RESUMO
ABSTRACT: The coronal incision is the mainstay for access in craniosynostosis surgery. Scarring is a common concern of parents whose children are offered an open procedure. To the author's knowledge, there are no previous studies looking at the psychosocial impact of scarring from coronal access incisions for craniosynostosis procedures. The author's study focused on patients undergoing procedures for nonsyndromic single-suture craniosynostosis.This study comprised 3 parts: worldwide survey regarding coronal access incisions for craniosynostosis surgery, questionnaire to determine the psychosocial impact of the scars on patients and their parents, and measurement of postoperative scars in craniosynostosis patients.Survey responses from 46 craniofacial centers worldwide revealed a zig-zag was the most commonly utilized incision. Seventy-two percent of survey responses reported problems with postoperative stretching of the scar; only 20% of centers reported formal data collection of whether families were affected by this.Psychology questionnaires revealed that the majority of patients and their parents were not bothered by the zig-zag coronal scars. Patient felt the scars were less noticeable than the parents. Parent perceptions improved with age and time postsurgery.Coronal access scars following craniosynostosis surgery appear to stretch more in the supra-auricular region compared with the midline.These findings are useful for the craniofacial multidisciplinary team to inform parents contemplating surgery and who may be concerned about the impact of the scar in the future.
Assuntos
Cicatriz , Craniossinostoses , Criança , Craniossinostoses/cirurgia , Humanos , Pais , Período Pós-Operatório , SuturasRESUMO
ABSTRACT: The Craniofacial Collaboration UK (CC-UK) has been established across the 4 highly specialized craniofacial centers in the UK since 2015. This joint collective aims to address the current limitations within developmental craniofacial research, using robust clinical data from a homogenous sample of children. This paper presents the third wave of findings from the CC-UK, with consideration to developmental and behavioral parent-report measures. Whilst previous data for sagittal synostosis have been presented, this article summarizes the analysis of these outcomes for children with metopic synostosis (MS) at 3 years who have undergone primary corrective surgery. Results highlight similar patterns to that of earlier CC-UK work, with the majority of children falling within 1 standard deviation of the population normative means across all measures. However, statistically significant difficulties were found between group means for children with MS on various developmental and behavioral domains. Prosocial skills and peer difficulties were reported as the greatest areas of behavioral concern for parents, with prosocial skills found to be below the level expected for their chronological age. In order to further understand the developmental trajectory of children with MS, longitudinal examination of individual diagnostic and specific age groups with single-suture craniosynostosis is crucial. The continuation of the CC-UK provides an opportunity to attain this goal.
Assuntos
Craniossinostoses , Pré-Escolar , Suturas Cranianas , Craniossinostoses/cirurgia , Ossos Faciais , Humanos , Lactente , Suturas , Reino UnidoRESUMO
ABSTRACT: Heterozygous mutations in the TCF12 gene were discovered in 2013 as a cause of craniosynostosis (CS). However, limited information regarding the behavioral phenotypic profile is available. Here the authors provide the first detailed study of the neurodevelopmental, cognitive, and psychosocial outcomes for patients with a pathogenic TCF12 variant and associated CS.A clinical casenote audit was conducted at the 4 UK highly specialized craniofacial centers. A total of 35 patients aged 18âmonths to 10âyears with an identified TCF12 pathogenic variant and CS (bicoronal CSâ=â45.7%, unicoronal CSâ=â40.0%, multisutureâ=â14.3%) were included. Standardized screening and/or assessment of full-scale intelligence quotient, social communication, development, behavior, and self-concept were conducted.In the majority of cases, outcomes were consistent with age-related expectations. About 75% of patients demonstrated no delay across any early developmental domain, while 84.6% demonstrated full-scale intelligence quotient scores within 1 standard deviation of the population mean. Significant behavioral difficulties were demonstrated by parent reporters in 26.3% to 42.1% of cases (dependent upon domain). Clinically elevated social communication profiles were present in (41.7%) of parent-reported cases. Levels of self-concept (at age 10) were consistent with age-related normative data.Most patients with a TCF12 pathogenic variant had a mild behavioral and cognitive phenotype, although they may be at a slightly increased risk of social communication difficulties and psychosocial issues. Although not measured statistically, there were no clear associations between surgical history and cognitive, behavioral, or psychosocial outcomes. This paper highlights the need for robust integrated developmental assessment of all CS patients, particularly those with an identified syndrome.
Assuntos
Fatores de Transcrição Hélice-Alça-Hélice Básicos/genética , Craniossinostoses , Criança , Pré-Escolar , Cognição , Craniossinostoses/genética , Heterozigoto , Humanos , Lactente , Mutação , FenótipoRESUMO
ABSTRACT: The Craniofacial Collaboration UK (CC-UK) is a joint initiative that seeks to address some of the limitations of previous developmental research with this patient group by providing systematically collected, robust data from clinically and chronologically homogenous representative samples of children. The current paper outlines the developmental outcomes at the age of 5 for children who had previously undergone primary surgery for single-suture sagittal synostosis (SS). It shows broad consistencies with the previous CC-UK work, indicating that the majority of children with SS will perform within the average range compared to peers across a number of developmental, behavioral and emotional domains. However, the group mean for children with SS indicates significantly greater difficulties with fine motor skills and hyperactivity, relative to normative data. Unexpectedly, children with SS had significantly better problem solving skills. While it is reassuring that the majority of children are broadly developing in line with their unaffected peers, these small but significant differences may be early indicators of some of the subtle difficulties documented in older children with craniosynostosis. Longitudinal follow up is therefore important to understand the developmental trajectory for children with SS and identification of potentially 'at risk' sub groups within this diagnostic cohort.
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Craniossinostoses , Anormalidades Maxilomandibulares , Pré-Escolar , Suturas Cranianas , Craniossinostoses/cirurgia , Ossos Faciais , Humanos , Reino UnidoRESUMO
The Craniofacial Collaboration United Kingdom (CC-UK) was established across the 4 Highly Specialized Craniofacial Centres (HSCCs) in the UK in 2015. This is the first wave of data to be analyzed, looking at 3-year-old children with sagittal synostosis who have had primary corrective surgery. This is a comprehensive, homogenous dataset, looking at parental measures of development and behavior. The results indicate that the majority of children are rated by their parents as falling within one standard deviation of the mean for both developmental and behavioral measures. However, there was a trend across the age groups within the sample which, although not statistically significant, indicates that more developmental difficulties may become apparent as children get older. Behavior was rated as more problematic, and the areas of greatest parental concern were Conduct (challenging or confrontational behavior) and Hyperactivity domains, where 24% of children were rated as within the clinically significant range. Although the majority of children were rated as falling within the average range, the difference in the mean between the sagittal and the normative group was significant in 5 of the 6 behavioral domains. Further research is required to examine whether these findings are stable over time and to look at the mechanism which might be driving these changes. It is anticipated that future CC-UK analysis will elucidate this more clearly.
Assuntos
Craniossinostoses/cirurgia , Pré-Escolar , Ossos Faciais , HumanosRESUMO
The clinical psychologists at the 4 highly specialized craniofacial centers in the United Kingdom have developed a systematic, developmental screening protocol, called the Craniofacial Collaboration UK (CC-UK). Previous systematic reviews of the literature into the developmental sequelae of single-suture craniosynostosis have identified a number of methodological flaws which the CC-UK seeks to avoid. This study presents an introduction to the rationale for its development, as well as the methodology of the CC-UK.