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Objective: Parents of children with a medical condition and a visible difference can experience challenging situations. We evaluated distress and parenting stress in parents of children with a cleft lip with or without cleft palate (CL±P) or a visible infantile hemangioma (IH). Setting: This cross-sectional study took place in an academic medical hospital in Rotterdam, the Netherlands. Participants: Three-hundred nine parents (mean age = 40.30, 56.00% mothers) of children with CL ± P and 91 parents (mean age = 36.40, 58.24% mothers) of children with IH. Main Outcome Measures: The Dutch version of the Parenting Stress Index - Short Form and the subscales Anxiety, Depression, and Hostility of the Symptom Checklist - 90. Results: One sample t tests and mixed linear modeling were used. On average, parents of children with CL ± P and of children with IH showed significantly lower parenting stress compared to normative data. Anxiety was significantly lower in parents of children with CL ± P than that in the norm group. Visibility of the condition was not related to distress or parenting stress. Child behavioral problems were positively related to parenting stress, depression, and hostility. Conclusions: Parents of children with CL ± P and IH report less distress and parenting stress compared to the norm. On average, these parents seem well adjusted. A practical implication is to monitor parents of children with behavioral problems. [ABSTRACT FROM AUTHOR]

There is considerable variability in the management of common orofacial clefts across Europe, reflecting differing opinions on optimal treatments. An updated overview of treatment protocols for orofacial clefts across 26 expert centres in the European Reference Network CRANIO is presented here. A structured questionnaire was distributed to map the surgical protocol and additional standard procedures for cleft palate (CP), unilateral cleft lip and palate (UCLP), and bilateral cleft lip and palate (BCLP). A surgical protocol was defined as the unique combination of a sequence of standard surgeries, their timing, and the surgical techniques employed. Overall, 33 unique surgical protocols for CP, 54 for UCLP, and 51 for BCLP were identified. Notable findings included the trend towards early hard palate closure, uniform timing of lip closure, and the popularity of primary cleft rhinoplasty. Nevertheless, practice variations were most pronounced in the timing of alveolar closure, the number of standard surgeries, and the application of additional standard procedures. This study highlights the diversity of treatment protocols across Europe, despite considerable convergence of treatment practices over time. To allow for further convergence, establishing objective criteria for protocol selection, adequate documentation of customizations, and consensus on the terminology of surgical techniques, are necessary., Competing Interests: Conflict of Interest This research is generated within the European Reference Network for rare and/or complex craniofacial anomalies and ear, nose and throat (ENT) disorders (ERN CRANIO). ERN CRANIO is funded by the European Union. The content of this paper represents the views of the authors only and it is their sole responsibility; it cannot be considered to reflect the views of the European Commission and/or the European Health and Digital Executive Agency (HaDEA) or any other body of the European Union. The European Commission and the Agency do not accept any responsibility for use that may be made of the information it contains. Thus, the authors of current study had no competing interest caused by the (partial) funding of this research. The authors that contributed to current study also have no other competing interest., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

There is still no unanimous agreement on the optimal surgical protocol(s) for the treatment of unilateral cleft lip and palate (UCLP), and a huge variety of protocols are employed by cleft centres across the world. The aim of this systematic review and meta-analysis was to compare reported patient outcomes of the Oslo protocol (and modifications) (OP) and delayed hard palate closure protocols (DHPCP) from a multidisciplinary perspective. A systematic search of multiple databases was conducted until September 2023. Studies reporting any patient outcomes of these protocols were included. Random-effects meta-analyses were performed for evidence synthesis, including comparisons of results between the types of protocol. The quality of evidence was evaluated using the ROBINS-I tool. In total, 62 articles (42 studies) reporting patients with UCLP were reviewed, involving 1281 patients following the OP and 655 following DHPCP. Equally poor long-term sagittal maxillofacial growth was found, and similar results for velopharyngeal insufficiency and nasolabial appearance. In contrast, OP was associated with a lower rate of oronasal fistulas. Disregarding the scarcity of comparable evidence for some domains, the results of this review, overall, favour OP over DHPCP. However, caution should be taken when interpreting the results on velopharyngeal insufficiency and oronasal fistulas, since the possibility of confounding and other biases remains., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: The aim of this study is to describe and compare head shape in surgically and conservatively treated patients using 3D photogrammetry., Methods: A retrospective review (2017-2020) of consecutive patients with isolated metopic synostosis based on 3D photogrammetry was conducted at the age of 4 years old. Images were aligned using a healthy age-matched template, and mean head shapes were reconstructed to evaluate shape development. A comparative sub-analysis based on phenotype was performed between patients that have been treated surgically and conservatively., Results: 44 patients with isolated metopic synostosis were included: 22 received conservative treatment and 22 underwent fronto-orbital advancement. At 4 years of age the surgical group showed retrusion of the complete frontal area, while the conservative group showed a slight frontal prominence. Both groups showed temporal depression with respect to the control. In the sub-analysis, a similar degree of temporal depression was observed between surgical and conservative treatment. Head shape patterns showed considerable similarity across all severity phenotypes., Conclusion: This study shows a deviation in forehead shape from normal controls in patients with metopic synostosis following both surgical and conservative treatment by the age of 4 years. Comparison between surgical and conservative treatment shows a similar degree of temporal depression, a slight prominence in the center of the forehead in the conservative group, and retrusion of the entire frontal area in the surgical group. This observed difference is of considerable similarity across all severity types., Level of Evidence Therapeutic: III., Competing Interests: Conflicts of interest: No conflicts of interest, (Copyright © 2024 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of the American Society of Plastic Surgeons. All rights reserved.)

This study was performed to evaluate the efficacy of outcome measures for the orofacial domain included in the International Consortium for Health Outcomes Measurement Standard Set for Cleft Lip and Palate (ICHOM-SCS). In this multicentre study involving two cleft centres, suggestions to optimize the type and timing of outcome measures were made based on data and clinical experience. Patient-reported outcome measures (PROMs) (CLEFT-Q Jaw, Teeth, Eating/Drinking; Child Oral Health Impact Profile-Oral Symptoms Scale (COHIP-OSS)) and clinical outcome measures (caries experience and dental occlusion) data were collected retrospectively for age 5, 8, 10, 12, 19, and 22 years. The data were categorized by cleft type and analysed within and between age groups using Spearman correlation, the distribution of responses per item, a two-sample test for equality of proportions, and effect plots. Most correlations between PROMs and clinical outcome measures were weak (r < 0.5), suggesting PROMs and clinical outcome measures complement each other. The COHIP-OSS and CLEFT-Q Eating/Drinking barely detected problems in any patient category and are no longer recommended. A suitable alternative appears complex to find; outcomes of this study and the recent literature doubt an added value. Similar problems were found in the CLEFT-Q Jaw at time-point 12 years. Therefore, time-points 15 and 17 years are currently suggested., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background  The best timing of closure of the hard palate in individuals with cleft lip, alveolus, and palate (CLAP) to reach the optimal speech outcomes and maxillary growth is still a subject of debate. This study evaluates changes in compensatory articulatory patterns and resonance in patients with unilateral and bilateral CLAP who underwent simultaneous closure of the hard palate and secondary alveolar bone grafting (ABG). Methods  A retrospective study of patients with nonsyndromic unilateral and bilateral CLAP who underwent delayed hard palate closure (DHPC) simultaneously with ABG at 9 to 12 years of age from 2013 to 2018. The articulatory patterns, nasality, degree of hypernasality, facial grimacing, and speech intelligibility were assessed pre- and postoperatively. Results  Forty-eight patients were included. DHPC and ABG were performed at the mean age of 10.5 years. Postoperatively hypernasal speech was still present in 54% of patients; however, the degree of hypernasality decreased in 67% ( p  < 0.001). Grimacing decreased in 27% ( p  = 0.015). Articulation disorders remained present in 85% ( p  = 0.375). Intelligible speech (grade 1 or 2) was observed in 71 compared with 35% of patients preoperatively ( p  < 0.001). Conclusion  This study showed an improved resonance and intelligibility following DHPC at the mean age of 10.5 years, however compensatory articulation errors persisted. Sequential treatments such as speech therapy play a key role in improvement of speech and may reduce remaining compensatory mechanisms following DHPC., Competing Interests: Conflict of Interest None declared., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. ( https://creativecommons.org/licenses/by/4.0/ ).)

This study was performed to describe observed healthcare utilization and medical costs for patients with a cleft, compare these costs to the expected costs based on the treatment protocol, and explore the additional costs of implementing the International Consortium for Health Outcomes Measurement (ICHOM) Standard Set for Cleft Lip and Palate (CL/P). Forty patients with unilateral CL/P between 0 and 24 years of age, treated between 2012 and 2019 at Erasmus University Medical Center, were included. Healthcare services (consultations, diagnostic and surgical procedures) were counted and costs were calculated. Expected costs based on the treatment protocol were calculated by multiplying healthcare products by the product prices. Correspondingly, the additional expected costs after implementing the ICHOM Standard Set (protocol + ICHOM) were calculated. Observed costs were compared with protocol costs, and the additional expected protocol + ICHOM costs were described. The total mean costs were highest in the first year after birth (€5596), mainly due to surgeries. The mean observed total costs (€40,859) for the complete treatment (0-24 years) were 1.6 times the expected protocol costs (€25,198) due to optional, non-protocolized procedures. Hospital admissions including surgery were the main cost drivers, accounting for 42% of observed costs and 70% of expected protocol costs. Implementing the ICHOM Standard Set increased protocol-based costs by 7%., Competing Interests: Competing interests None., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Routine use of patient-reported outcome measures (PROMs) and computerized adaptive tests (CATs) may improve care in a range of surgical conditions. However, most available CATs are neither condition-specific nor coproduced with patients and lack clinically relevant score interpretation. Recently, a PROM called the CLEFT-Q has been developed for use in the treatment of cleft lip or palate (CL/P), but the assessment burden may be limiting its uptake into clinical practice., Objective: We aimed to develop a CAT for the CLEFT-Q, which could facilitate the uptake of the CLEFT-Q PROM internationally. We aimed to conduct this work with a novel patient-centered approach and make source code available as an open-source framework for CAT development in other surgical conditions., Methods: CATs were developed with the Rasch measurement theory, using full-length CLEFT-Q responses collected during the CLEFT-Q field test (this included 2434 patients across 12 countries). These algorithms were validated in Monte Carlo simulations involving full-length CLEFT-Q responses collected from 536 patients. In these simulations, the CAT algorithms approximated full-length CLEFT-Q scores iteratively, using progressively fewer items from the full-length PROM. Agreement between full-length CLEFT-Q score and CAT score at different assessment lengths was measured using the Pearson correlation coefficient, root-mean-square error (RMSE), and 95% limits of agreement. CAT settings, including the number of items to be included in the final assessments, were determined in a multistakeholder workshop that included patients and health care professionals. A user interface was developed for the platform, and it was prospectively piloted in the United Kingdom and the Netherlands. Interviews were conducted with 6 patients and 4 clinicians to explore end-user experience., Results: The length of all 8 CLEFT-Q scales in the International Consortium for Health Outcomes Measurement (ICHOM) Standard Set combined was reduced from 76 to 59 items, and at this length, CAT assessments reproduced full-length CLEFT-Q scores accurately (with correlations between full-length CLEFT-Q score and CAT score exceeding 0.97, and the RMSE ranging from 2 to 5 out of 100). Workshop stakeholders considered this the optimal balance between accuracy and assessment burden. The platform was perceived to improve clinical communication and facilitate shared decision-making., Conclusions: Our platform is likely to facilitate routine CLEFT-Q uptake, and this may have a positive impact on clinical care. Our free source code enables other researchers to rapidly and economically reproduce this work for other PROMs., (©Conrad Harrison, Inge Apon, Kenny Ardouin, Chris Sidey-Gibbons, Anne Klassen, Stefan Cano, Karen Wong Riff, Andrea Pusic, Sarah Versnel, Maarten Koudstaal, Alexander C Allori, Carolyn Rogers-Vizena, Marc C Swan, Dominic Furniss, Jeremy Rodrigues. Originally published in the Journal of Medical Internet Research (https://www.jmir.org), 27.04.2023.)

Objective: Parents of children with a medical condition and a visible difference can experience challenging situations. We evaluated distress and parenting stress in parents of children with a cleft lip with or without cleft palate (CL±P) or a visible infantile hemangioma (IH)., Setting: This cross-sectional study took place in an academic medical hospital in Rotterdam, the Netherlands., Participants: Three-hundred nine parents (mean age = 40.30, 56.00% mothers) of children with CL±P and 91 parents (mean age = 36.40, 58.24% mothers) of children with IH., Main Outcome Measures: The Dutch version of the Parenting Stress Index - Short Form and the subscales Anxiety, Depression, and Hostility of the Symptom Checklist - 90., Results: One sample t tests and mixed linear modeling were used. On average, parents of children with CL±P and of children with IH showed significantly lower parenting stress compared to normative data. Anxiety was significantly lower in parents of children with CL±P than that in the norm group. Visibility of the condition was not related to distress or parenting stress. Child behavioral problems were positively related to parenting stress, depression, and hostility., Conclusions: Parents of children with CL±P and IH report less distress and parenting stress compared to the norm. On average, these parents seem well adjusted. A practical implication is to monitor parents of children with behavioral problems.

An increased risk of upper airway obstruction (UAO) is seen in up to 95% of patients with facial dysostosis. Secondary to respiratory problems are feeding difficulties and increased nutritional requirements. Little has been described regarding these outcomes in this patient population. Hence, a retrospective cohort study was performed to gather data on functional outcomes. Eighteen patients with facial dysostosis and severe UAO were included. The median follow-up time was 3.42 years. A tracheostomy tube was placed in 13 patients, of whom 10 subsequently underwent mandibular distraction. Three of the five patients without a tracheostomy underwent mandibular distraction as the primary surgical treatment; the remaining two patients were treated conservatively with oxygen supplementation. At presentation, 13 patients had feeding difficulties. Overall malnutrition was present in 16 patients during follow-up. At the end of follow-up, severe UAO was present in 12 patients, feeding difficulties in seven patients, and malnutrition in four patients, while two patients died. In conclusion, patients with facial dysostosis have a high prevalence of severe UAO, feeding difficulties, and malnutrition. Importantly, mandibular distraction has limited success in treating severe UAO in these patients. Close follow-up by a specialized craniofacial team is of paramount importance to manage the long-term consequences., (Copyright © 2020 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Importance: Patients, governments, health care providers, and insurance companies show an increased interest in health outcomes, especially in centralized medical care, such as cleft lip nose treatment. Transparent outcome reporting requires a thorough methodological design, dedicated prospective data collection process, and, preferably, no interference with the efficacy of daily practice., Objective: To describe the implementation of an automated and prospective secondary cleft lip rhinoplasty outcome routine., Design, Setting, and Participants: A prospective analytic cohort pilot study was conducted among 123 consecutive patients referred for secondary cleft lip rhinoplasty from July 1, 2014, to March 31, 2018, at an academic teaching hospital., Exposures: Secondary cleft lip rhinoplasty or revision., Main Outcomes and Measures: Preoperative and 3- and 12-month postoperative scores on the Nasal Obstruction Symptom Evaluation scale (range 0-100, lower scores indicate better outcome), Utrecht Questionnaire (range 0-100, lower scores indicate better outcome), and visual analog scales (range 0-10: 0, no obstruction; 10, completely blocked nose) were obtained. Data were exported for automated statistical outcome analysis that was supported by graphic output on a customized web-based dashboard., Results: Of the 123 patients (68 male and 55 female; mean age, 23 years [range, 17-68 years]) included in the outcome routine, 103 patients (57 male and 46 female; mean age, 22 years [range, 17-50 years]) were eligible for surgery. The web-based dashboard provided demographic characteristics, reasons that surgery was not performed or indicated, and real-time, short- and long-term change in functional and aesthetic outcome after secondary cleft lip rhinoplasty. Among 66 patients with sufficient follow-up, mean (SD) Nasal Obstruction Symptom Evaluation sum scores after rhinoplasty improved from 30.8 (27.6), which is comparable to a moderate problem, to 19.2 (22.2), which is comparable to a very mild problem (P < .001), and mean Utrecht Questionnaire sum scores decreased from 13.1 (5.6) to 7.1 (3.3) (P < .001)., Conclusions and Relevance: Routine prospective outcome monitoring provides an evidence-based response to the increasing demand for transparency in health care. The web-based dashboard used during patient counseling, selection, and management of expectations has the potential to compare results of secondary cleft lip rhinoplasty between surgeons and institutions provided that the populations share similar characteristics. The administrative interference with a busy daily practice was limited., Level of Evidence: 4.

Background: This series describes the results of minimally invasive strip craniotomy with additional spring distraction., Methods: Included are the first 83 consecutive patients who underwent this procedure (January 1, 2010, to January 1, 2014). Outcome parameters were collected prospectively and included surgical parameters and complications, the occurrence of papilledema, skull growth, cephalic index, and photographic scores., Results: Duration of surgery was 63 minutes, 19 percent required blood transfusion, and complications were minor. Postoperative papilledema occurred in two patients (2.4 percent). Head growth increased after insertion of the springs and declined afterward to 0.7 SD, comparable to earlier cohorts in the authors' center. The cephalic index increased from 67 before surgery to 74 after surgery and showed a small decrease during the 4-year follow-up. Photographic scores confirmed the initial improvement and showed a trend to further improvement during follow-up., Conclusions: In this cohort, spring-assisted, minimally invasive strip craniotomy was safe and effective. Results were similar to those from other techniques but with smaller incisions, shorter interventions, reduced blood loss, and a lower incidence of postoperative papilledema., Clinical Question/level of Evidence: Therapeutic, IV.

Background: Due to the increasing incidence of basal cell carcinoma (BCC) and rising health care costs, health care insurance companies seek ways to shift skin surgery for BCC from secondary to primary care., Objectives: To study the differences in complete excision of BCC by general practitioners (GPs), dermatologists, and plastic surgeons., Methods: A retrospective cross-sectional study of pathology records of 2,986 standard excisions of primary BCCs performed by a GP, dermatologist, or plastic surgeon in the area of Southwest Netherlands between 2008 and 2014. To compare the risk of an incomplete BCC excision between the specialties, the odds ratio (OR) was used adjusted for patient age, sex, tumor site, size, and histological subtype., Results: BCCs were completely excised by GPs in 70%, which was lower than the 93% by dermatologists and 83% by plastic surgeons (p < 0.001). Compared to the dermatologist, BCCs which were excised by a GP were 6 times higher at risk of an incomplete excision (adjusted OR 6, 95% CI 5-8) and 2 times higher at risk when excised by a plastic surgeon (adjusted OR 2, 95% CI 2-3)., Conclusion: BCCs were more often completely excised by dermatologists than by GPs and plastic surgeons. Dermatologists probably perform better because of their extensive training and high experience in BCC care. To minimize incomplete BCC excision, GPs should receive specific training before the shift of BCC care from secondary to primary care is justifiable., (© 2018 The Author(s) Published by S. Karger AG, Basel.)

Background: Patients with severe congenital facial disfigurement have a long track record of operations and hospital visits by the time they are 18 years old. The fact that their facial deformity is congenital may have an impact on how satisfied these patients are with their appearance. This study evaluated the level of satisfaction with facial appearance of congenital and of acquired facially disfigured adults, and explored demographic, physical and psychological determinants of this satisfaction. Differences compared with non-disfigured adults were examined., Methods: Fifty-nine adults with a rare facial cleft, 59 adults with a facial deformity traumatically acquired in adulthood, and a reference group of 201 non-disfigured adults completed standardised demographic, physical and psychological questionnaires., Results: The congenital and acquired groups did not differ significantly in the level of satisfaction with facial appearance, but both were significantly less satisfied than the reference group. In facially disfigured adults, level of education, number of affected facial parts and facial function were determinants of the level of satisfaction. High fear of negative appearance evaluation by others (FNAE) and low self-esteem (SE) were strong psychological determinants. Although FNAE was higher in both patient groups, SE was similar in all three groups., Conclusion: Satisfaction with facial appearance of individuals with a congenital or acquired facial deformity is similar and will seldom reach the level of satisfaction of non-disfigured persons. A combination of surgical correction (with attention for facial profile and restoring facial functions) and psychological help (to increase SE and lower FNAE) may improve patient satisfaction., (Copyright 2009 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Unilateral maxillary hypoplasia is a characteristic feature of an oblique facial cleft. This three-dimensional shortage of osseous structures and soft tissue becomes more prominent over the years and is difficult to correct. The authors describe a 17-year-old boy born with a unilateral nasomaxillary dysplasia and nasal dysplasia (Tessier type 1, 2, 3) who underwent a hemi-Le Fort III distraction with a rigid external distraction (RED) system. This distraction, in combination with initial peroperative advancement and retained with elastic traction with a facial mask, achieved a substantial horizontal improvement of the hemi-midface; this resulted in a better projection of the left cheek, infra-orbital rim, nasal ala, and improved occlusion. Owing to the underdevelopment of the maxilla and zygoma in all three dimensions, contour deformities remain. Creating several bone segments for multidirectional distraction would jeopardize vascularization of the bone. Good long-term planning is essential in these complicated cases, and more long-term results need to be assessed. The major reconstructive challenge for this complex pathology continues.