The Development of a European Multidisciplinary Cleft Lip and Palate Registry by the European Reference Network CRANIO: Experiences, Barriers, And Facilitators.

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.

The Development, Deployment, and Evaluation of the CLEFT-Q Computerized Adaptive Test: A Multimethods Approach Contributing to Personalized, Person-Centered Health Assessments in Plastic Surgery.

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.

Prenatal Ultrasound Parameters of Twins With Sagittal Suture Craniosynostosis Question Mechanical Constraint as the Leading Cause.

BACKGROUND: Multiple pregnancy is a known predisposing factor for sagittal suture synostosis, and intrauterine constraint has been suggested as a potential cause. This study evaluates prenatal images of single and multiple pregnancies of children with sagittal suture synostosis to assess the possible pathogenesis. MATERIALS AND METHODS: Prenatal ultrasound data of scaphocephaly twins treated at Erasmus MC was retrieved retrospectively. The head circumference, cephalic index (CI), and biparietal diameter (BPD) were analyzed. Data were compared with the nonaffected sibling, singletons with scaphocephaly, and to general fetal growth reference values. RESULTS: Data of 8 twin pregnancies with 10 cases of scaphocephaly was gathered. For the head circumference no difference was found between affected fetuses and reference values throughout pregnancy. For the BPD a reduced growth was found, compared with control values. The growth curve for the CI of the affected twins is lower from the start and declines over time. When comparing affected fetuses to nonaffected fetuses, the same differences for the BPD and CI were found as compared with controls. No differences in growth parameters were found between the nonaffected siblings and reference values, nor between the affected scaphocephaly twins and singletons with scaphocephaly. CONCLUSIONS: Scaphocephaly in twin pregnancy develops early on in pregnancy, similar to singleton pregnancy, making the theory of constraint as a cause less likely. The development of scaphocephaly in twins is likely to be attributed to the same disturbed developmental processes in suture development as in singletons, and not to mechanical constraint.

Barriers and Facilitators to the International Implementation of Standardized Outcome Measures in Clinical Cleft Practice.

OBJECTIVE: To identify barriers and facilitators to international implementation of a prospective system for standardized outcomes measurement in cleft care. DESIGN: Cleft teams that have implemented the International Consortium for Health Outcomes Measurement Standard Set for cleft care were invited to participate in this 2-part qualitative study: (1) an exploratory survey among clinicians, health information technology professionals, and project coordinators, and (2) semistructured interviews of project leads. Thematic content analysis was performed, with organization of themes according to the dimensions of the reach, effectiveness, adoption, implementation and maintenance (RE-AIM) framework: reach, effectiveness, adoption, implementation, and maintenance. RESULTS: Four cleft teams in Europe and North America participated in this study. Thirteen participants completed exploratory questionnaires and 5 interviewees participated in follow-up interviews. Survey responses and thematic content analysis revealed common facilitators and barriers to implementation at all sites. Teams reach patients either via email or during the clinic visit to capture patient-reported outcomes. Adopting routine data collection is enhanced by aligning priorities at the organizational and cleft team level. Streamlining workflows and developing an efficient data collection platform are necessary early on, followed by pilot testing or stepwise implementation. Regular meetings and financial resources are crucial for implementing, sustaining, analyzing collected data, and providing feedback to health care professionals and patients. Fostering patient-centered care was articulated as a positive outcome, whereas time presented challenges across all RE-AIM dimensions. CONCLUSIONS: Identified themes can inform ongoing implementation efforts. Intentionally investing time to lay a sound foundation early on will benefit every phase of implementation and help overcome barriers such as lack of support or motivation.

Emotional and Behavioral Problems in Children With a Cleft Lip With or Without Palate or an Infantile Hemangioma.

OBJECTIVE: Life can be challenging for children with a visible difference due to a medical condition, and they might be at risk for emotional and behavioral problems. This study examines emotional and behavioral problems in children with a cleft lip with or without palate (CL ± P) or an infantile hemangioma (IH) in relation to the visibility of the condition, the presence of additional condition-related problems, and parental affect. SETTING: This cross-sectional study took place in an academic medical hospital in the Erasmus MC Sophia Children's Hospital, the Netherlands. PARTICIPANTS: A total of 309 parents (mean age = 40.34, 44.00% male) of 182 children with CL ± P and 48 parents (mean age = 39.21, 37.50% male) of 33 children with an IH completed questionnaires. Children were 1.5 to 12 years old. RESULTS: Parents reported fewer child emotional and behavioral problems compared to normative data. Problems reported were mainly related to learning difficulties and parent gender, while visibility of the condition had no significant influence. Parental negative affect was related to child internalizing problems. Parental positive affect was not related to any of the outcome measures. CONCLUSIONS: Parents reported fewer problems for their children compared to normative data. This is inconsistent with previous research, showing similar or worse scores for these children compared to peers. Our findings may be explained by a protective parenting style, a response shift in parents, or problems developing at a later point in life.

Rasch Analysis of Patient- and Parent-Reported Outcome Measures in the International Consortium for Health Outcomes Measurement Standard Set for Cleft Lip and Palate.

OBJECTIVES: The aim of this study was to evaluate the psychometric performance of the patient- and parent-reported measures in the International Consortium for Health Outcomes Measurement (ICHOM) Standard Set for Cleft Care, and to identify ways of improving concept coverage. METHODS: Data from 714 patients with cleft lip and/or palate, aged 8 to 9, 10 to 12.5, and 22 years were collected between November 2015 and April 2019 at Erasmus University Medical Center, Boston Children's Hospital, Duke Children's Hospital, and from participating sites in the CLEFT-Q Phase 3 study. The Standard Set includes 9 CLEFT-Q scales, the Nasal Obstruction Symptom Evaluation (NOSE) questionnaire, the Child Oral Health Impact Profile-Oral Symptoms Scale (COHIP-OSS), and the Intelligibility in Context Scale (ICS). Targeting, item-fit statistics, thresholds for item responses, and measurement precision (PSI) were analyzed using Rasch measurement theory. RESULTS: The proportion of the sample to score within each instruments range of measurement varied from 69% (ICS) to 92% (CLEFT-Q teeth and COHIP-OSS). Specific problems with individual items within the NOSE and COHIP-OSS questionnaires were noted, such as poor item fit to the Rasch model and disordered thresholds (6 of 10). Reliability measured with PSI was above 0.82 for the ICS and all but one CLEFT-Q scale (speech distress). PSIs were lowest for the COHIP-OSS (0.43) and NOSE questionnaire (0.35). CONCLUSION: The patient- and parent-reported components within the facial appearance, psychosocial function, and speech domains are valid measures; however, the facial function and oral health domains are not sufficiently covered by the CLEFT-Q eating and drinking, NOSE, and COHIP-OSS, and these questionnaires may not be accurate enough to stratify cleft-related outcomes.

Saethre-Chotzen syndrome: long-term outcome of a syndrome-specific management protocol.

AIM: To assess the long-term outcomes of our management protocol for Saethre-Chotzen syndrome, which includes one-stage fronto-orbital advancement. METHOD: All patients born with Saethre-Chotzen syndrome between January 1992 and March 2017 were included. Evaluated parameters included occipital frontal head circumference (OFC), fundoscopy, neuroimaging (ventricular size, tonsillar position, and the presence of collaterals/an abnormal transverse sinus), polysomnography, and ophthalmological outcomes. The relationship between papilledema and its associated risk factors was evaluated with Fisher's exact test. RESULTS: Thirty-two patients (21 females, 11 males) were included. Median (SD) age at first surgery was 9.6 months (3.1mo) for patients who were primarily referred to our center (range: 3.6-13.0mo), the median (SD) age at last follow-up was 13 years (5y 7mo; range: 3-25y). Seven patients had papilledema preoperatively, which recurred in two. Two patients had papilledema solely after first surgery. Second cranial vault expansion was indicated in 20%. Thirteen patients had an OFC deflection, indicating restricted skull growth, one patient had ventriculomegaly, and none developed hydrocephalus. Eleven patients had emissary veins, while the transverse sinus was aberrant unilaterally in 13 (hypoplastic n=10 and absent n=3). Four patients had mild tonsillar descent, one of which was a Chiari type I malformation. Four patients had obstructive sleep apnoea (two mild, one moderate, and one severe). An aberrant transverse sinus was associated with papilledema (p=0.01). INTERPRETATION: Single one-stage fronto-orbital advancement was sufficient to prevent intracranial hypertension for 80% of our patients with Saethre-Chotzen syndrome. Follow-up should focus on OFC deflection and venous anomalies.

Disappointing results of spring-assisted cranial vault expansion in patients with Crouzon syndrome presenting with sagittal synostosis.

The aim of this study was to report on a single center's experience with spring-assisted cranial vault expansion (SAE) in patients with Crouzon syndrome and sagittal suture synostosis. Strip craniotomy with SAE has resulted in successful outcomes with low complication and revision rates in patients with isolated scaphocephaly. However, recent experience suggests that outcomes in patients with Crouzon syndrome and sagittal synostosis (SS) who undergo SAE are less favorable compared with the outcomes of those who undergo frontobiparietal (FBP) expansion. The authors reviewed both operations performed at a single center and noticed an upward expansion of the skull, which may be related to ventriculomegaly, with concurrent intracranial hypertension and poor aesthetic outcome. All patients diagnosed with Crouzon syndrome and SS who were treated with SAE required a revision FBP operation. Based on this outcome, the authors consider Crouzon syndrome a contraindication for correcting SS with springs.

Posterior Distraction Using Springs in Syndromic and Multisuture Craniosynostosis: Improving the Technique.

INTRODUCTION: Posterior distraction is the preferred surgical treatment for particularly Apert and Crouzon syndrome in most craniofacial centers, using either external distractors or springs. The authors prefer the use of springs and have adapted their technique to further improve outcomes. METHODS: All patients who were treated with the adapted technique for occipital expansion using springs were included. The most significant adaption that the authors introduced in 2017 is using a bony hinge at the top of the vault instead of at the caudal edge of the occiput. RESULTS: A total of 8 posterior expansions with springs were performed. No complications occurred and the springs were also successfully applied in cases with extremely thin bone. If indicated, a simultaneous foramen magnum decompression was performed and this was easier to combine with a hinge at the top of the vault. CONCLUSIONS: Posterior distraction with springs is a safe and effective procedure and allows a simultaneous foramen magnum decompression. Planning the hinge at the vault allows intracranial volume gain at the site of the posterior skull base.

Differences in Rate of Complete Excision of Basal Cell Carcinoma by Dermatologists, Plastic Surgeons and General Practitioners: A Large Cross-Sectional Study.

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.

The clinical applicability of sensor technology with body position detection to combat pressure ulcers in bedridden patients.

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 second-largest 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.

Most Efficient and Meaningful Patient-Reported Appearance Assessment in Different Cleft Types and Age Groups with CLEFT-Q.

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.

Effect of Midface Surgery on Ocular Outcomes in Patients with Orbital and Midface Malformations.

(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.

Optimizing the Psychosocial Function Measures in the International Consortium for Health Outcomes Measurement Standard Set for Cleft.

BACKGROUND: To ensure the feasibility of implementing PROMs in clinical practice, they must be continually appraised for undue burden placed on patients and clinicians and their usefulness for decision-making. This study assesses correlations between the CLEFT-Q psychosocial scales in the International Consortium for Health Outcomes Measurement Standard Set for cleft and explores their associations with patient characteristics and psychosocial care referral. METHODS: Spearman correlation coefficients were calculated for CLEFT-Q psychological function, social function, school function, face, speech function, and speech-related distress scales. Logistic regressions were used to assess the association of cleft phenotype, syndrome, sex, and adoption status on scale scores and clinical referral to psychosocial care for further evaluation and management. RESULTS: Data were obtained from 3067 patients with cleft lip and/or palate at three centers. Strong correlations were observed between social function and psychological function (r > 0.69) and school function (r > 0.78) scales. Correlation between school function and psychological function scales was lower (r = 0.59 to 0.68). Genetic syndrome (OR, 2.37; 95% CI, 1.04 to 5.41), psychological function (OR, 0.92; 95% CI, 0.88 to 0.97), school function (OR, 0.94; 95% CI, 0.90 to 0.98), and face (OR, 0.96; 95% CI, 0.94 to 0.98) were significant predictors for referral to psychosocial care. CONCLUSIONS: Because social function as measured by the CLEFT-Q showed strong correlations with both school and psychological function, its additional value for measuring psychosocial function within the Standard Set is limited, and it is reasonable to consider removing this scale from the International Consortium for Health Outcomes Measurement Standard Set for cleft.

What is the optimal assessment of speech? A multicentre, international evaluation of speech assessment in 2500 patients with a cleft.

OBJECTIVES: Speech problems in patients with a cleft palate are often complex and multifactorial. Finding the optimal way of monitoring these problems is challenging. The International Consortium of Health Outcomes Measurement (ICHOM) has developed a set of standardised outcome measures at specific ages for patients with a cleft lip and/or palate, including measures of speech assessment. This study evaluates the type and timing of speech outcome measures currently included in this ICHOM Standard Set. Additionally, speech assessments in other cleft protocols and initiatives are discussed. DESIGN, SETTING AND PARTICIPANTS: An international, multicentre study was set up including centres from the USA and the Netherlands. Outcomes of clinical measures and Patient Reported Outcome Measures (PROMs) were collected retrospectively according to the ICHOM set. PROM data from a field test of the CLEFT-Q, a questionnaire developed and validated for patients with a cleft, were collected, including participants from countries with all sorts of income statuses, to examine the value of additional moments of measurement that are used in other cleft initiatives.Data from 2500 patients were included. Measured outcomes contained univariate regression analyses, trend analyses, t-tests, correlations and floor and ceiling effects. RESULTS: PROMs correlated low to moderate with clinical outcome measures. Clinical outcome measures correlated low to moderate with each other too. In contrast, two CLEFT-Q Scales correlated strongly with each other. All PROMs and the Percent Consonants Correct (PCC) showed an effect of age. In patients with an isolated cleft palate, a ceiling effect was found in the Intelligibility in Context Scale. CONCLUSION: Recommendations for an optimal speech outcome assessment in cleft patients are made. Measurement moments of different cleft protocols and initiatives are considered in this proposition. Concerning the type of measures, adjustment of the current PCC score outcome seems appropriate. For centres with adequate resources and specific interest in research, translation and validation of an upcoming tool, the Cleft Audit Protocol for Speech Augmented, is recommended.

Frontorhiny, a distinctive presentation of frontonasal dysplasia caused by recessive mutations in the ALX3 homeobox gene.

We describe a recessively inherited frontonasal malformation characterized by a distinctive facial appearance, with hypertelorism, wide nasal bridge, short nasal ridge, bifid nasal tip, broad columella, widely separated slit-like nares, long philtrum with prominent bilateral swellings, and midline notch in the upper lip and alveolus. Additional recurrent features present in a minority of individuals have been upper eyelid ptosis and midline dermoid cysts of craniofacial structures. Assuming recessive inheritance, we mapped the locus in three families to chromosome 1 and identified mutations in ALX3, which is located at band 1p13.3 and encodes the aristaless-related ALX homeobox 3 transcription factor. In total, we identified seven different homozygous pathogenic mutations in seven families. These mutations comprise missense substitutions at critical positions within the conserved homeodomain as well as nonsense, frameshift, and splice-site mutations, all predicting severe or complete loss of function. Our findings contrast with previous studies of the orthologous murine gene, which showed no phenotype in Alx3(-/-) homozygotes, apparently as a result of functional redundancy with the paralogous Alx4 gene. We conclude that ALX3 is essential for normal facial development in humans and that deficiency causes a clinically recognizable phenotype, which we term frontorhiny.

Defense mechanisms in congenital and acquired facial disfigurement: a clinical-empirical study.

It is of clinical interest to investigate the degree to which patients with facial disfigurement use defense styles. Therefore, 59 adults born with rare facial clefts, 59 patients with facial deformities acquired at an adult age, and a reference group of 141 adults without facial disfigurements completed standardized questionnaires. There was a significant difference between the group with and the group without disfigurements on immature defense styles, with the disfigured group using the immature style more frequently. There was a trend for the nondisfigured group to use more mature defense styles. No difference between congenital and acquired groups was seen on individual types of defense style. Self-esteem had the strength to differentiate mature and immature defense styles within our disfigured groups. The association of low self-esteem and the utilization of immature defense styles suggests that professional help may tailor treatment on discussing immature defense style and problems triggering or maintaining this style.

Assessing nonacceptance of the facial appearance in adult patients after complete treatment of their rare facial cleft.

BACKGROUND: Treatment of patients with severe congenital facial disfigurements is aimed at restoring an aesthetic and functional balance. Besides an adequate level of satisfaction, an individual's acceptance of facial appearance is important to achieve because nonacceptance is thought to lead to daily psychological struggles. This study objectified the prevalence of nonacceptance among adult patients treated for their severe facial clefts, evaluated risk factors, and developed a screening tool. METHODS: The study included 59 adults with completed treatment for their severe facial cleft. All the patients underwent a semistructured in-depth interview and filled out the Body Cathexis Scale. RESULTS: Nonacceptance of facial appearance was experienced by 44% of the patients. Of the nonaccepting patients, 72% experienced difficulties in everyday activities related to their appearance versus 35% of the accepting patients. Acceptance did not correlate with objective severity or bullying in the past. Risk factors for nonacceptance were high self-perceived visibility, a troublesome puberty period, and an emotion-focused coping strategy. Also, the presence of functional problems was shown to be highly associated. CONCLUSIONS: The objective severity of the residual deformity did not correlate with the patients' acceptance of their facial appearance, but the self-perceived visibility did correlate. The process of nonacceptance resembles the process seen in patients with body dysmorphic disorders. Surgical treatment is no guarantee for an improvement in acceptance and is therefore discouraged for patients who match the risk factors for nonacceptance unless it solves a functional problem. The authors therefore recommend screening patients for nonacceptance and considering psychological treatment before surgery is performed. LEVEL OF EVIDENCE III: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors at www.springer.com/00266.

Prevalence of Ocular Anomalies in Craniosynostosis: A Systematic Review and Meta-Analysis.

BACKGROUND: The aim of this study was to describe the ophthalmic abnormalities and their prevalence in craniosynostosis prior to craniofacial surgery. METHODS: A systematic search was conducted on Medline OVID, Embase, Cochrane, Google Scholar, Web of Science Core Collection. Inclusion criteria were English papers, children aged <18 years with non-syndromic and syndromic craniosynostosis, case reports, case series, and case-control studies. A system of domains was established consisting of an anatomic and functional ophthalmic domain. A meta-analysis of single proportions was carried out using random effects model and pooled mean proportions with 95% confidence intervals (CI) were calculated. RESULTS: Thirty-two papers analyzing 2027 patients were included. Strabismus was the most common anomaly in non-syndromic craniosynostosis: Horizontal strabismus was highest prevalent in unicoronal craniosynostosis (UCS) 19% (95% CI 9-32), followed by vertical strabismus 17% (95% CI 5-33). In syndromic craniosynostosis, horizontal strabismus was most prevalent in Crouzon syndrome 52% (95 CI 26-76), followed by Apert syndrome 50% (95% CI 42-58). Vertical strabismus was most prevalent in Saethre-Chotzen 60% followed by Muenke's syndrome 36%. Furthermore, astigmatism was the second most reported outcome in non-syndromic craniosynostosis and highest prevalent in UCS 35% (95% CI 21-51). In syndromic craniosynostosis, astigmatism was most frequently seen in Crouzon syndrome 43% (95% CI 22-65), followed by Apert syndrome 34% (95% CI 14-58). Moreover, in syndromic craniosynostosis, 5-40% had a decrease in visual acuity (VA) ≤ 0.3 LogMAR in the better eye and 11-65% had a VA ≤ 0.3 LogMAR in at least one eye. DISCUSSION: This review demonstrates the high prevalence of ocular anomalies in non-syndromic and syndromic craniosynostosis. A multidisciplinary and systematic approach is needed for the screening and optimal treatment of these conditions in a timely manner.

Cerebral Blood Flow of the Frontal Lobe in Untreated Children with Trigonocephaly versus Healthy Controls: An Arterial Spin Labeling Study.

BACKGROUND: Craniofacial surgery is the standard treatment for children with moderate to severe trigonocephaly. The added value of surgery to release restriction of the frontal lobes is unproven, however. In this study, the authors aim to address the hypothesis that the frontal lobe perfusion is not restricted in trigonocephaly patients by investigating cerebral blood flow. METHODS: Between 2018 and 2020, trigonocephaly patients for whom a surgical correction was considered underwent magnetic resonance imaging brain studies with arterial spin labeling to measure cerebral perfusion. The mean value of cerebral blood flow in the frontal lobe was calculated for each subject and compared to that of healthy controls. RESULTS: Magnetic resonance imaging scans of 36 trigonocephaly patients (median age, 0.5 years; interquartile range, 0.3; 11 female patients) were included and compared to those of 16 controls (median age, 0.83 years; interquartile range, 0.56; 10 female patients). The mean cerebral blood flow values in the frontal lobe of the trigonocephaly patients (73.0 ml/100 g/min; SE, 2.97 ml/100 g/min) were not significantly different in comparison to control values (70.5 ml/100 g/min; SE, 4.45 ml/100 g/min; p = 0.65). The superior, middle, and inferior gyri of the frontal lobe showed no significant differences either. CONCLUSIONS: The authors' findings suggest that the frontal lobes of trigonocephaly patients aged less than 18 months have a normal cerebral blood flow before surgery. In addition to the very low prevalence of papilledema or impaired skull growth previously reported, this finding further supports the authors' hypothesis that craniofacial surgery for trigonocephaly is rarely indicated for signs of raised intracranial pressure or restricted perfusion for patients younger than 18 months. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.