Importance:The risk of increased intracranial pressure (ICP) in metopic synostosis remains poorly defined, leading to uncertainty about the need for routine surgical intervention vs conservative management. Objective:To compare head growth trajectories and the prevalence of signs of increased ICP among patients with metopic synostosis managed surgically or conservatively. Design, Setting, and Participants:This prospective cohort study included all 209 new patients with single-suture metopic synostosis presenting to Erasmus Medical Center, Rotterdam, the Netherlands, from January 1, 2017, to December 31, 2024. The current analysis included data collection up to July 31, 2025. Patients underwent annual follow-up with head circumference measurement and fundoscopy. Intervention:Surgical or conservative management of metopic synostosis determined through shared decision-making between clinicians and parents. Main Outcome and Measures:The primary outcome was longitudinal head circumference standard deviation (SD), analyzed using linear mixed models. Secondary outcomes were head growth deflection and papilledema, understood as signs of increased ICP, detected on fundoscopy. Results:Among 209 patients (78 surgical and 131 conservative; median age at presentation, 4 months [IQR, 2-7 months]; 154 boys [74%]), the median age at last follow-up was 40 months (IQR, 24-61 months). Head circumference SD increased significantly with age in a nonlinear pattern (age spline df = 1; β = 0.87; 95% CI, 0.54-1.21; P < .001; and age spline df = 2; β = 0.73; 95% CI, 0.29-1.16; P < .001). Female sex was associated with higher SD (β = 0.31; 95% CI, 0.03-0.60; P = .03). Surgical treatment was associated with higher initial SD but a significantly flatter growth trajectory over time compared with conservative treatment (β = -1.02; 95% CI, -1.52 to -0.52; P < .001 for interaction age spline and treatment). Severe deformity phenotype was associated with lower SD values compared with mild deformity phenotype (β = -0.39; 95% CI, -0.75 to -0.04; P = .03). Among 158 patients with 2 or more years of follow-up, head growth deflection occurred among 3 patients (1.9%): 2 of 65 (3.1%) in the surgical group and 1 of 93 (1.1%) in the conservative group (P = .57). Papilledema was observed in 3 patients (1.4%): 2 of 78 (2.6%) in the surgical group and 1 of 131 (0.8%) in the conservative group (P = .56). None of these patients required additional surgery. Conclusions and Relevance:In this prospective cohort of patients with metopic synostosis, signs of elevated ICP were rare and did not differ between surgical and conservative groups. Although surgery altered head growth trajectories, the low prevalence of ICP-related findings supports close monitoring and selective surgical intervention as safe alternatives to routine surgery.

OBJECTIVE:Pressure injuries (PIs) are a major health care issue, and timely and correct repositioning of patients at risk for PIs is crucial in lowering the PI risk. However, the ideal bed positions and the impact on the PI risk are often unclear to health care staff. Therefore, the authors used a pressure-detecting mat to quantify and visualize the effects of bed settings and body position on the pressure distribution to provide insightful graphics for educating health care staff. METHODS:The authors quantitatively assessed and visualized the pressure distribution of different in-bed lying and sitting positions with a pressure-measuring mat on 2 different hospital mattresses by including healthy volunteers. RESULTS:Differences between positions were visualized by the pressure mat. The median pressure on the sacrum increased by 30% to 35% when the head of bed (HOB) angle was raised from 0° to 30° and further increased 17% to 27% when the HOB angle was raised from 30° to 45°. Pressure on sacrum, when sitting with an HOB angle of 30°, reduced by 19% to 35% when raising the legs to semi-Fowler position. During lateral rotation, median pressure on the greater trochanter increased by 27% to 35% when rotating from 30° to 60°. Pressures are reported in a range between the 2 different mattresses. CONCLUSIONS:Pressure maps and peak pressure values of the different bed positions can help educate nurses on how body position changes the pressure distribution and provides additional support to follow the international guideline on PI prevention.

Background: Congenital craniofacial conditions are a diverse group of rare and complex disorders, leading to significant functional and psychosocial challenges. Their rarity and variability hinder research and clinical standardisation, resulting in substantial practice variation across centres. International collaboration and high-quality evidence are therefore essential to improve and converge care for affected individuals. This article presents the discussion section of a doctoral thesis that addressed these challenges through multiple methodological approaches, the majority of which were conducted within the European Reference Network for Rare and Complex Craniofacial Conditions (ERN CRANIO). Methods: The studies combined systematic reviews, multicentre cohort analyses, expert consensus and registry development to advance understanding and care for three conditions: unilateral cleft lip and palate (UCLP), Treacher Collins syndrome and Miller syndrome. Results: The thesis provides evidence supporting early hard palate closure in UCLP, contributing to improved speech outcomes and fewer oronasal fistulas without increasing surgical burden. For facial dysostosis syndromes, the European clinical consensus statement and the establishment of the first international registry provide a foundation for more standardised multidisciplinary care and collaborative research. The systematic review on Miller syndrome clarifies its phenotypic spectrum and aids differential diagnosis with other syndromes. Conclusions: Through its comprehensive and collaborative approach, this thesis promotes the convergence of care for congenital craniofacial conditions in Europe. It highlights the importance of methodological rigour, standardisation and international collaboration to achieve evidence-based and equitable care for these rare and complex disorders.

Nasal deviations have been observed in patients following midface surgery. Therefore, the purpose of this study was to evaluate the development of clinically visible nasal deviations following midface surgery and to assess the natural course of the nose over time. This retrospective study included all Apert and Crouzon patients who underwent midface surgery (Le Fort III (LF3), monobloc (MB), or facial bipartition (FB)). Clinical nasal deviation was assessed on preoperative, short-term postoperative (≤ 1 year), and long-term postoperative (> 1 year) 2D facial photographs by consensus of pediatric ENT surgeons and one craniofacial surgeon. Additionally, pre- and postoperative CT scans were reviewed when available. Sixty-eight procedures were included (27 Apert, 41 Crouzon); 34 LF3, 31 MB, and 3 FB. The median age at surgery was 10.2 years. Twenty-five (37%) patients were found to have clinically worsening nasal asymmetry, of whom 4 (16%) had pre-existing deviation preoperatively. Seventeen patients with worsening deviation had long-term postoperative facial photographs available (median time 5.8 years), and in 9 (53%), the postoperative clinical nasal deviation appeared to improve spontaneously. We were unable to identify predictive factors for postoperative nasal deviation. In 16% of the patients, a clinically deviated nose was observed preoperatively. Nasal deviation worsened in 37% of the patients after midface surgery, but also spontaneously improved in 53% of these patients over the long-term. Since predicting the occurrence of nasal deviation and self-correction is difficult, the possibility of developing nasal deviation should be discussed with the patient and their parents.

OBJECTIVE:Pressure injuries (PIs) are a burden because they cause pain, prolong hospital stays, and increase mortality. Especially for individuals who are bedridden, the support surface is one of the main factors influencing the PI risk. Therefore, we quantitatively assessed the interface pressures of four hospital mattresses aiming to reduce the risk of PI of our patients by an optimized mattress selection. METHODS:We assessed the pressure distribution and median peak pressure on the sacrum and trochanter in supine, semi-fowler and lateral position with a pressure-measuring mat on four different mattresses using eight healthy subjects. RESULTS:The observed pressure distribution varied significantly between mattresses. Despite a subject and body position dependent peak pressure, the relative performance of the mattresses was consistent across subjects. In the Semi-fowler position, median peak pressure was lowest on the Powered_FIS mattress (25.7 mmHg), followed by Non-powered_foam (35.6 mmHg), Powered_air (43.5 mmHg) in continuous mode, and highest on the Non-powered_hybrid mattress (55.5 mmHg). CONCLUSION:Pressure measurements can be used as an objective tool that can assist the process of selecting a mattress. Extra attention should be focused on assessing hybrid mattresses as the pressure distribution can be considerably worse in the reactive state when compared to a dedicated reactive mattress.

Study design: Systematic review. Objective: Obstructive sleep apnea (OSA) is a possible complication following secondary velopharyngeal insufficiency surgery in patients with repaired cleft palate. Various surgical techniques are used to treat secondary velopharyngeal insufficiency after cleft palate repair, but the optimal procedure remains debatable. This review provides an overview of the incidence of airway obstructive outcomes related to different surgical modalities. Methods: A systematic search was performed on the 1st of February following the PRISMA guidelines and registered on PROSPERO (CRD42022299715). The following databases were reviewed: Medline, EMBASE, Web of Science, Google Scholar, and the Cochrane Library databases. Studies that included data on the occurrence of OSA following velopharyngeal surgery in children with a repaired non-syndromic cleft palate were included. Non-English articles and studies that included syndromic cleft palate patients were excluded. Results: Twenty-eight articles met the inclusion criteria. The surgical procedures are classified into three groups: pharyngeal flap procedure (PF), sphincter pharyngoplasty (SP), and palatal muscle repositioning (PMR). Incidence of post-operative OSA and symptoms of OSA were lowest after PMR compared to SP and PF (3%; 34%; 29%, respectively). Pharyngeal flap procedures resulted in the best speech outcomes. Conclusions: PMR results in fewer postoperative complications in terms of OSA and achieves a satisfactory reduction in hypernasal speech. PF procedure carries a higher risk of developing OSA postoperatively but seems to be superior in the reduction in hypernasality.

Miller syndrome is an extremely rare condition in the group of facial dysostosis syndromes. These syndromes have great phenotypic overlap and variability, even within families. To facilitate the differentiation of Miller syndrome from related facial dysostosis syndromes, such as Treacher Collins and Nager syndrome, this study aims to provide an overview of the phenotypic spectrum of the syndrome. A systematic literature search of Embase, MEDLINE/PubMed, Web of Science, and CINAHL was conducted until November 2024. Case reports and case series of patients with a clinical or genetic diagnosis of Miller syndrome in all languages were included. The quality of the included reports was assessed using the Joanna Briggs Institute critical appraisal tool and the Fichas de Lectura Critica 3.0 web application. In total, 44 cases of Miller syndrome were found, with only 18.2% having genetic confirmation. Craniofacial anomalies were prominent, including midface hypoplasia (72.7%) and micrognathia (75.0%), orofacial clefts (77.3%), eyelid anomalies (70.5%), and external ear anomalies (63.6%). Limb anomalies were present in all cases, primarily involving the hands (95.5%), forearms (52.3%), and feet (90.9%). Anomalies in other extracraniofacial tracts were also reported. Despite limitations, including limited genetic confirmation and reliance on literature, this study provides valuable insights into the phenotypic spectrum of Miller syndrome. Efforts for genetic confirmation, international collaboration, and comprehensive reporting are essential to advance research and care for rare conditions like Miller syndrome. Therefore, a detailed checklist for phenotypic evaluation in Miller syndrome cases is provided in this study.

Facial dysostosis syndromes (FDS) are rare congenital conditions that significantly impact facial function and appearance. At the time of this writing, standardised monitoring protocols for FDS are lacking, hampering research, and evidence-based care. Thus, a comprehensive dataset was developed within the European Reference Network for Rare and Complex Craniofacial Anomalies (ERN CRANIO). Candidate data elements were identified through a systematic literature review (1985-2024) and supplemented with elements from existing ERN CRANIO datasets and expert panel suggestions. A Delphi survey was then conducted among 61 clinicians and 3 patient representatives to assess each element's relevance and reliability using a 9-point Likert scale. A subsequent hybrid consensus meeting with the expert panel shaped the final dataset, ensuring comprehensive coverage, avoiding overlap, and determining the appropriate timing for data collection. Of 200 data elements that entered the Delphi voting, 98 were strongly recommended, 102 scored neutral, and none were strongly discouraged. Ultimately, 110 elements were included, organised into 2 levels: Level 1, comprising exclusively patient-reported and parent-reported outcome measures; and Level 2, encompassing patient characteristics, treatment information, clinical outcomes, and imaging/diagnostics. This newly developed dataset marks the first international registry for FDS, offering considerable potential for collaborative research, cross-centre comparisons, and substantial improvements in care for patients with FDS worldwide. Real-world implementation will be essential to evaluate its feasibility and guide further refinements.

BACKGROUND:The CLEFT-Q, a questionnaire developed and validated specifically for cleft patients, contains seven appearance scales. The International Consortium of Health Outcomes Measurement (ICHOM) has incorporated only some CLEFT-Q appearance scales in the Standard Set to minimize burden. This study evaluates which appearance scales provide the most meaningful information in the different cleft types at specific ages, for the most efficient cleft appearance outcome assessment.METHODS:Within this international multicenter study, outcomes of the seven appearance scales were collected, either as part of the ICHOM Standard Set, or as part of the field test study performed to validate the CLEFT-Q. Analyses were performed in separate age groups and cleft types, and involved univariate regression analyses, trend analyses, t tests, correlations, and floor and ceiling effects.RESULTS:A total of 3116 patients were included. Scores for most appearance scales showed a downward trend by age group, with the exception of the Teeth and Jaw scales. In all cleft types, several scales correlated strongly with each other. No floor effects were observed, but ceiling effects were found in several scales in different age groups, most often in the CLEFT-Q Jaw scale.CONCLUSIONS:A proposition for the most meaningful and efficient appearance outcome assessment in cleft patients is made. It was composed so that recommendations are of value for different cleft protocols and initiatives. Suggestions for the use of scales in the ICHOM Standard Set at different ages are given, and also from a clinical perspective. Use of the CLEFT-Q Scar, Lips, and Nose scales will provide additional relevant information.

Background:The CLEFT-Q is a questionnaire developed for patients with a cleft lip and/or palate (CL/P). Numerous scales have been implemented as part of the ICHOM Standard Set for CL/P. Although validated for completion by patients only, clinicians noted that caregivers are often involved in completion of the scales. Aim of the study was to promote further standardization of Patient Reported Outcome Measures (PROMs) in pediatric patients by examining the preferences of patients and parents concerning the reporter type. Moreover, possible discrepancies in outcomes between reporter types were explored. Methods: Data from 567 patients with CL/P and their caregivers that completed scales of the CLEFT-Q questionnaire were collected. Reporter group sizes and proportions were examined at the ages of 8, 12, and 15 years to determine the preferred manner of completion. Mean outcomes were analyzed per scale at the 3 ages, and compared between the 3 reporter groups: “patient,” “caregiver,” and “together.” Results: In all age-groups, the majority completed the PROMs together. Concerning the reporter types per age-group, an upward trend was seen in the proportion of patients that completed the scales alone. In the caregiver group, a downward trend was observed, and the highest proportion of parents that completed the scales was found at age 8. No significant differences were found between the reporter types in any of the scales. Conclusion: Even if a PROM questionnaire is validated for patient report only, it is recommended to record the reporter type when a pediatric PROM is completed. In order to capture outcomes that represent the patient’s voice validly and reliably, though with support of the caregiver, a pediatric PROM should be filled out by the patient alone and thereafter evaluated with the caregiver(s). Concerning the CLEFT-Q, there seems to be demand for a validated parent-version of the scales.

Introduction: Pressure Ulcers (PUs) are a major healthcare issue leading to prolonged hospital stays and decreased quality of life. Monitoring body position changes using sensors could reduce workload, improve turn compliance and decrease PU incidence. Method: This systematic review assessed the clinical applicability of different sensor types capable of in -bed body position detection. Results: We included 39 articles. Inertial sensors were most commonly used (n = 14). This sensor type has high accuracy and is equipped with a 2-4 hour turn -interval warning system increasing turn compliance. The secondlargest group were piezoresistive (pressure) sensors (n = 12), followed by load sensors (n = 4), piezoelectric sensors (n = 3), radio wave -based sensors (n = 3) and capacitive sensors (n = 3). All sensor types except inertial sensors showed a large variety in the type and number of detected body positions. However, clinically relevant position changes such as trunk rotation and head of bed elevation were not detected or tested. Conclusion: Inertial sensors are the benchmark sensor type regarding accuracy and clinical applicability but these sensors have direct patient contact and (re)applying the sensors requires the effort of a nurse. Other sensor types without these disadvantages should be further investigated and developed. We propose the Pressure Ulcer Position System (PUPS) guideline to facilitate this.

The European Reference Network for Rare Craniofacial Aanomalies and Ear-Nose-Throat disorders aims to improve care for patients with such afflictions, including cleft lip and palate (CL/P) across Europe. Cleft treatment remains varied throughout European centers, inhibiting meaningful comparison of treatment outcomes. To overcome these issues, a European-wide common CL/P dataset and registry was developed, facilitating standardized treatment endpoints and outcome measures for international comparison and benchmarking of CL/P centers. Questionnaires and semi-structured interviews were used to determine the set-up of the registry. Previous CL/P initiatives were analyzed to create an initial dataset, refined through consensus meetings. In total, 87 cleft specialists working in specialized CL/P centers from 16 European nations participated. Consensus on a common dataset was reached. A "Level 1" dataset, with mandatory clinical and patient-reported outcome measures, and "Level 2" dataset with additional outcome measures. Finally, 2 dashboards were developed for data dissemination. The development of the European CL/P common dataset and registry tackled challenges with resource disparities, variations in specialists within CL/P teams, regulatory differences in patient data usage, patient-reported outcome measures availability in European languages, and use of assessment tools. This study described the successful development of the European Reference Network for Rare Craniofacial Aanomalies and Ear-Nose-Throat disorders CL/P common dataset and registry. This achievement will help improve patient care and outcomes for patients with CL/P in Europe. Furthermore, this study provides useful information for initiatives with similar aims.

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.

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.

Patient-Reported Outcome Measures (PROMs), such as the CLEFT-Q, have become essential for outcomes in patients with CL/P. Normative values of the CLEFT-Q for non-CL/P peers have not yet been established. This study aims to establish normative values for the CLEFT-Q in the general Dutch population. Dutch nationals aged 16-24 years without CL/P were recruited through an online survey. Participants completed the CLEFT-Q, excluding the lip scar and eating and drinking scales. Data were weighted based on the Dutch Central Bureau of Statistics. Normative values were calculated as means and standard deviations, stratified by sex and education category. Tobit regression models were used to analyze associations between CLEFT-Q scores and demographic variables. In total, 870 participants responded, of which 160 were excluded due to potential craniofacial anomalies. Significant variations in CLEFT-Q scores based on sex were found, with females scoring lower than males. Level of education had a modest impact on CLEFT-Q scores, with lower education having lower scores on certain scales. Age marginally influenced CLEFT-Q scores, with younger participants scoring lower than older participants. Positive correlations were found between all CLEFT-Q scales. The strongest correlation was observed between the social and school functioning scales. This study provides the first set of normative values for the CLEFT-Q in the Dutch general population. Significant differences in CLEFT-Q scores based on sex, level of education and age were found. These normative values are useful for clinicians interpreting CLEFT-Q scores and help make informed decisions.

Background:The aim of this study was to describe and compare head shape in surgically and conservatively treated patients with isolated metopic synostosis using 3-dimensional photogrammetry.Methods:A retrospective review (2017 through 2020) of consecutive patients, age 4 years, with isolated metopic synostosis based on 3-dimensional photogrammetry was conducted. Images were aligned using a template based on healthy age-matched controls, and mean head shapes were reconstructed to evaluate shape development. A comparative subanalysis based on phenotype was performed between patients who were treated surgically and those who were treated conservatively.Results:A total of 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, whereas the conservative group showed a slight frontal prominence. Both groups showed temporal depression with respect to the controls. In the subanalysis, a similar degree of temporal depression was observed between surgical and conservative treatment. Head shape patterns showed considerable similarity across all severity phenotypes.Conclusions:This study shows a deviation in forehead shape from normal controls in patients with metopic synostosis following both surgical and conservative treatment by age 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.CLINICAL QUESTION/LEVEL OF EVIDENCE:Therapeutic, III.

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.

(1) Background: Orbital and midface malformations occur in multiple craniofacial disorders. Depending on the deformity, surgical corrections include orbital box osteotomy (OBO), Le Fort III (LFIII), monobloc (MB), and facial bipartition (FB). The aim of this study was to determine the effect of these procedures on ocular outcomes. (2) Methods: A retrospective analysis was performed. All patients with craniofacial disorders who had previously undergone midface surgery were included. The Wilcoxon signed ranks test was used for statistical analysis. (3) Results: In total, 63 patients were included: two patients were treated by OBO, 20 by LFIII, 26 by MB, and 15 by FB. Pre-operatively, strabismus was present in 39 patients (61.9%), in whom exotropia was most common (n = 27; 42.9%), followed by esotropia (n = 11; 17.5%). Postoperatively, strabismus significantly worsened (p = 0.035) in the overall population (n = 63). Pre-operative binocular vision (n = 33) was absent in nine patients (27.3%), poor in eight (24.2%), moderate in 15 (45.5%), and good in one (3.0%). Postoperatively, binocular vision significantly improved (p < 0.001). Before surgery, the mean visual acuity (VA) in the better eye was 0.16 LogMAR (Logarithm of the Minimum Angle of Resolution), and 0.31 LogMAR in the worse eye. Furthermore, pre-operative astigmatism was present in 46 patients (73.0%) and hypermetropia in 37 patients (58.7%). No statistical difference was found for VA (n = 51; p = 0.058) postoperatively. (4) Conclusions: Midface surgery has a direct and indirect substantial effect on several ocular outcomes. This study emphasizes the importance of appropriate ophthalmological evaluation in patients with craniofacial disorders undergoing midface surgery.

Background Headaches are the most common complaints among pediatric populations. Determining the cause and appropriate treatment for headaches may be challenging and costly, and the impact of headaches on the lives of patients and their families is not well understood. Objective A systematic literature review was conducted to examine what PROMs are currently used, and to identify quality of life (QoL) concepts important to children suffering from headaches and any known determinants of QoL. Methods Embase, Medline, Web of Science, CINAHL, EBSCOhost, PsychINFO, Cochrane CENTRAL and Google Scholar were searched from their inception through to June 2021. Studies investigating QoL, using a validated outcome measure in pediatric patients with headaches, were included. Relevant studies were identified through title and abstract screening and full text review by two independent reviewers. A citation review of included studies was performed. QoL concepts were extracted from the outcome measures that were used in each study to develop a preliminary conceptual model of QoL in children suffering from headaches. Determinants of QoL were also identified and categorized. Results A total of 5421 studies were identified in the search. Title and abstract screening resulted in the exclusion of 5006 studies. Among the 415 studies included for full text review, 56 were eligible for final analysis. A citation review resulted in the addition of five studies. Most studies were conducted in high-income countries and included a patient-sample accordingly ( n = 45 studies). Sixteen different PROMs were identified in the included studies, of which the PedsQL was used the most often ( n = 38 studies). The most common health concepts reported were physical functioning ( n = 113 items), social and psychological wellbeing ( N = 117, n = 91 resp.). Twenty-five unique determinants of QoL were extracted from the included studies. Conclusion There is a need for a condition-specific PROM to facilitate the measurement of QoL outcomes in the pediatric headache population. A conceptual model was developed based on the findings from the health concepts. Findings from this review could be used for future qualitative interviews with pediatric patients with headaches to elicit and refine important QoL concepts.

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%.