Comment on Ombashi, van der Goes, Versnel, Khonsari, van der Molen: guidance to develop a multidisciplinary, international, pediatric registry: a systematic review, Orphanet Journal of Rare diseases, 2023.

Recently, Ombashi et al. published a systematic review aiming to identify the pitfalls in the development and implementation as well as factors influencing long-term success of a multidisciplinary, international registry for cleft care on a global scale. The purpose of this letter to the editor is to highlight that the review failed to include the Swedish quality registry for patients born with cleft lip and palate, which fulfils the inclusion criteria. The Swedish cleft lip and palate registry is multidisciplinary, has a high coverage and reporting degree, and most outcome measures have been checked for reliability and validity. It is regularly used for open comparisons between treatment centers. Several research studies have been published based on the Swedish cleft lip and palate registry, and more are ongoing. The information we provide about the Swedish cleft lip and palate registry complements and expands the information of the results reported by Ombashi et al. in their research.

Surgical treatment of velopharyngeal dysfunction: Incidence and associated factors in the Swedish cleft palate population.

INTRODUCTION: Speech in children with cleft palate can be affected by velopharyngeal dysfunction, which persists after primary palate repair. The incidence of surgery to correct velopharyngeal dysfunction in this patient group has previously been reported as 2.6-37%. We aimed to investigate the incidence of velopharyngeal dysfunction surgery in Swedish children with cleft palate and to examine potential associations of independent variables with this incidence. METHODS: In this cohort study, we analysed data from the Swedish cleft lip and palate quality registry for 1093 children with cleft palate with or without cleft lip. Kaplan-Meier analysis was used to estimate the risk of having velopharyngeal dysfunction surgery. Multivariable Cox proportional hazards models were used to estimate the associated effect of cleft subtype, additional diagnoses, gender, and age at and number of stages for primary palate repair on the primary outcome. RESULTS: The risk of having velopharyngeal dysfunction surgery was 25.6%. Complete primary palate repair after the age of 18 months or in more than one stage was associated with a higher risk, but it could not be determined which of these was the more significant factor. Cleft soft palate was associated with a significantly lower risk than other cleft subtypes. CONCLUSIONS: Primary palate repair at a higher age or in more than one stage may increase the risk of having velopharyngeal dysfunction surgery. Further analysis of potential unknown confounding factors and the association between the incidence of velopharyngeal dysfunction and surgery to correct this condition is needed.

Quantitative assessment of cleft volume and evaluation of cleft's impact on adjacent anatomical structures using CBCT imaging.

OBJECTIVES: To determine pre-operative cleft volume and evaluate cleft´s impact on surrounding anatomical structures in children and adolescents with orofacial clefts using cone bean computed tomography (CBCT) imaging. METHODS: The present retrospective study retrieved CBCT examinations of 68 patients from a previous study. The examinations had been exposed either before (n = 53) or after (n = 15) alveolar bone grafting. Pre-operative volume of cleft was determined, and type and location were evaluated. Morphological changes on the adjacent anatomical structures, including the incisive foramen, the nasal septum and floor, and the inferior turbinate, were assessed. RESULTS: Mean bilateral cleft volume was 0.76 cm3, while mean unilateral cleft volume was 1.08 cm3; the difference was significant (p < 0.001). Variation in cleft volume, however, was large. The incisive foramen was not visible in the majority of cases with bilateral clefts (71%); the difference was significant (p = 0.001). In cases with unilateral clefts, the nasal septum in 87% was curved towards the cleft or graft side. Also, the mean size of the widest part of the inferior turbinate was 8.8 mm on the cleft or graft side and 10.4 mm on the non-cleft side. The difference was significant (p < 0.001). CONCLUSIONS: When required, CBCT is a feasible method for quantitatively illustrating alveolar clefts and their impact on the morphological development of surrounding structures. Variation in cleft volume was large.

A systematic review of differences in outcome between one and two stage palate repair in cleft lip and palate.

The aim of this systematic review was to determine whether one-stage palatoplasty for children born with cleft lip and palate shows overall advantages in outcome compared with two-stage palatoplasty. The included studies were controlled studies of syndromic and non-syndromic children born with unilateral cleft lip and palate, bilateral cleft lip and palate, or isolated cleft palate. The interventions studied were one-stage palatoplasty and two-stage palatoplasty starting with the soft palate. The outcomes were facial growth, speech, hearing, presence of fistulae, other complications related to surgery, health-related quality of life, and health economics. In total, 14 original studies were included. Results were dichotomized into showing advantage for one- or two-stage palatoplasty for the respective outcome and compared with the results from six included systematic reviews. No overall advantage for either surgical strategy was found for any of the outcome measures. The certainty of evidence was highest for the presence of fistulae, followed by facial growth and speech. For several outcomes, the quality of the existing evidence was too low to allow for any conclusions to be drawn. Neither one- nor two-stage palatoplasty showed significant advantages in clinical outcomes compared with the other. Other aspects such as ethics, economics, or surgeon's preference might hence be of more importance. Homogenous choices of outcome measures and defined minimal clinically important differences would facilitate further research.

Expander prosthesis and DIEP flaps in delayed breast reconstruction: Sensibility, patient-reported outcome, and complications in a five-year randomised follow-up study.

Breast reconstruction is a given choice for many women following mastectomy. There are a multitude of methods available today, and thus, comparative studies are essential to match patients with suitable methods. The aim of this study was to compare 5-year outcomes following delayed breast reconstruction with expander prosthesis (EP) and with deep inferior epigastric perforator (DIEP) flaps. Seventy-three patients, previously randomised to either a permanent EP or a DIEP flap breast reconstruction, were invited for a 5-year follow-up. Assessments included symmetry measurements, breast sensibility with Semmes-Weinstein monofilaments and patient-reported outcome (PRO) with the BREAST-Q. Complications within the first 5 postoperative years were recorded. Additionally, BREAST-Q questionnaires were collected from non-randomised patients with an EP breast reconstruction. Between 2019 and 2022, 65 patients completed the follow-ups. Symmetry and PRO were significantly higher in the DIEP flap group. However, EP-reconstructed breasts were significantly more sensate and demonstrated areas with protective sensibility, unlike the DIEP flap breasts. The overall complication rates were comparable between the two groups (p = 0.27). Regression analysis identified body mass index as a risk factor for reoperation in general anaesthesia and for wound infection. No significant differences were found in a comparison of the randomised and the non-randomised EP groups' BREAST-Q results. This randomised 5-year follow-up study found PRO to be favourable following a DIEP flap reconstruction and sensibility to be better in EP reconstructions. The complication rates were comparable; however, longer follow-ups are warranted to cover the complete lifespans of the two breast reconstruction methods.

Additional diagnoses in children with cleft lip and palate up to five years of age.

Cleft lip and palate (CL/P) is the most common congenital craniofacial malformation and is often associated with additional diagnoses. The purpose of this study was to explore the cumulative five-year incidence of additional diagnoses for patients with cleft lip and palate. Further aims were, type of cleft and type of additional diagnose and to validate CLP registry data on additional diagnoses. Data from the CLP registry regarding children with CL/P in the Southern Health Care Region were retrieved and based on the registry, participants were selected. A review of medical records of participants born 2006-2016 was performed and data regarding participant characteristics and additional diagnoses were collected. Of the 250 participants included in the review of medical records, 90 participants (36%) had an additional diagnosis. Of the total number of identified additional diagnoses (n = 137), cardiovascular system (20.4%) and extremities and skeletal system (17.5%) were the most prevalent categories. The comparison between medical records and the CLP registry of all children showed a 14.4 percentage points higher incidence of additional diagnoses in the medical records. Roughly every third child received an additional diagnosis and diagnoses related to the cardiovascular system were the most frequent. This study also shows that additional diagnoses were under-reported in the CLP registry. Future research is necessary to strengthen associations of additional diagnoses to CL/P.

Long-term patient-reported outcomes after anterior distraction osteogenesis of the maxilla in patients with cleft.

Maxillary growth inhibition in patients with cleft lip and palate (CLP) is an undesired effect that may occur in the teens despite proper primary care. Dental malocclusion and distortion of facial appearance can be treated with external distraction osteogenesis (DO) of the maxilla. This entails a Le Fort I osteotomy, fastening a semi-circular distractor to the skull, distraction for three weeks, and fixation for three months before removal of the device.The aim of this descriptive long-term follow-up study was to evaluate DO of the maxilla from the patient-reported long-term perspective.Fourteen patients underwent a long-term follow-up including a questionnaire regarding their experience of DO. Sex, CLP diagnosis, age at DO and follow-up, and time required for active distraction and fixation were noted. Furthermore, documentation on rhinoplasty, lip plasty and velopharyngeal plasty after DO was registered. Objective results were assessed by a positive dental overjet in the front.Ten patients considered the distractor an everyday constraint, but all thought the procedure was worthwhile and would recommend it to others. Thirteen patients experienced improved bite and chewing, whereas one considered function unchanged. All were satisfied with their dental alignment. Three patients underwent a velopharyngeal plasty after DO. Moreover, six rhinoplasties and two lip plasties were performed.Despite a long and challenging treatment, teenagers and young adults with CLP and maxillary hypoplasia tolerate DO of the maxilla very well. Secondary measures to improve speech and appearance are often indicated.

Evaluation of an assessment scale for aesthetic outcome in breast reconstructions based on digital photos in both 2D and 3D format.

The aesthetic outcome is crucial in a breast reconstruction. Our aim was to evaluate the intra- and interrater reliability of an aesthetic outcome assessment scale with digital photos of breast reconstructions in two-dimensional (2D) and three-dimensional (3D) format. Thirty-three women with delayed breast reconstructions, consecutively participating in a five-year follow-up between November 2019 and June 2021, were included in the study. Of these, 14 were reconstructed with an expander prosthesis (EP) and 19 with a deep inferior epigastric perforator (DIEP) flap. Photos of the breasts were assessed in 2D and 3D format by expert, layman and patient panels. Data were analysed with the weighted kappa (wk) statistics. The intrarater agreements were moderate to substantial, with wk between 0.66 and 0.73 for the panels. Within the panels, the interrater agreements were 0.46-0.62. Moderate agreements were found between the matched 2D and 3D format photos (wk 0.62-0.66). The patient panel graded scar appearance worse in 3D compared with 2D format. In all panels, there was a tendency towards DIEP flap reconstructions receiving higher aesthetic outcome grades compared with EP. Thus, the aesthetic outcome assessment scale demonstrated acceptable agreements between the individual panellists and within the panels. Scars captured in 3D format may provide a greater resemblance to the reality compared with 2D. Implications for clinics remain to be further studied.

Do preterm-born children and adolescents have greater need for dental care as compared to full term-born controls?

BACKGROUND: Preterm birth has been shown to cause various long-term health issues. Children who were born preterm have also been observed to have more dental behaviour management problems (DBMP) during dental examinations and treatment than children born full term. It is known that dental radiographic examinations can be uncomfortable and cause anxiety in paediatric patients. Thus, our aims were to retrospectively compare dental care related examinations and treatments carried out in three different age intervals (3-6 years, 7-12 years, and 13-19 years) among preterm- and full-term born children and adolescents. METHODS: The present study included 311 patient files: 122 very preterm-born and 33 extremely preterm-born children and adolescents (< 32 gestational weeks). A matched control group of 156 full term-born children and adolescents (≥ 37 gestational weeks) was analysed for comparison. Various factors, including DBMP, missed appointments, dental caries, and radiographic examinations, were retrieved from the dental records for three age intervals: 3-6 years, 7-12 years, and 13-19 years. RESULTS: Extremely preterm-born children missed significantly more dental appointments and presented with more DBMP during dental examinations and treatment than full term-born children in the 3-6-year age group; the same was observed for the very preterm-born in the 7-12-year age group. No significant differences in DBMP during bitewing and periapical examinations or in number of bitewing, periapical and panoramic radiographs occurred between the groups in any age interval. CONCLUSION: Preterm-born children and adolescents may need more flexibility in booking and receive reminders for scheduled visits with the general dental team. Due to the non-significant differences in dental care related oral examinations and treatments, the same dental care service may be applied to the preterm- and full-term born children and adolescents.

Inter-centre comparison of data on surgery and speech outcomes at 5 years of age based on the Swedish quality registry for patients born with cleft palate with or without cleft lip.

BACKGROUND: The objective of the Swedish cleft lip and palate registry (CLP registry) is to promote quality control, research and improvement of treatment, by the comparison of long-term results. The aim was to compare data from the CLP registry among the six treatment centres, regarding data on surgery and speech outcomes at 5 years of age. METHODS: The participants were 430 children born in Sweden from 2009 to 2014, with cleft palate with or without cleft lip and without known syndromes and/or additional malformations. The number of primary and secondary palatal surgeries up to 5 years of age, timing of the last primary palatal surgery, percentage consonants correct, percentage non-oral speech errors and perceived velopharyngeal competence at 5 years were assessed. Multivariable binary logistic regression adjusted for sex and cleft type was used to compare results between the six centres. RESULTS: At one centre (centre 4), the palate was closed in one to three stages, and at the remaining centres in one or two stages. At centre 4, more children underwent a higher number of palatal surgeries, and the last primary palatal surgery was performed at a higher age. Children in centre 4 were also less likely to achieve ≥86% correct consonants (OR = 0.169, P = < 0.001), have no non-oral speech errors (OR = 0.347, P = < 0.001), or have competent or marginally incompetent velopharyngeal competence (OR = 0.244, P = < 0.001), compared to the average results of the other centres. No clear association between patient volume and speech outcome was observed. CONCLUSIONS: The results indicated the risk of a negative speech result if the last primary palatal surgery was performed after 25 months of age. Whether the cleft in the palate was closed in one or two stages did not affect speech outcome. The Swedish CLP registry can be used for open comparisons of treatment results to provide the basis for improvements of treatment methods. If deviating negative results are seen consistently at one centre, this information should be acted upon by further investigation and analysis, making changes to the treatment protocol as needed.

Assessment of local tissue water in breasts following breast reconstruction with an expander prosthesis or DIEP flap.

The role of breast oedema in breast reconstruction is unknown. Therefore, our aim was to investigate local tissue water (LTW) and breast oedema-related symptoms in breasts reconstructed with either an expander prosthesis (EP) or with a deep inferior epigastric perforator (DIEP) flap at a minimum of one year postoperatively. Sixty-eight patients randomised to breast reconstruction with an EP or DIEP flap completed follow-up. Objective evaluation was performed at a mean of 25 (standard deviation, SD 9.5) months following breast reconstruction, and included measurements of breast volume and LTW with the MoistureMeterD® instrument. The patients completed the BREAST-Q questionnaire pre- and postoperatively. No significant differences in LTW were found when comparing EP and DIEP flap reconstructed breasts. The reconstructed breasts had an increase in LTW compared with the non-operated contralateral breasts. The BREAST-Q responses related to breast oedema symptoms were overall low and the median responses ranged from 1 to 2. A score of 1 indicated that symptoms were experienced 'None of the time'. Our findings indicate that mastectomy followed by breast reconstruction inflicts damage on the lymphatic system, shown as an increase in LTW. However, no breast oedema-related symptoms were reported in the BREAST-Q questionnaire, and therefore, we consider our objective results to be below a potential threshold for symptomatic breast oedema. A threshold for clinical indication of breast oedema remains to be defined.

Speech in a consecutive series of children born with cleft lip and palate with and without syndromes and/or additional malformations.

BACKGROUND: When evaluating speech in children with cleft palate with or without cleft lip (CP/L), children with known syndromes and/or additional malformations (CP/L+) are usually excluded. The aim of this study was to present speech outcome of a consecutive series of 5-year-olds born with CP/L, and to compare speech results of children with CP/L + and children with CP/L without known syndromes and/or additional malformations (CP/L-). METHODS: One hundred 5-year-olds (20 with CP/L+; 80 with CP/L-) participated. All children were treated with primary palatal surgery in one stage with the same procedure for muscle reconstruction. Three independent judges performed phonetic transcriptions and rated perceived velopharyngeal competence from audio recordings. Based on phonetic transcriptions, percent consonants correct (PCC) and percent non-oral errors were investigated. Group comparisons were performed. RESULTS: In the total group, mean PCC was 88.2 and mean percent non-oral errors 1.5. The group with bilateral cleft lip and palate (BCLP) had poorer results on both measures compared to groups with other cleft types. The average results of PCC and percent non-oral errors in the CP/L + group indicated somewhat poorer speech, but no significant differences were observed. In the CP/L + group, 25 % were judged as having incompetent velopharyngeal competence, compared to 15 % in the CP/L- group. CONCLUSIONS: The results indicated relatively good speech compared to speech of children with CP/L in previous studies. Speech was poorer in many children with more extensive clefts. No significant differences in speech outcomes were observed between CP/L + and CP/L- groups.

Comparison of Corresponding Scores From the Cleft Hearing Appearance and Speech Questionnaire (CHASQ) and CLEFT-Q in Swedish Patients With Cleft Lip and/or Palate.

OBJECTIVE: The primary aim of this study was to compare corresponding scores between 2 existing cleft-specific patient-reported outcome measures (PROMs)-Cleft Hearing Appearance and Speech Questionnaire (CHASQ) and CLEFT-Q. The second aim of the study was to investigate patient opinion on the 2 PROMs. DESIGN: Cross-sectional questionnaire study. SETTING: Participants were recruited from a University Hospital. They answered CHASQ and CLEFT-Q either in the hospital or at home. PARTICIPANTS: Thirty-three participants with cleft lip and/or palate, aged 10 to 19 years. MAIN OUTCOME MEASURE: CHASQ and CLEFT-Q. RESULTS: The CHASQ scores and the corresponding CLEFT-Q scores on appearance correlated significantly. Corresponding scores regarding speech did not correlate significantly. A majority, 15 (58%) participants, answered that they liked CLEFT-Q more than CHASQ, 18 participants (69%) thought CHASQ was easier to complete, and 19 (76%) thought CLEFT-Q would better inform health care professionals. CONCLUSION: Both instruments showed strengths and limitations. Clinicians will have to consider each instrument's respective qualities when choosing to implement either PROM.

Scores of the Cleft Hearing, Appearance and Speech Questionnaire (CHASQ) in Swedish Participants With Cleft lip and/or Cleft Palate and a Control Population.

OBJECTIVE: The primary aim of this study was to investigate whether there was any difference in scores of the Cleft Hearing, Appearance and Speech Questionnaire (CHASQ) between patients with cleft lip and/or cleft palate (CL/P) and a control population. The second aim was to compare CL/P and control population scores in this study with a British norm CL/P population. DESIGN: Single-site, cross-sectional study with an age-matched control population. SETTING: Participants were recruited from a hospital, a school, and a sports club. They answered the CHASQ in the hospital or at home. PARTICIPANTS: Sixty-four participants with CL/P (7-19 years of age) and a control population of 56 participants without CL/P (9-20 years of age). MAIN OUTCOME MEASURE: CHASQ. RESULTS: There was no statistically significant difference in satisfaction with cleft-related features between the CL/P and the control population. Participants with CL/P were significantly more satisfied with non-cleft-related features than the control population. Cleft Hearing, Appearance and Speech Questionnaire scores were also similar to earlier established British normative data of a CL/P population. CONCLUSION: The results indicated that children and young people with CL/P were as satisfied with their appearance, hearing, and speech as children and young people without CL/P. Swedish CHASQ scores were also similar to British scores.

Coverage, reporting degree and design of the Swedish quality registry for patients born with cleft lip and/or palate.

BACKGROUND: The objective of the Swedish cleft lip and palate (CLP) registry is to promote quality control, research and improvement of treatment, by comparison of the long-term results of surgery, orthodontics and speech from all six Swedish CLP centres. The purpose of the study was to investigate the coverage and reporting degree of the Swedish CLP registry, and to describe the design of the registry and discuss questions of reliability and validity of the data included. METHODS: All six Swedish CLP centres participate in the registry. All children in Sweden with cleft lip and/or cleft palate, born from 2009 onwards, are included in the registry. Baseline data such as cleft type (ICD-10 diagnosis), heredity, birth weight and additional deformities and/or syndromes, as well as pre-surgical treatment, are recorded at first visit. Data on surgical treatment are recorded continuously. Treatment outcome regarding dentofacial development and speech are recorded at follow-ups at 5, 10, 16 and 19 years of age. Data on dentofacial development are also recorded 1 year after orthognathic surgery. In addition, data on babbling and speech are recorded at 18 months of age. Coverage degree and reporting degree of surgery was assessed by comparison with registrations in the Swedish Central patient registry. Reporting degree of orthodontic and speech registrations at 5 years of age was assessed by comparison with registrations at baseline. RESULTS: The average coverage degree for children born 2009 to 2018 was 95.1%. For cleft-related surgeries, the average reporting degree was 92.4%. Average reporting degree of orthodontic registrations and speech registrations at age 5 years was 92 and 97.5% respectively. CONCLUSION: In order to achieve valid and reliable data in a healthcare quality registry, the degree of coverage and reporting needs to be high, the variables included should be limited and checked for reliability, and the professionals must calibrate themselves regularly. The Swedish CLP registry fulfils these requirements.

On the role of the frontal projection in videoradiography of velopharynx in decision-making for a velopharyngeal flap plasty in patients with cleft palate.

Despite uneventful primary surgery, patients with cleft palate may experience velopharyngeal insufficiency (VPI) and hypernasal speech. Videoradiography of velopharynx is a commonly used method to visualize velopharyngeal function and a velopharyngeal flap is often used to counteract VPI. The aim of this study was to investigate whether the frontal projection on videoradiography plays a role in the decision-making about velopharyngeal flap surgery, or possibly the width and orientation of the flap. A secondary aim was to evaluate the effect of the flap in improving velopharyngeal function. Between 2007 and 2016, 75 patients had received a flap at our department. During the same period of time, 41 patients who had undergone videoradiography did not receive a flap. Medical records, particularly regarding speech assessments, videoradiography statements and operating records, were scrutinised to seek information about the factors leading up to the decision about whether or not to perform a flap. In only one instance, reduced lateral pharyngeal wall movement found on the frontal projection was clearly taken into account when deciding to refrain from performing a velopharyngeal flap. Only a slight agreement was found between pre-operative speech assessment and findings in videoradiography. Hypernasality was reduced by flap surgery in 97% of the patients. We conclude the frontal projection of the videoradiographic examination seems to have no crucial role in the decision-making on performing a velopharyngeal flap or not in patients with cleft palate. Even with reduced lateral pharyngeal wall movement, a velopharyngeal flap effectively reduces hypernasality and VPI.

Validation of reported dentoalveolar relationships in the Swedish Quality Registry for Cleft Lip and Palate.

OBJECTIVES: The present study validated data that had been reported to the Swedish Quality Registry for Cleft Lip and Palate (CLP) under new requirements from 2016, when use of the 5-year-old (5YO) and the Modified Huddart and Bodenham (MHB) indices for rating occlusion in children born with unilateral CLP (UCLP) was introduced. MATERIALS AND METHODS: The sample included blinded study casts (n = 97) and photos (n = 4) of 5-year-old children who had been born with UCLP in 2009-2011 and were enrolled at one of six cleft centres in Sweden. Fourteen orthodontists from the centres assessed the patients (n = 101) using the 5YO and the MHB indices. Median 5YO and MHB scores of the 14 assessments were compared with original registry data (n = 61). Each centre devised code keys to protect the identities of their patients in the registry. RESULTS: Interrater agreement among the 14 orthodontists was good for the 5YO index (quadratic-weighted kappa: 0.72-0.92) and the MHB index (intraclass correlation coefficient: 0.991-0.994). Comparisons of median 5YOs for each identifiable child with their registry data (n = 61) found total agreement for 70.5 per cent. Comparisons between median MHBs and registry data showed very good or good agreement in 93.4 per cent of the cases. LIMITATIONS: Two teams lost their code keys, which reduced the sample to 61 patients. CONCLUSIONS: The dentoalveolar outcome data in the CLP registry was trustworthy. There was good agreement among the Swedish cleft teams assessing the 5YO and MHB indices in children born with UCLP at age 5 years.

Speech in 5-Year-Olds With Cleft Palate With or Without Cleft Lip Treated With Primary Palatal Surgery With Muscle Reconstruction According to Sommerlad.

OBJECTIVE: To evaluate speech in 5-year-olds with cleft palate with or without cleft lip (CP±L) treated with primary palatal surgery in 1 stage with muscle reconstruction according to Sommerlad at about 12 months of age. DESIGN: Retrospective study. SETTING: Primary care university hospital. PARTICIPANTS: Eight 5-year-olds with cleft soft palate (SP), 22 with cleft soft/hard palate (SHP), 33 with unilateral cleft lip and palate, and 17 with bilateral CLP (BCLP). MAIN OUTCOME MEASURES: Percent oral consonants correct (POCC), percent consonants correct adjusted for age (PCC-A), percent oral errors, percent nonoral errors, and variables related to velopharyngeal function were analyzed from assessments of audio recordings by 3 independent speech-language pathologists. RESULTS: The median POCC was 75.4% (range: 22.7%-98.9%), median PCC-A 96.9% (range: 36.9%-100%), median percent oral errors 3.4% (range: 0%-40.7%), and median percent nonoral errors 0% (range: 0%-20%), with significantly poorer results in children with more extensive clefts. The SP group had significantly less occurrence of audible nasal air leakage than the SHP and the BCLP groups. Before age 5 years, 1.3% of the children underwent fistula surgery and 6.3% secondary speech improving surgery. At age 5 years, 15% of the total group was perceived as having incompetent velopharyngeal function. CONCLUSIONS: Speech was poorer in many children with more extensive clefts. Children with CP±L had poorer speech compared to normative data of peers without CP±L, but the results indicated relatively good speech compared to speech of children with CP±L in previous studies.

Evaluation of Facial Anthropometry Using Three-Dimensional Photogrammetry and Direct Measuring Techniques.

In this study, 14 standard facial distances on 10 adult volunteers were measured directly with a caliper and indirectly on two-dimensional images using the 3dMDtrio system. Two raters performed the measurements with at least 1 week between rating sessions. The intra- and inter-rater reliabilities and agreement of the measurements were calculated using intra-class correlation coefficient (ICC), mean absolute difference (MAD), and Bland-Altman plots with limits of agreement (LOA). The 2 raters had an average discrepancy (MAD) of 1.6 mm when their digital measurements were compared to their direct measurements. The reliability of the digital and direct methods varied greatly depending on which of the 14 anthropometric distances that was being assessed. Only 6 digitally and 5 directly measured anthropometric distances showed both an ICC >0.75 and a MAD <1 mm, in the intra-rater as well as the inter-rater measurements. The Bland-Altman plots and LOA displayed the same pattern. In summary, the digital and direct methods were generally compatible in terms of reliability and agreement. However, the reliability and agreement between the 14 anthropometric measurements varied considerably, indicating that poor landmark identification is the main limitation to both modern and traditional measuring techniques in the face. Consequently, some anthropometric landmarks warrant further definition or prior anthropometric training by the evaluators. The authors also recommend that the MADs and LOAs provided in this report are put into relation to the facial distance that is being evaluated and its clinical context.

Problems With Reliability of Speech Variables for Use in Quality Registries for Cleft Lip and Palate-Experiences From the Swedish Cleft Lip and Palate Registry.

OBJECTIVE: To test the reliability of the speech data in the Swedish quality registry for cleft lip and palate. DESIGN: Retrospective study. SETTING: Primary care university hospital. PARTICIPANTS: Ninety-four children born with cleft palate with or without cleft lip between 2005 and 2009 who had been assessed and registered in the quality registry at the age of 5 years. MAIN OUTCOME MEASURES: Data in the registry on percent oral consonants correct, percent oral errors, percent nonoral errors, perceived velopharyngeal function (PVPF), and intelligibility were compared with results based on reassessments by 3 independent raters from audio recordings. Agreement was calculated by the intraclass correlation coefficient (ICC), quadratic weighted kappa, and percentage agreement. RESULTS: Absolute agreement calculated by average measures ICC for percent oral consonants correct, percent oral errors, and percent nonoral errors was above >0.90. Single measures ICC for percent oral consonants correct was 0.82, for percent oral errors 0.69, and for percent nonoral errors 0.83. The kappa coefficient for PVPF was 0.5 to 0.59 and for intelligibility 0.65 to 0.77. Exact percentage agreement for PVPF was 33% and for intelligibility 47.8%. CONCLUSIONS: The data on oral consonants correct and nonoral errors in the quality registry seem to be reliable. The data on oral errors, PVPF, and intelligibility should be interpreted with caution. If differences among treatment centers are detected, one should go back and examine the collected raw data before drawing any definitive conclusions about treatment outcome.