Validity of the Cleft Aesthetic Rating Scale Does Not Improve with Inclusion of Fixed Scores for Atypical Outcomes.

OBJECTIVE: The Cleft Aesthetic Rating Scale (CARS) is a valid tool to assess the aesthetic outcome after unilateral cleft lip and palate (UCLP) repair. The goal of this study was to investigate whether the reliability of CARS increases by including fixed rates for atypical outcomes such as a smaller nostril, lower vermillion border or whistling deformity. DESIGN: 197 patients with UCLP who underwent cleft repair were included. Three cleft experts rated the post-operative photos using the original CARS, followed by a second assessment employing the modified CARS. SETTING: Medisch Centrum Leeuwarden, Universitair Medisch Centrum Groningen, Radboud Universitair Medisch Centrum and Amsterdam University Medical Center. PATIENTS: Photographs of 16- to 25-year-old patients with repaired UCLP. INTERVENTIONS: Two separate assessments using the original and modified CARS. MAIN OUTCOME MEASURES: The Intraclass Correlation Coefficient (ICC) was used to test inter-rater reliability of the original and modified version of the CARS. RESULTS: The reported ICC for the nose scored by means of the original and modified CARS were 0.68 (95% CI 0.62-0.74) and 0.66 (95% CI 0.59-0.72) respectively. The reported ICC for the lip assessed with the original and modified CARS were 0.53 (95% CI 0.33-0.67) and 0.57 (95% CI 0.34-0.72) respectively. CONCLUSIONS: No significant difference was found between the original and modified ICC of the nose and lip. The implementation of the fixed rates did not result in an increased reliability of the CARS. Therefore, we recommend the utilization of the original CARS.

What Parents of Children Born with a Cleft Lip and/or Palate Want to Know About the Care for their Child.

OBJECTIVE: Adequate health information that matches the needs of care recipients is a prerequisite for patient-centered care. To facilitate the provision of tailored and timely information, it isimportant to understand the information needs of parents of children and adolescents with cleft lip and/or palate (CL/P) themselves, and in addition they were asked how they experienced the provided care-related information. DESIGN: A cross-sectional study employing questionnaires and semi-structured interviews. SETTING: Participants were recruited from a cleft palate-craniofacial care unit in a major tertiary hospital in the Netherlands. PARTICIPANTS: Participants were parents or guardians of children with CL/P, and two adolescents with CLP. They were recruited through the outpatient clinic during multidisciplinary consultation or after clinical admission. RESULTS: In total, fifty-five questionnaires were completed by parents or guardians and eleven interviews were conducted with nine parents of children with CL/P and two adolescents with CL/P. In general, participants reported to be satisfied with provided information during hospital admission or multidisciplinary cleft team consultations (mean 8.0, scale 0-10). In addition, 25.5% (n = 14) indicated that information to prepare for hospital admission was lacking (eg, practical information). Thematic qualitative analysis yielded five main information needs: 1) Clear communication during the care process, 2) Overview of the care trajectory, 3) Specific care plan information, 4) Presentation of information and 5) Guidance and support. CONCLUSIONS: Our findings emphasize the importance of gaining insights into wishes and information needs from care recipients who can provide insights in their information needs. With these findings, information provision should be redesigned to improve and to foster the further transition to family-centered care.

Dentition patterns in bilateral cleft lip subphenotypes: multicenter study.

OBJECTIVES: Here, we retrospectively investigated cases of bilateral oral clefts (OCs) to determine the clinical relevance of detailed distinction of incomplete cleft lip subphenotypes, based on morphological severity of the cleft, within the categories cleft lip with or without alveolus (CL ± A) and cleft lip, alveolus, and palate (CLAP). We further assessed possible associations between CL subphenotypes (complete vs different incomplete types) and different dentition patterns of the lateral incisor. MATERIALS AND METHODS: Our analysis included 151 non-syndromic Caucasian bilateral OC-patients (8-20 years old) from the Dutch Association for Cleft Palate and Craniofacial Anomalies registry. Six different deciduous and permanent lateral incisor patterns were distinguished: normal position (z/Z), supernumerary lateral incisor (n/N), presence in the anterior (x/X) or posterior (y/Y) segment of the cleft, one in each cleft segment (xy/XY), and agenesis (ab/AB). Logistic regression was performed to show the associations between the CL subphenotypes and dentition patterns of the lateral incisor. RESULTS: One hundred three had complete, while 48 had incomplete CLs. Patterns z/Z and n/N were associated with a submucous/vermillion notch, incomplete CL, and intact alveolus. Patterns x/X, y/Y, and xy/XY were most common in patients with two-thirds to subtotal CL and complete CL. The most severe pattern, ab/AB, was most commonly associated with complete CL. CONCLUSIONS: Based on the morphological severity of the CLs, it can be stated that the more severe the CL in bilateral CL ± A and CLAP, the more severe the abnormal pattern of the dentition. CLINICAL RELEVANCE: Further distinction of incomplete cleft lip subphenotypes (submucous/vermillion notch, one-third to two-thirds CL, two-thirds to subtotal CL) in bilateral CL ± A and CLAP has clinical relevance.

The Reproducibility of the Jaw Index in the Measurement of Healthy Newborns.

OBJECTIVE: Establish the reliability of the jaw index to objectify the relationship between the maxilla and mandible in healthy newborns. DESIGN: Cohort study. SETTING: Tertiary setting. PATIENTS: A total of 52 healthy newborns were included to detect an inter and intraclass correlation coefficient (ICC) of 0.8 with a 95% confidence interval (95% CI) of width 0.3. Inclusion criteria were children born full term without respiratory or feeding problems, and without congenital malformations or facial deformities due to birth trauma. Uncooperative patients were excluded. INTERVENTIONS: The jaw index, a measuring tool for objectifying micrognathia in children suspected of having Robin sequence, was used. An ICC of greater than 0.8 was considered clinically relevant. MAIN OUTCOME MEASURE(S): Primary outcomes are the reliability of the jaw index expressed as interclass correlation coefficient and ICC. Secondary outcomes are the mean jaw index and mean length of the mandible, maxilla, and the alveolar overjet. RESULTS: An interclass correlation coefficient of 0.74 (95% CI: 0.49-0.86) and an ICC of 0.81 (95% CI: 0.66-0.89) were found. The mandible had an average length of 162.6 mm (standard deviation [SD] 11.1), the maxilla 168.7 mm (SD 9.4), the alveolar overjet 2.0 mm (SD 0.60), and the mean jaw index was 2.1 (SD 0.64). CONCLUSION: The jaw index is a consistent instrument between different observers as well as for one observer measuring consecutively in the same child, to objectify the size of the lower jaw compared to that of the upper jaw in healthy newborns.

Aesthetics Assessment and Patient Reported Outcome of Nasolabial Aesthetics in 18-Year-Old Patients With Unilateral Cleft Lip.

OBJECTIVE: To determine if there is a correlation between objective nasolabial aesthetics assessment using the Cleft Aesthetic Rating Scale (CARS) and patient satisfaction. DESIGN: Retrospective analysis of a generic satisfaction questionnaire and independent assessment by three cleft surgeons of the nasolabial area of these patients on 2D frontal photographs, using the CARS. SETTING: The Vrije Universiteit Medical Center and The Academic Center for Dentistry Amsterdam. PATIENTS: Thirty-nine 18-year old patients with a repaired complete or incomplete unilateral cleft lip, with or without a cleft palate, and a completed satisfaction questionnaire. Exclusion criteria were an incomplete questionnaire; a history of facial trauma; and congenital syndromes affecting facial appearance. MAIN OUTCOME MEASURES: The correlation between surgeon evaluation (on a 5-point Likert scale) and patient satisfaction (not, moderately or very satisfied) on nasolabial appearance was assessed using Spearman rho (ρ). RESULTS: There was a negligible correlation between surgeon evaluation and patient satisfaction on nose assessment (ρ = 0.20) and a moderate correlation on lip assessment (ρ = 0.32). CONCLUSIONS: Most literature supports this discrepancy between different objective aesthetics evaluation methods and subjective patient-reported outcome measures, suggesting there are factors playing a role in patient satisfaction that are impossible to objectify with assessment methods. Therefore, a strong emphasis should remain on clear communication between the physician and patient regarding their expectations, perception, and satisfaction of surgery results.

The Cleft Aesthetic Rating Scale for 18-Year-Old Unilateral Cleft Lip and Palate Patients: A Tool for Nasolabial Aesthetics Assessment.

OBJECTIVE: To develop a reliable and easy-to-use method to assess the nasolabial appearance of 18-year-old patients with unilateral cleft lip and palate (CLP). DESIGN: Retrospective analysis of nasolabial aesthetics using a 5-point ordinal scale and newly developed photographic reference scale: the Cleft Aesthetic Rating Scale (CARS). Three cleft surgeons and 20 medical students scored the nasolabial appearance on standardized frontal photographs. SETTING: VU University Medical Center, Amsterdam. PATIENTS: Inclusion criteria: 18-year-old patients, unilateral cleft lip and palate, available photograph of the frontal view. EXCLUSION CRITERIA: history of facial trauma, congenital syndromes affecting facial appearance. Eighty photographs were available for scoring. MAIN OUTCOME MEASURES: The interobserver and intraobserver reliability of the CARS for 18-year-old patients when used by cleft surgeons and medical students. RESULTS: The interobserver reliability for the nose and lip together was 0.64 for the cleft surgeons and 0.61 for the medical students. There was an intraobserver reliability of 0.75 and 0.78 from the surgeons and students, respectively, on the nose and lip together. No significant difference was found between the cleft surgeons and medical students in the way they scored the nose ( P = 0.22) and lip ( P = 0.72). CONCLUSIONS: The Cleft Aesthetic Rating Scale for 18-year-old patients has a substantial overall estimated reliability when the average score is taken from three or more cleft surgeons or medical students assessing the nasolabial aesthetics of CLP patients.

Comparison of two- and three-dimensional assessment methods of nasolabial appearance in cleft lip and palate patients: Do the assessment methods measure the same outcome?

OBJECTIVE: For the assessment of the nasolabial appearance in cleft patients, a widely accepted, reliable scoring system is not available. In this study four different methods of assessment are compared, including 2D and 3D asymmetry and aesthetic assessments. METHODS: The data and ratings from an earlier study using the Asher-McDade aesthetic index on 3D photographs and the outcomes of 3D facial distance mapping were compared to a 2D aesthetic assessment, the Cleft Aesthetic Rating Scale, and to SymNose, a computerized 2D asymmetry assessment technique. The reliability and correlation between the four assessment techniques were tested using a sample of 79 patients. RESULTS: The 3D asymmetry assessment had the highest reliability and could be performed by just one observer (Intraclass correlation coefficient (ICC): 0.99). The 2D asymmetry assessment of the nose was highly reliable when performed by just one observer (ICC: 0.89). However, for the 2D asymmetry assessment of the lip more observers were needed. For the 2D aesthetic assessments 3 observers were needed. The 3D aesthetic assessment had the lowest single-observer reliability (ICC: 0.38-0.56) of all four techniques. The agreement between the different assessment methods is poor to very poor. The highest correlation (R: 0.48) was found between 2D and 3D aesthetic assessments. Remarkably, the lowest correlations were found between 2D and 3D asymmetry assessments (0.08-0.17). CONCLUSION: Different assessment methods are not in agreement and seem to measure different nasolabial aspects. More research is needed to establish exactly what each assessment technique measures and which measurements or outcomes are relevant for the patients.

The Asher-McDade Aesthetic Index in Comparison With Two Scoring Systems in Nonsyndromic Complete Unilateral Cleft Lip and Palate Patients.

OBJECTIVE: To compare the Asher-McDade aesthetic index with 2 systems used to score the appearance of the nasolabial area in patients with a complete cleft lip and palate. DESIGN: Retrospective analysis of the results of complete unilateral cleft lip and palate patients. SETTING: Academic Center for Dentistry of Amsterdam and the VU University Medical Center. PATIENTS: Six-year-olds with complete unilateral cleft lip and palate. MAIN OUTCOME MEASURES: Cleft lip and palate patients assessed using the scoring system proposed by Prahl et al, a 5-point ordinal scale, and the scoring system proposed by Asher-McDade et al by 6 judges, 3 orthodontists, and 3 plastic surgeons. A calculation of intra- and interobserver reliability was made. A comparison was made of all the assessment methods using Kendalls' tau. RESULTS: Photographs of 55 children (38 boys and 17 girls) with complete unilateral cleft lip and palate were assessed. For the scoring system of Prahl et al, interobserver reliability varied from 0.43 to 0.53, for the 5-point scale between 0.45 and 0.57, and for the scoring system by Asher-McDade et al these varied between 0.52 and 0.66. Multiple significant correlations were found between the used scoring systems. CONCLUSION: It can be concluded that the Asher-McDade aesthetic index is still superior to the other scoring systems used in this study. However, all 3 scoring systems can reliably be used when 3 or more observers are used.

Comparison of two scoring systems in the assessment of nasolabial appearance in cleft lip and palate patients.

OBJECTIVE: The aim of this study was to determine the reliability of 2 scoring systems. DESIGN: This study used a retrospective analysis of the results of complete unilateral cleft lip and palate patients. SETTING: The study was conducted at the VU Medical Center and the Academic Center for Dentistry of Amsterdam. PATIENTS: Patients were complete unilateral cleft lip and palate patients at the age of 6 years. MAIN OUTCOME MEASURES: Assessment of the nose and lip together and separately with a numerical photographic reference scoring system and with a 5-point ordinal scale without the use of a reference photograph by 6 judges. Intraobserver and interobserver reliability was calculated; both ways of assessment were compared by using Kendall tau. RESULTS: Photographs were available of 55 children (6 years old, 38 boys and 17 girls) with a complete unilateral cleft lip and palate. The interobserver reliabilities of the lip and nose together were 0.53 and, for the nose and lip separately, 0.51 and 0.43, respectively with the use of the numerical scale. In the 5-point scale, these were 0.55 for the nose and lip together and 0.57 and 0.45 for the nose and lip separately, respectively. Furthermore, it was found that the lip dominates in the scorings of the lip and nose together (linear regression analysis). CONCLUSIONS: The 2 tested systems are equivalent in their reliability and outcome. The lip is dominating in the overall scorings. It is advocated to use the 5-point scale without the use of a reference photograph and to assess the lip and nose separately.

Tissue expansion for correction of baldness in aplasia cutis congenita.

UNLABELLED: Aplasia cutis is a congenital absence of the skin, usually presenting on the scalp. In 20% of all cases, part of the skull is also absent. A residual area of baldness may still be present some years after surgical or conservative treatment. It is possible to excise the scarred hairless region and cover that area with expanded hair-bearing skin from the rest of the skull. We present three patients who underwent tissue expansion and discuss the indications and pitfalls of this procedure. CONCLUSION: Tissue expansion can be used to cover a residual alopecia defect in young children with aplasia cutis congenita and associated bone abnormalities. The quality of the bone appears to be normal in our three patients. We demonstrate that even in young children with aplasia cutis and an underlying bony defect, tissue expansion is a safe and effective modality as a second stage reconstruction procedure.

Regional underreporting of associated congenital anomalies in cleft patients in the Netherlands.

OBJECTIVE: The Dutch Cleft Palate Association (DCPA) registers all patients with cleft lip or palate and associated congenital anomalies in the Netherlands. The aim of this study was to assess if early registration of cleft patients leads to underreporting of associated congenital anomalies and, if so, whether reregistration is necessary. METHODS: The DCPA registration of the birth cohort 1997 to 2001 was compared with the medical files of these cleft patients for prevalence, type, and moment of registration of associated congenital anomalies. To assess possible long-term underregistration, a second birth cohort of 1990 to 1991 was analyzed. RESULTS: The percentage of cleft patients with associated congenital anomalies was 26% in the DCPA database and 33% in the retrospective medical file review. A syndrome, sequence, or association was recognized in 8% of the cleft patients by the DCPA compared with 13% in our medical file review. Of all associated congenital anomalies diagnosed during a follow-up of 12 years, 53% were diagnosed in the first year of life. The cumulative percentage was 59% after 2 years, 62% after 3 years, 80% after 6 years, and 97% after 10 years. CONCLUSION: Early registration of cleft patients leads to underreporting of other associated anomalies. For a complete registration of associated congenital anomalies in cleft patients, reregistration at a later age is necessary.