IRF6) has been identified as the major Van der Woude (VWS) syndrome and popliteal pterygium (PPS) syndrome gene with mutations in the majority of the kindreds. We have studied altogether 17 kindreds from Sweden, Finland, Norway, Thailand and Singapore, and report here 10 mutations, six of them previously unseen. In two kindreds, we could document de novo mutations, both of them changing a codon for a glutamine residue to a stop. No mutation could be detected in the four VWS kindreds from Finland, suggesting a founder effect for a mutation in an atypical noncoding position. Our findings demonstrate that several distinct mutations occur in the Swedish population, and confirm the general notion of a broad spectrum of IRF6 mutations underlying the VWS/PPS phenotypes.

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.

Associated malformations in infants with cleft lip and palate: a prospective, population-based study.

OBJECTIVE: Infants with cleft lip and palate may often have other associated congenital defects although the reported incidence and the types of associated malformations vary between different studies. The purpose of this investigation was to assess the prevalence of associated malformations in a geographically defined population. METHODS: The prevalence of associated malformations in infants with clefts were collected prospectively between 1975 to 1992 on all infants born in greater Stockholm, Sweden. The patient records were also compared with data from the National Malformation Registry and other hospital records if any. RESULTS: Of the 616 cleft infants (367 boys, 249 girls) born during this period, 21% had associated malformations that either required follow-up or treatment. Associated malformations were more frequent in infants who had both cleft lip and palate (28%) than in infants with isolated cleft palate (22%) or infants with isolated cleft lip (8%). Malformations of the upper or lower limbs or the vertebral column were the most common other anomalies and accounted for 33% of all associated defects. Twenty-four percent of associated malformations were in the cardiovascular system and congenital heart disease was the most common isolated associated malformation. Fifteen percent of all associated malformations were multiple and they were frequently associated with mental retardation or chromosomal anomalies. Twenty-two percent of infants with associated malformations were born preterm, compared with an expected 5% incidence of preterm delivery in Sweden. CONCLUSION: A more extensive cleft seems to be associated with a higher risk for associated malformations. Although many associated congenital defects can be detected at a physical examination, the high prevalence of congenital heart disease (16 times that of general population) may justify a routine echocardiographic screening.

Dental occlusion after Veau-Wardill-Kilner versus minimal incision technique repair of isolated clefts of the hard and soft palate.

OBJECTIVE: To compare the Veau-Wardill-Kilner technique with a technique similar to the minimal incision technique described by Mendosa et al. on the basis of surgical complications and dentoalveolar status in the deciduous dentition. DESIGN: Retrospective study of medical and dental records and casts. PATIENTS: A consecutive series of 129 Caucasian children born with isolated cleft palate between 1980 and 1992. MAIN OUTCOME MEASURES: From medical records, the variables of time for surgery, blood loss, complications in the immediate postoperative period, and frequency of fistulas were evaluated. On dental casts, the variables of sagittal, transversal, and vertical relations; structure of the palatal mucosa; and height of the palatal vault were studied. RESULTS: Time for surgery was shorter in the extensive clefts repaired with a Veau-Wardill-Kilner technique. Blood loss was higher using the Veau-Wardill-Kilner technique. The width of the upper jaw was significantly narrower in the Veau-Wardill-Kilner group, compared with the minimal incision group. Scar tissue and pits of the palate were more frequently found in the Veau-Wardill-Kilner group. CONCLUSIONS: The minimal incision technique in this study has been shown to result in better development of the upper jaw with a better dental occlusion and palatal mucosa with significantly less scar tissue.