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.

Long-term, longitudinal follow-up of individuals with unilateral cleft lip and palate after the Gothenburg primary early veloplasty and delayed hard palate closure protocol: speech outcome.

OBJECTIVE: To investigate long-term, longitudinal speech outcome in patients born with unilateral cleft lip and palate treated according to a two-stage primary palatal protocol with early veloplasty and delayed hard palate closure. DESIGN: Retrospective, longitudinal cohort study. Setting : A university hospital in western Sweden. SUBJECTS: A consecutive series of 55 patients from the total cohort of 65 were included. All patients had surgical procedures at Sahlgrenska University Hospital, Gothenburg, Sweden. METHODS: Standardized audio recordings were blindly analyzed at 5, 7, 16, and 19 years of age and after at a clinical visit at 10 years of age. Typical cleft speech variables were rated independently on ordinal scales. Intelligibility and perceived velopharyngeal function were assessed also. Prevalences of speech characteristics were determined, and interrater and intrarater agreement were calculated. Results : Prominent hypernasality, nasal air leakage, and retracted oral articulation at 5 years were markedly reduced throughout the years with low prevalences at ages 16 and 19 years. Perceived velopharyngeal competence was noted in 82% at age 16 and 87% at age 19 years along with normal intelligibility. Pharyngeal flap surgery was performed in 6 of the 55 patients (11%). CONCLUSIONS: Long-term speech outcome in patients with two-stage palatoplasty with early soft palate repair was considered good and improved even before hard palate repair. The typical retracted oral articulation was quite frequent during the early ages; whereas, nonoral misarticulations were almost nonexistent, implying good velopharyngeal competence.

Long-term, longitudinal follow-up of individuals with UCLP after the Gothenburg primary early veloplasty and delayed hard palate closure protocol: maxillofacial growth outcome.

OBJECTIVE: To study long-term maxillofacial development in a sample of patients born with unilateral cleft lip and palate. The patients' palatal repair had been performed according to a two-stage protocol with early velar closure and delayed hard palate surgery. DESIGN: Retrospective, longitudinal cohort study. SUBJECTS: The sample consisted of 50 consecutive patients with unilateral cleft lip and palate born from 1980 to 1989. All of them had been operated on at the Sahlgrenska University Hospital in Gothenburg, Sweden. METHODS: Certain maxillary casts as well as lateral roentgencephalograms, obtained at 5, 10, 16, and 19 years of age, were analyzed. Results : Our patients' maxillofacial growth was very good even up to the final examination in early adulthood. We ascribe the advantageous midfacial morphology of our patients particularly to limited growth restriction from palatal scars due to the surgical protocol used in our cleft center. CONCLUSION: The two-stage protocol we advocate for repair of the palatal cleft resulted in very satisfactory growth outcome. A palatal scar, which might impair maxillary development, was created only by the velar surgery. If this scar is located close to the posterior border of the hard palate, it might result in less maxillary growth restriction than if it is positioned further anteriorly.

Maxillary growth controversies after two-stage palatal repair with delayed hard palate closure in unilateral cleft lip and palate patients: perspectives from literature and personal experience.

OBJECTIVE: To analyze published papers dealing with delayed hard palate repair within a two-stage palatal surgery protocol in treatment of cleft lip and palate. Timing of the procedures, methods used, as well as growth results were considered. METHOD: By utilizing this information in relation to knowledge about normal maxillary development, efforts were made to explain differences in growth outcome between different investigations. Particularly, follow-up reports of unilateral cleft lip and palate patients with records up to at least 10 years of age were studied. RESULTS: Most papers reported an excellent or very good maxillary growth outcome after their delayed hard palate closure protocols. Where unsatisfactory results were published, reasonable explanations were found accounting for why the method had failed the expectation of good maxillary growth. CONCLUSION: Based on the published reports and the experience from a cleft team where the studied protocol has been practiced since 1975, recommendation for method as well as timing for the two-stage protocol is laid out in some detail.

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.

Speech development in patients with unilateral cleft lip and palate treated with different delays in closure of the hard palate after early velar repair: a longitudinal perspective.

We wanted to find out if different timing of delayed repair of the hard palate in a two-stage procedure had an impact on the speech of 26 patients with unilateral cleft lip and palate (UCLP). The soft palate was closed at the age of 7 months and the hard palate between 38 and 89 months of age. Speech audio recordings at the age of 3 years (baseline, before any repair of the hard palate) and at the ages of 5, 7, and 10 years (the latter obtained at least one year after closure) were analysed. We used standardised speech assessments at routine follow-up and assessment by one external listener. The prevalence of speech errors caused by the cleft was similar to those described in previous reports from our centre in which hard palate repair was delayed. Unexpectedly, the results showed no difference in speech production related to timing of hard palate repair, except for nasal air leakage at the age of 7 years.

Analysis of dental arch relationships in Swedish unilateral cleft lip and palate subjects: 20-year longitudinal consecutive series treated with delayed hard palate closure.

OBJECTIVE: To evaluate the dental arch relationships for a consecutive series from Goteborg, Sweden, who had delayed hard palate closure. DESIGN: Retrospective study. SETTING: Sahlgrenska University Hospital, Goteborg, Sweden. PATIENTS: The dental study models of 104 consecutive unilateral cleft lip and palate subjects. The study cohort was born between 1979 and 1994. Longitudinal records were available at ages 5 (n = 94), 10 (n = 97), 16 (n = 59), and 19 years (n = 46). Five assessors rated models according to the GOSLON Yardstick on two separate occasions each. INTERVENTIONS: These patients had been operated upon according to the Goteborg protocol of delayed hard palate closure (at age 8 years). RESULTS: 85% of subjects were rated in groups 1 and 2 (excellent or very good outcome), 12% were rated in group 3 (satisfactory), and 3% were assigned to group 4 (poor). No patients presented in Group 5 (very poor). Weighted kappa statistics for double determination of Yardstick allocation for five assessors demonstrated values between .65 and .90 for interrater agreement (good/very good) and between .70 and .90 for intrarater agreement (very good). CONCLUSIONS: Delayed hard palate closure as practiced in Goteborg since 1979 has produced the best GOSLON Yardstick ratings in a consecutive series of patients ever recorded worldwide, since the Yardstick was first used in 1983. However, it is noteworthy that a new protocol has been introduced in Goteborg since 1994, in which hard palate closure is done at 3 years due to concerns regarding speech.

Sagittal synostosis: I. Preoperative morphology of the skull.

The aim of this study was to characterise the preoperative morphology of the skull in sagittal synostosis in an objective and quantified way. The shapes of the skulls of 105 patients with isolated premature synostosis of the sagittal suture (SS group) were studied and compared with those of a control group of 72 children with unilateral incomplete cleft lip (UICL). A standardised radiocephalometric technique was used to obtain the images. A modification of a method developed by Kreiborg was used to analyse the radiocephalograms, which included the digitisation of 88 landmarks in the calvaria, skull base, and orbit (42 in the lateral and 46 in the frontal projections), the production of plots of mean shape for each group, and the intergroup comparison of a series of 81 variables (linear distance between selected landmarks, and angles defined by groups of three landmarks). Data from a subgroup of 66 patients aged 5 to 8 months were further compared to age-matched normative data in terms of seven angular and linear calvarial, cranial base and orbital variables. In a comparative analysis of the mean lateral plots, the foreheads of the study group (SS) had a more pronounced anterior slope and were also more convex. The vertex area was located more anteriorly, and was less convex. The occipital curvature was more prominent. Analysis of the mean frontal plots revealed a lack in convexity and lateral projection of the upper parietal regions, as well as a lower location of the line of maximum skull width. Comparison of the mean values of an SS subgroup to age-matched normative data showed a longer (p<0.001) and narrower skull (p<0.001) and a greater interorbital distance (p<0.001). The cranial base angle, the sella to nasion, and sella to basion lengths did not differ significantly. Sagittal synostosis is characterised by an extensive deformity of the cranial vault, with an essentially normal cranial base. The widened interorbital distance is probably related to compensatory metopic hyperactivity.

Sagittal synostosis: II. Cranial morphology and growth after the modified pi-plasty.

The aim of this study was to characterise the postoperative cranial growth and morphology after a modified pi-plasty for sagittal synostosis. The shape of the skull of 82 patients with isolated premature synostosis of the sagittal suture (SS group) operated on with a modified pi-plasty was studied longitudinally. Forty-five children with unilateral incomplete cleft lip (UICL), evaluated longitudinally at the ages of 2.4 and 23.2 months were used as controls. A standardised radiocephalometric technique was used for image acquisition. The radiocephalograms were analysed using a modification of a method developed by Kreiborg, which included the digitisation of 89 landmarks of the calvaria, cranial base, and orbit (43 in the lateral and 46 in the frontal projections), the production of mean shape plots for each group, and the intergroup comparison of a series of 78 variables (linear distance between selected landmarks, and angles defined by groups of three landmarks). Paired and unpaired t tests were used to assess the differences between the variables studied. These were accepted as significant for values of p<0.01 and were presented as coloured segments or areas in the respective plots. In a comparative analysis with the mean UICL lateral plots, the mean preoperative lateral plots of the study group (SS) showed that the anterior slope of the forehead was more pronounced and it was also more convex. The vertex area was located more anteriorly and was less convex. The occipital curvature was more prominent. Comparison of the mean frontal plots showed a deficiency in convexity and lateral projection of the upper parietal regions, and the line of maximum skull width was lower. The postoperative mean lateral plots of the study group showed a correction of the exaggerated anterior inclination of the forehead and a reduction of the abnormal occipital convexity. However, there was little change in the vertex region and it remained flatter than in the control group. In the mean frontal plots, the increase in convexity and in the lateral projection of the upper parietal areas led to a shape that was similar to that of the UICL group. The mean (SD) cephalic index changed from 64.9% (1.8%) to 71.4% (3.5%) (p<0.001). The longitudinal comparison between the mean postoperative plots at 3 and 5 years of age showed that there had been little change in cranial shape. In conclusion, after a modified pi-plasty for sagittal synostosis, significant objective changes in cranial shape towards normality were produced. The postoperative profile cranial shape was improved except in the vertex area, which remained flatter than normal. In the frontal projection an almost normal shape was obtained. The postoperative cranial shape obtained at 3 years of age had remained stable at the age of 5 years.

Timing of cleft palate closure should be based on the ratio of the area of the cleft to that of the palatal segments and not on age alone.

BACKGROUND: Retrospective and prospective serial spatiotemporal investigations were carried out primarily to determine whether the ratio of the size of the posterior cleft space relative to the palatal surface area limited laterally by the alveolar ridges can be used to select the appropriate time for surgical closure of the palatal cleft space. Two subsamples were compared to determine whether the size of the palate and velocity of palatal development in well growing cases differ from those in cases treated by vomer flap surgery. The prospective investigation asked whether presurgical orthopedics increases the rate of palatal growth and palatal size. METHODS: Using the palatal casts of 242 male and female individuals from eight institutions in the United States and Western Europe that followed a variety of treatment protocols, separate serial analyses were conducted of well growing cases with excellent aesthetics, dental occlusion, and speech and a control series of 17 cases of various clefts of the lip and alveolus and/or soft palate but no clefts in the hard palate. Twelve groupings of cases were established depending on their institutional location and type of cleft. RESULTS: Among the various institutions in the study, palatal growth rates and size were statistically similar. Growth in the various clinical series (size, mm2) was less than that of the control series. The ratio of cleft space size to palatal surface area medial to the alveolar ridges was 10 percent or less at 18 months of age in most cases. There was no statistical difference in total surface size between groups, except for one series whose total growth size was least of all. Right and left lateral palatal segments, whether large or small, grew at the same rate. The sample of bilateral cases was too small for statistical comparisons. Presurgical orthopedics did not stimulate palatal growth. The coefficient of variance was less than 10 percent in all measurements. CONCLUSIONS: Delaying all cleft closure surgery until 5 years of age and older is unnecessary to maximize palatal growth. The best time to close the palatal cleft space is when the palatal cleft size is 10 percent or less of the total palatal surface area bounded laterally by the alveolar ridges. The 10 percent ratio generally occurs between 18 and 24 months but can occur earlier or later. There is more than one good type of palatal cleft closure surgery.

Long-term skeletal stability after maxillary advancement with distraction osteogenesis using a rigid external distraction device in cleft maxillary deformities.

Rigid external distraction is a highly effective technique for correction of maxillary hypoplasia in patients with orofacial clefts. The clinical results after correction of sagittal maxillary deformities in both the adult and pediatric age groups have been stable. The purpose of this retrospective longitudinal cephalometric study was to review the long-term stability of the repositioned maxilla in cleft patients who underwent maxillary advancement with rigid external distraction. Between April 1, 1995, and April 1, 1999, 17 consecutive patients with cleft maxillary hypoplasia underwent maxillary advancement using rigid external distraction. There were 13 male patients and four female patients, with ages ranging from 5.2 to 23.6 years (mean, 12.6 years). After a modified complete high Le Fort I osteotomy and a latency period of 3 to 5 days, patients underwent maxillary advancement with rigid external distraction until proper facial convexity and dental overjet and overbite were obtained. After active distraction, a 3- to 4-week period of rigid retention was undertaken; this was followed by removable elastic retention for 6 to 8 weeks using, during sleep time, an orthodontic protraction face mask. Cephalometric radiographs were obtained preoperatively, after distraction, at 1 year after distraction, and 2 or more years after distraction. The mean follow-up was 3.3 years (minimum, 2.1 years; maximum, 5.3 years). The following measurements were obtained in each cephalogram: three linear horizontal and two linear vertical maxillary measurements, two angular craniomaxillary measurements, and one craniomandibular measurement. Differences between the preoperative and postoperative cephalometric values were analyzed by paired t tests (p < 0.05). The cephalometric analysis demonstrated postoperatively significant advancement of the maxilla. In addition, the mandibular plane angle opened 1.2 degrees after surgery. After the 1- to 3-year follow-up period, the maxilla was stable in the sagittal plane. Minimal anteroposterior growth was observed in the maxilla compared with that exhibited in the anterior cranial base. However, there was significant vertical maxillary growth over the 3-year observation period. The mandibular plane angle tended to decrease during the follow-up period. The cephalometric data from this study support the clinical impression of maxillary stability after maxillary advancement with rigid external distraction in cleft patients. This effective and stable technique is now considered for all pediatric patients with severe cleft maxillary hypoplasia and for adolescent and adult patients with moderate to severe deformities.

The impact of early infant jaw-orthopaedics on early speech production in toddlers with unilateral cleft lip and palate.

The purpose of study was to investigate the impact of pre-surgical Infant Orthopaedics (IO) on consonant production at 18 months of age in children with Unilateral Cleft Lip and Palate (UCLP) and to compare the consonant production to that of age-matched children without clefts. The first ten children in a consecutive series of 20 with UCLP received IO and the following ten did not. Both groups had soft palate repair at 6.3 months of age. The cleft in the hard palate was unrepaired. Ten normally developing children without clefts served as controls. Nine children in each group accomplished the study. Phonetic transcriptions of consonants were made from audiotape recordings obtained during a 45-60 minute interactive session. No significant differences in the number of consonant tokens or of consonant types were found between the UCLP children with and without IO but both groups had significantly lower numbers than the control group. There was no significant difference in frequency of different manners or places of articulation of plosives between the UCLP groups. The frequency of occurrence of bilabial and dental consonant placements of plosives were significantly higher in the control group than in both UCLP groups.

Spring-mediated cranioplasty compared with the modified pi-plasty for sagittal synostosis.

The aim of this study was to compare the safety, morphological outcome, and degree of parental satisfaction of the new spring-mediated cranioplasty with those of the modified pi-plasty in the management of sagittal synostosis. Ten patients with non-syndromic sagittal synostosis treated with the spring-mediated cranioplasty were followed prospectively. A control group of 10 sex-matched patients operated on with the modified pi-plasty procedure was chosen. Cephalometric radiographs were obtained preoperatively and postoperatively at 1 year of age. Cephalic index, axial width ratio, length ratio, width ratio and height ratio were used as objective measures of outcome. Parents were sent a questionnaire to obtain a subjective aesthetic assessment of outcome. Significantly less blood replacement was required (p = 0.003), and shorter duration of postoperative anaesthesia (p = 0.030) and postoperative hospital stay (p = 0.013) were found in the spring-mediated cranioplasty group. There were no complications or deaths in either group. Also significant was the inter-group difference in the postoperative change in the height ratio (p = 0.030), the most change being seen in the spring group. The change in the subjective parental aesthetic evaluation of skull shape was significant in both groups. In conclusion, the spring-mediated procedure was morphologically more effective than the modified pi-plasty procedure in the management of sagittal synostosis with the additional benefits of less blood transfusion needed and shorter duration of hospital stay.

Dental eruption and exfoliation chronology in the ferret (Mustela putorius furo).

Substituting ferrets for rats and dogs as animal models for craniofacial research is favourable because of the similarity of many of the ferret's anatomical, metabolic and physiological features to those of man. Other advantages are cost-effectiveness and possibly less ethical controversy. However, information on the dental chronology of ferrets needs to be supplemented if this animal is to be promoted as an alternative model. Dental development was here examined in 16 ferrets (eight males, eight females) from three litters at between 12 and 90 days of age. Dental eruption and exfoliation were assessed and recorded every second day. The sequence of eruption of deciduous and permanent teeth was determined and data were analysed statistically. Also, any sex-related differences in eruption and exfoliation ages were defined. No deciduous incisors were observed to erupt in this group of animals. Other deciduous teeth erupted between the 19th and 31st postnatal days, and exfoliated between days 51 and 76. The time of eruption of the permanent teeth ranged from 42 to 77 days, in accordance with the stage of the mixed dentition. The female ferrets were generally ahead of the males in the exfoliation age of their deciduous teeth and the eruption age of their permanent teeth, but this, a sex difference did not apply to the eruption age of the deciduous teeth. These extended basic data might facilitate the introduction of this alternative experimental animal into craniofacial research.

Dental arches and occlusion in bilateral cleft lip and palate patients after two different routines for palatal surgery.

BACKGROUND: Delayed hard palate repair (DHPR) is believed by many researchers to improve maxillary growth and facial appearance in patients born with cleft lip and palate. However, only few studies dealing with the midfacial growth outcome after this type of surgery in bilateral cleft patients have been published. PATIENTS AND METHOD: The purpose of this retrospective study was to compare long-term results of maxillary morphology, dental arches and occlusion in two groups of patients with bilateral cleft lip and palate. The palatal surgery differed between the two groups, particularly with respect to the timing of hard palate repair. The DHPR group (n = 16) underwent soft palate closure at 12 months and hard palate repair at around 8 years, whereas the early palatal repair group (EPR) (n = 12) had completed two-stage palatal closure during the first year of life. These latter subjects had undergone more traditional palatal surgery with vomer flaps for repair of the anterior part and push-back closure for the posterior part of the cleft. Surgery was performed in both groups by the same surgical team at Sahlgrenska University Hospital, Göteborg, Sweden. Dental casts were used to analyze the pre- and postoperative maxillary morphology, dental arch dimensions, and occlusion of both samples, which were followed longitudinally from infancy to early adulthood. RESULTS: Differences recorded in both maxillary growth and occlusion were generally in favor of the DHPR group. However, major intragroup variations and relatively small sample sizes precluded statistical verification of the differences, except for development during the early stages.