Scandcleft randomized trials of primary surgery for unilateral cleft lip and palate: Speech proficiency at 10 years of age.

BACKGROUND & AIM: To assess consonant proficiency and velopharyngeal function in 10-year-old children born with unilateral cleft lip and palate (UCLP) within the Scandcleft project. METHODS & PROCEDURES: Three parallel group, randomized, clinical trials were undertaken as an international multicentre study by nine cleft teams in five countries. Three different surgical protocols for primary palate repair (Arm B-Lip and soft palate closure at 3-4 months, hard palate closure at 36 months, Arm C-Lip closure at 3-4 months, hard and soft palate closure at 12 months, and Arm D-Lip closure at 3-4 months combined with a single-layer closure of the hard palate using a vomer flap, soft palate closure at 12 months) were tested against a common procedure (Arm A-Lip and soft palate closure at 3-4 months followed by hard palate closure at 12 months) in the total cohort of 431 children born with a non-syndromic UCLP. Speech audio and video recordings of 399 children were available and perceptually analysed. Percentage of consonants correct (PCC) from a naming test, an overall rating of velopharyngeal competence (VPC) (VPC-Rate), and a composite measure (VPC-Sum) were reported. OUTCOMES & RESULTS: The mean levels of consonant proficiency (PCC score) in the trial arms were 86-92% and between 58% and 83% of the children had VPC (VPC-Sum). Only 50-73% of the participants had a consonant proficiency level with their peers. Girls performed better throughout. Long delay of the hard palate repair (Arm B) indicated lower PCC and simultaneous hard and soft palate closure higher (Arm C). However, the proportion of participants with primary VPC (not including velopharyngeal surgeries) was highest in Arm B (68%) and lowest in Arm C (47%). CONCLUSIONS & IMPLICATIONS: The speech outcome in terms of PCC and VPC was low across the trials. The different protocols had their pros and cons and there is no obvious evidence to recommend any of the protocols as superior. Aspects other than primary surgical method, such as time after velopharyngeal surgery, surgical experience, hearing level, language difficulties and speech therapy, need to be thoroughly reviewed for a better understanding of what has affected speech outcome at 10 years. WHAT THIS PAPER ADDS: What is already known on the subject Speech outcomes at 10 years of age in children treated for UCLP are sparse and contradictory. Previous studies have examined speech outcomes and the relationship with surgical intervention in 5-year-olds. What this study adds to the existing knowledge Speech outcomes based on standardized assessment in a large group of 10-year-old children born with UCLP and surgically treated according to different protocols are presented. While speech therapy had been provided, a large proportion of the children across treatment protocols still needed further speech therapy. What are the potential or actual clinical implications of this work? Aspects other than surgery and speech function might add to the understanding of what affects speech outcome. Effective speech therapy should be available for children in addition to primary surgical repair of the cleft and secondary surgeries if needed.

The Americleft Project: A Comparison of Short- and Longer-Term Secondary Alveolar Bone Graft Outcomes in Two Centers Using the Standardized Way to Assess Grafts Scale.

OBJECTIVE: To compare length of follow-up and cleft site dental management on bone graft ratings from two centers. DESIGN: Blind retrospective analysis of cleft site radiographs and chart reviews for determination of cleft-site lateral incisor management. PATIENTS: A total of 78 consecutively grafted patients with complete clefts from two major cleft/craniofacial centers (43 from Center 1 and 35 from Center 2). INTERVENTIONS: Secondary iliac crest alveolar bone grafting, at a mean age of 9 years 9 months (Center 1: 9 years 7 months; Center 2: 10 years 0 month). MAIN OUTCOME MEASURES: The Americleft Standardized Way to Assess Grafts scale from 0 (failed graft) to 6 (ideal) was used to rate graft outcome at two time points (T1, T2). Average T1 was 11 years 1 month of age, 1 year 3 months postgraft. Average T2 was 17 years 11 months of age, 8 years 0 months postgraft. Six trained and calibrated raters scored each radiograph twice. Reliability was calculated at T1 and T2 using weighted kappa. A paired Wilcoxon signed rank test (P < .05) tested T1 and T2 differences for each center. A Kruskal-Wallis test was used to determine the significance of differences between centers at T1 and T2. Correlation tested whether T1 ratings predicted T2. Linear regression determined possible factors that might contribute to graft rating changes over time. RESULTS: Reliability was good at T1 and T2 (interrater = .713 and .701, respectively; intrarater = .790 and .805, respectively). Center 1 scores were significantly better than those from Center 2 at both T1 (5.21 versus 3.29) and T2 (5.18 versus 3.44). There was no statistical difference between T1 and T2 scores for either center; although, there was a greater chance of bone graft score improving with completion of canine eruption and substitution for missing lateral incisors. CONCLUSIONS: Short-term ratings of graft outcomes identified significant differences between centers that persisted over time. Dental cleft-site management influenced final graft outcome.

The Americleft Project: A Proposed Expanded Nasolabial Appearance Yardstick for 5- to 7-Year-Old Patients With Complete Unilateral Cleft Lip and Palate (CUCLP).

OBJECTIVE: To develop a yardstick of reference photographs for nasolabial appearance assessments of 5- to 7-year-old patients with complete unilateral cleft lip and palate (CUCLP). DESIGN: Blind retrospective analysis of clinical records and comparison to historical controls. PATIENTS: Subjects were two groups of 6- to 12-year-olds (n = 124 and n = 135) and one group of 5- to 7-year-olds (n = 149) with nonsyndromic CUCLP from three previous Americleft studies, including cohorts from seven different cleft/craniofacial centers. INTERVENTIONS: All patients received the infant management protocols of their respective centers. Eleven trained and calibrated judges (five participated in all three studies) did blind ratings of nasolabial appearance using the Asher-McDade method. MAIN OUTCOME MEASURES: Patients receiving the most consistent ratings between judges, selected first from the groups of 6- to 12-year-olds, were used to create a pilot yardstick for eventual use in the third study of 5- to 7-year-olds. For each of the Asher-McDade categories, 8 of the 5- to 7-year-old patients receiving the most consistent scores between raters were ranked by 10 judges for a final elimination to leave three per category. RESULTS: Using this method of successive changes in rating methods, a new reference yardstick for nasolabial appearance rating was established and linked to the original Asher-McDade method as well as the single examples in a previously published yardstick for patients with CUCLP. Pilot testing using the yardstick improved reliabilities. CONCLUSIONS: Use of an expanded nasolabial yardstick of reference photographs representative of the range of possibilities of each of the five Asher-McDade categories is now available to see if reliability of these ratings can be improved.

Reduced complement activation with heparin-coated oxygenator and tubings in coronary bypass operations.

Complement activation after cardiopulmonary bypass is correlated with postoperative organ dysfunction. Heparin coating of the entire blood-contact surface of the cardiopulmonary bypass circuit has proved to reduce complement activation in vitro. A membrane oxygenator and tubing setup coated with functionally active heparin was compared with an uncoated, otherwise identical setup in 20 patients undergoing routine coronary bypass operations. The concentrations of C3 activation products and the terminal complement complex were measured in sensitive and specific enzyme immunoassays. Peak concentrations of C3 activation products were 90.1 (74.7 to 107.4) AU/ml (medians and 95% confidence intervals) and 52.4 (35.7 to 76.4) AU/ml with the uncoated and coated setups, respectively (p = 0.02). The corresponding concentrations of the terminal complement complex were 26.2 (20.1 to 37.5) AU/ml and 13.7 (11.1 to 25.1) AU/ml (p = 0.03). Blood loss from the mediastinal drains during the first 12 postoperative hours was 533 (416 to 975) ml in patients treated with the uncoated setup and 388 (313 to 579) ml in the coated treatment group (p = 0.06) and was significantly correlated with peak concentrations of the terminal complement complex (p = 0.01). There were no differences in neutrophil counts nor platelet numbers between the treatment groups. The approximate 45% reduction in complement activation with the heparin-coated cardiopulmonary bypass device indicates a substantial improvement of biocompatibility.

Reduced complement and granulocyte activation with heparin-coated cardiopulmonary bypass.

Plasma concentrations of the complement activation products C3b, iC3b, and C3c; the terminal C5b-9 complement complex; and the granulocyte proteins calprotectin, myeloperoxidase, and lactoferrin were assessed in two groups of patients undergoing aortocoronary bypass procedures. In 10 patients operated on, the bypass circuits were coated by the Carmeda Bio-Active Surface and systemic heparinization was reduced to 1.5 mg/kg; in another 10, the systems were uncoated and the dosage of systemic heparinization was 4 mg/kg. In both groups, significant complement activation was observed after the onset of cardiopulmonary bypass, but the maximum levels of C3b, iC3b, and C3c and the terminal C5b-9 complement complex were significantly lower in the heparin-coated group. In both groups, a significant increase in calprotectin, myeloperoxidase, and lactoferrin release was observed by the end of operation. The maximum myeloperoxidase levels were significantly lower in the heparin-coated group than those in the uncoated group (p = 0.03). There was a correlation of borderline significance between the formation of terminal C5b-9 complement complex and lactoferrin release, as well as between the formation of terminal C5b-9 complement complex and myeloperoxidase release (p = 0.05). The postoperative blood loss did not differ significantly between the two groups. We conclude that coating by end point-attached and functionally active heparin allows a significant reduction in the amount of systemic heparinization, and significantly reduces complement and granulocyte activation.

The origin of creatine kanase BB occurring in serum during aortocoronary bypass surgery.

The serum concentration of creatine kinase (CK)-BB during and after coronary bypass surgery was measured by a sensitive and highly specific radioimmunoassay technique. All patients showed a distinct and temporary increase in CK-BB concentration shortly after the start of operation. Our results indicate that this increase has no connection with damage of the heart or the brain. Most probably the BB isoenzyme is liberated mainly from the saphenous vein as a consequence of the surgical manipulations during the heart operation.

Rhabdomyoma of the heart with intrapericardial expansion.

A rare case of a rhabdomyoma originating from the surface of the right atrium in a 20-year-old female is reported. The tumor showed electrical activity and caused both outflow obstruction and tamponade symptoms. It was successfully removed by a right-sided thoracotomy. The clinical course, diagnostic investigations, and the therapeutical approach of this rare lesion are discussed.

Strut fracture with Björk-Shiley 70 degrees convexo-concave valve. An international multi-institutional follow-up study.

Between 1980 and 1983, 831 Björk-Shiley 70 degrees convexo-concave prosthetic heart valves were implanted at five institutions in Sweden, Germany. The Netherlands, and Canada. As of January 1991, there were 34 outlet strut fractures occurring from 0.2 to 10.1 years (median = 4.6 years) after implantation. In addition, there were 28 sudden, unexplained deaths. The mortality after strut fracture was 84%. The mortality after emergency valve replacement for strut fracture was 50%. The 10-year actuarial fracture rate (standard error) was 10.5 (2.4)% for large (29-33 mm) valves vs. 3.3 (1.2)% for 21-27 mm valves (P less than 0.001). Within valve size groups, fracture rates for aortic and mitral valves were similar. Cox regression analysis found only valve size to be significantly associated with strut fracture. There is a further subgrouping of the valves according to the manufacturer: group I are the earlier large 29-33 mm) valves; group II are the later large valves; group III are the small size (21-27 mm) valves. The risk of strut fracture was highest in group I (12.3% at 10 years) with an approximatively constant hazard (1.4% per year). A comparison was made with a statistical model incorporating all cases reported to the manufacturer. This model estimates fracture rates approximately 63%-73% of those found in the present study. These findings lead us to recommend that group I patients should be considered for elective reoperation on an individual basis, giving careful attention to risk factors and contraindications.

Risk factors for morbidity and mortality in mitral valve replacement.

Risk factors of operative mortality and long term survival were identified in 219 patients who underwent mitral valve replacement (MVR) using Bjørk-Shiley mechanical prostheses. Early mortality was 7.3%. The accumulated follow-up time was 1134 patient-years, and the 5-year survival for the total cohort was 78 +/- 3%. Independent prognostic factors of early mortality were poor NYHA class, which carried a relative risk (RR) of 3.2, and ischaemic aetiology, with a RR of 2.2. Ischaemic aetiology was the sole predictor of heart pump failure requiring intra-aortic balloon pump support (RR = 2.7). Independent risk factors of total mortality (early and late) were male sex (RR = 2.3), NYHA class III-IV (RR = 2.4), presence of mitral regurgitation (RR = 3.2) and relative heart volume (RR = 1.6 for a 800 ml/m2 size compared to a heart of 550 ml/m2). Our results underline the importance of patient-related factors in MVR, and indicate that care is needed in comparing the quality of MVR from different institutions with respect to mortality and morbidity. The results of MVR are palliative rather than curative except in female patients with NYHA class II function and mitral stenosis, in whom cure was attained.

The Eurocleft project 1996-2000: overview.

INTRODUCTION: The original Eurocleft project, a European intercentre comparison study, revealed dramatic differences in outcome, which were a powerful stimulus for improvement in the services of respective teams. The study developed a preliminary methodology to compare practices and the potential for wider European collaboration including opportunities for the promotion of clinical trials and intercentre comparison was recognized by the European Commission. Therefore, the project: 'Standards of Care for Cleft Lip and Palate in Europe: Eurocleft' ran between 1996 and 2000 and aimed to promote a broad uplift in the quality of care and research in the area of cleft lip and palate. RESULTS: The results of the 1996-2000 project include: a register of services in Europe, with details of professionals and teams involved in cleft care, service organization, clinical protocols and special facilities for research; a set of common Policy Statements governing clinical practice for European cleft teams, Practice Guidelines describing minimum recommendations for care that all European children with clefts should be entitled to and recommendations for Documentation governing minimum records that cleft teams should maintain; encouraging initial efforts to compare outcomes (results) of care between centres. A survey showed a wide diversity in models of care and national policies as well as clinical practices in Europe. Of the 201 centres that registered with the network, the survey showed 194 different protocols being followed for only unilateral clefts. CONCLUSION: Cleft services, treatment and research have undoubtedly suffered from haphazard development across Europe. Attainment of even minimum standards of care remains a major challenge in some communities and both the will to reform and a basic strategy to follow are overdue. It is hoped that the Eurocleft Consensus Recommendations reached during the present project will assist in improving the opportunities for tomorrow's patients. It is also hoped that the collaborative research now beginning under the European Commission's Framework V Programme will provide a focus for European researchers wishing to improve understanding, treatment and prevention of clefts of the lip, alveolus and palate in the years ahead.

Results of multidisciplinary management of bilateral cleft lip and palate at the Iowa Cleft Palate Center.

Bilateral cleft of the lip and palate is by many standards the most complex and severe form of the defect. The complexity and severity of the defect require an unusual degree of cooperation among all specialists and especially between the surgeon and the orthodontist. There are no published findings that we know about in which comprehensive data from a number of disciplines are reported for the same group of bilateral cleft patients. Fifty randomly selected patients with bilateral complete clefts were examined by the Iowa team and two orthodontists from other institutions. The evaluations revealed that a large number of patients over the age of 10 have multiple residual problems requiring further treatment. Only 23 percent of the older patients studied were judged to have had treatment completed by the surgeon, speech pathologist, and orthodontist. It is very difficult to state whether the results obtained by our team can be considered satisfactory because there are no comparable studies that have attempted to evaluate the same parameters in multidisciplinary management.

Outcomes of cleft care in Western Australia: a pilot study.

The purpose of this study was to compare outcomes and delivery of cleft care in Western Australia with the average standard of care in the United Kingdom (UK). This was achieved through a cross-sectional study involving children born with unilateral cleft lip and palate between April 1983 and March 1985 (12 year olds) or between April 1990 and March 1992 (5 year olds). A total of 38 children born with unilateral cleft lip and palate were under the care of the cleft team based at Perth's Princess Margaret Hospital. Dental arch relations, facial skeletal pattern, speech, hearing, success of alveolar bone grafting and dental health were measured. It was found that fewer Princess Margaret Hospital children in both age cohorts had revision surgery and speech therapy compared with the UK average. The facial skeletal pattern, speech, hearing and alveolar bone grafting outcomes from Princess Margaret Hospital were similar to the UK at age 12. Seventeen per cent of the Princess Margaret Hospital 12 year olds had a poor dental arch relationship compared with 39 per cent in the UK. In the 5 year olds, most outcomes in Princess Margaret Hospital patients appeared better than the UK with lower residual treatment needs. While it is difficult to draw firm conclusions because of the small numbers involved, this study indicates standards need to be set and determined for Australia.

The effects of mastery criteria and assignment length on college student test performance.

This study analyzed the function of two components of a personalized instruction course -mastery criteria for passing a test and assignment length. A high mastery criterion (100% correct) and short assignments produced better test performance than either a low mastery criterion (60% correct) or long assignments (four short assignments combined) on both study question items that students had in their possession and probe items that were not available to students in advance.

The effects of study questions and grades on student test performance in a college course.

Two experiments demonstrated the effects of study questions on student test performance in an introductory college course. Students in both experiments correctly answered study question items 20 to 30% more frequently than non-study question probes. Furthermore, mean performance on study question items was better than 90% during all phases of both experiments. The present experiments were also designed to study the effects of grades on test performance, and the relationship between long and short sets of study questions. The results of Experiment I clearly illustrated the importance of using grades to maintain high levels of student test performance. The results of Experiment II suggested that long sets of study questions may produce better performance on probe items than do short sets of study questions, but the effect was small.

A procedure for maintaining student progress in a personalized university course.

Ten students in a personalized university course were given target dates for completing each of 26 lessons. The lessons could be completed before those dates, but not after. The first two failures to complete a lesson by the target date led to "warnings"; the next failure required the student to withdraw from the course. When each student's rate of lesson completion was compared with and without target dates, it was found that students completed an average of 1.0 lesson a day with the target-date contingency and 0.3 without it. Individual data indicated that most students did few or no lessons without the contingency. It was concluded that a target-date contingency is an effective method for maintaining student progress in personalized university courses.

Craniofacial development in children with unilateral clefts of the lip, alveolus, and palate treated according to four different regimes. III. The soft tissue profile at 16-18 years of age.

The soft tissue profiles of 85 16-18 year old children with unilateral clefts of the lip, alveolus, and palate treated according to four different regimes were studied using lateral skull radiographs. Regimes that included secondary bone grafting to the alveolus in the mixed dentition gave better convexity of the soft tissue profile (excluding the nose) and better soft tissue sagittal jaw relationships compared with treatment regimens that included primary bone grafting. The soft tissue profile in regimes that excluded bone grafting was almost as favourable as those in regimes in which bone grafting was done during the mixed dentition.

Craniofacial development in children with unilateral clefts of the lip, alveolus, and palate treated according to three different regimes. Assessment of nasolabial appearance.

From extraoral photographs taken from the front and in profile of 61 16-year old children with unilateral cleft lip, alveolus, and palate (UCLAP) who had been treated by three different treatment regimes, the nasolabial appearances were assessed by a panel. The photographs were masked, leaving only the mid face including the nose and lips. The following features were assessed using a five point scale: nasal form, symmetry of the nose, vermilion of the upper lip, shape of the vermilion border, total symmetry of upper lip, and nasal profile including the upper lip. The number and type of secondary operations required were recorded. Intraobserver reliability was good but interobserver agreement was poor, some observers systematically scoring more severely than others. A panel of six was therefore set up to establish an acceptable mean assessment. The treatment regime that included secondary bone grafting, and the one that included primary bone grafting and presurgical orthopaedic-T-traction, scored better on all features assessed compared with the group that underwent primary bone grafting but no T-traction. The latter group required fewer secondary revisionary procedures, however, which could explain these results.

Craniofacial development in children with unilateral clefts of the lip, alveolus, and palate treated according to four different regimes. I. Maxillary development.

Lateral skull radiographs of 85 patients with unilateral clefts of the lip, alveolus, and palate treated according to four different regimes were compared at three different ages regarding maxillary development. Regimes that included primary bone grafting to the alveolus at six months of age resulted in inhibited anterior maxillary growth and reduced maxillary inclination. Regimes that included secondary bone grafting after eruption of the incisors but before the eruption of the canines, resulted in better maxillary development, but were not as good as regimes that omitted bone grafting altogether.

Craniofacial development in children with unilateral clefts of the lip, alveolus, and palate treated according to four different regimes. II. Mandibular and vertical development.

Lateral skull radiographs of 85 patients with unilateral clefts of the lip, alveolus and palate treated according to four different regimes were compared longitudinally, at three different ages, regarding mandibular and vertical facial development. It was found that there were no lasting differences in mandibular morphology resulting from regimes including primary or early secondary bone grafting. The non grafted group, however, showed increased mandibular length and anterior height. The jaw angle was increased and there was a more favourable sagittal jaw relationship. Regimes that included primary bone grafting were associated with reduced upper anterior facial height, which resulted in less harmonious facial proportions compared with treatment regimes including early secondary bone grafting done during the mixed dentition, or no bone grafting at all. Vertical development was greatest where bone grafting was excluded.