Ethical Considerations in Surgery for Single-suture Craniosynostosis.

Singe-suture craniosynostosis (SSC) describes the premature fusion of one cranial suture, which restricts cranial growth and consequently results in unaffected regions presenting a compensatory expansion. Surgery can redistribute intracranial volume, reduce the risk of elevated intracranial pressure, and improve head shape, potentially leading to improved neurocognitive function and social acceptance. However, there is limited evidence that surgery for SSC improves neurocognitive function and social acceptance. Given the inherent surgical risks and uncertainty of outcomes, the conditions under which this surgery should be allowed remain uncertain. Here, we discuss ethical questions regarding the permissibility of surgery, value of neurocognitive function and social acceptance, research ethics associated with SSC, patient autonomy and parental roles, and the process of recommending surgery and obtaining consent. Because surgery for SSC has become a routine procedure, its practice now presents a relatively low risk of complications. Furthermore, having acquired an understanding of the risks associated with this surgery, such knowledge fulfils the principle of non-maleficence although not beneficence. Thus, we advocate that surgery should only be offered within Institutional Review Board-approved research projects. In these situations, decisions concerning enrollment in scientific research involves health care providers and parents or guardians of the child, with the former acting as gate-keepers upon recognition of a lack of coping skills on the part of the parent or guardian in dealing with unforeseen outcomes. To minimize associated surgical risks and maximize its benefits, there exists a moral obligation to refer patients only to highly specialized centers.

Health-related quality of life of children treated for non-syndromic craniosynostosis.

Health-related quality of life (HRQoL) allows the acquisition of the subjective perspective of patients regarding their health and function; yet a very few studies have been evaluated HRQoL of patients treated for craniosynostosis (CS). In this retrospective, descriptive cohort study, school-aged children (7-16 years) treated for non-syndromic CS were assessed using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales. Seventy-three patients and their parents responded to the PedsQL (response rate: 80.2%). Patients generally estimated average HRQoL with no difference compared to the normal population sample. Further, no difference in HRQoL was found between treated sagittal (SS) or metopic synostosis. In the SS group, surgical methods involving spring-assisted surgery and pi-plasty were unrelated to HRQoL outcomes. Additionally, HRQoL was highly correlated with intelligence quotient (IQ, r = 0.42; p = 0.0004) and adaptive behavior skills (ABAS, r = 0.57; p = 0.0001). Furthermore, differences were observed in estimated physical function (p = 0.002) and school function (p = 0.012) between self- and proxy reports (i.e. parents estimated child HRQoL as higher than did the children). Children treated for CS have a generally average HRQoL, and neither CS type nor surgical method influenced HRQoL outcomes. Moreover, children and parents estimated HRQoL differently, suggesting the importance of using both self- and proxy reporting in patient-reported measures. HRQoL was strongly related to IQ and ABAS, indicating that the PedsQL can be used as a screening instrument to identify craniofacial patients in need of further psychological assessment.

Children Treated for Nonsyndromic Craniosynostosis Exhibit Average Adaptive Behavior Skills with Only Minor Shortcomings.

BACKGROUND: Adaptive behavior skills are important when assessing cognitive functions related to daily life; however, few studies have assessed these skills in patients treated for nonsyndromic craniosynostosis. In this study, the authors assessed the adaptive behavior skills of children treated for craniosynostosis and examined whether their outcomes are related to surgical technique. METHODS: The Adaptive Behavior Assessment System, 2nd Edition, parent report was used for children (age, 7 to 16 years) treated for sagittal (n = 41), metopic (n = 24), and other rare synostoses (n = 8). Background data, including intelligence quotient, were controlled for confounders. RESULTS: All evaluated children treated for craniosynostosis were estimated as lower in all aspects of adaptive behavior skills (full-scale, conceptual, social, and practical composites; effect size, 0.36 to 0.44) as compared with norms. The sagittal group showed shortcomings in social composite (effect size, 0.48) and subscales measuring self-care and self-direction, although no difference was observed between spring-assisted surgery and pi-plasty regarding outcomes of adaptive behavior skills. In addition, children treated for metopic synostosis showed results indicating shortcomings with adaptive behavior according to the full-scale, conceptual, and social composites (effect size, 0.53 to 0.61) relative to norms. Furthermore, attrition analysis revealed no significant differences between responders (rate, 80.2 percent) and nonresponders. CONCLUSION: These results found that children treated for craniosynostosis display average adaptive behavior skills, and that the two surgical techniques used to treat sagittal synostosis did not differ in their behavioral outcomes. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

The reliability of the health related quality of life questionnaire PedsQL 3.0 cancer module in a sample of Swedish children.

BACKGROUND: The Pediatric Quality of Life Inventory (PedsQL) is a modular instrument, designed to integrate generic and disease specific measures, and includes both self- and proxy-reports. The aim of the study was to assess the reliability and limited validity of the Swedish version of the disease specific Pediatric Quality of Life Inventory 3.0 Cancer Module Scales (PedsQL 3.0), in a sample of Swedish children diagnosed with cancer. METHOD: A total of 94 families at The Queen Silvia Children's Hospital, Sahlgrenska University participated in the study. The Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL 4.0) and the PedsQL 3.0 were administered to 63 children (aged 5-18 years) with cancer and to 94 parents of children with cancer aged 2-18 years. RESULTS: The internal consistency of the PedsQL 3.0, reached or exceeded Cronbach's alpha values of 0.70 for both -self- and proxy-reports. The PedsQL 4.0 and PedsQL 3.0 were highly correlated (r = 0.94 for proxy-reports and r = 0.91 for self-reports), indicating convergent validity. CONCLUSION: PedsQL 3.0 Cancer Module Scales can be used as a valuable tool for measuring cancer-specific HRQOL in child populations, both in research and in clinical practice.

[Formula: see text] Sustained attention and vigilance of children treated for sagittal and metopic craniosynostosis.

Attention problems are common in patients with craniosynostosis. Craniosynostosis is a rare condition, studies face challenges of selection bias, small sample sizes, and wide age ranges. The aim of the study was to assess the sustained attention and vigilance of children treated for sagittal and metopic craniosynostosis. To reduce selection bias, we included children that had previously undergone surgery for craniosynostosis, were between 8 and 16 years, and lived close to the craniofacial centre. The Connors Continuous Performance Test (3rd edition) was used to measure sustained attention and vigilance (n = 61; response rate: 76.3%). Attrition analysis revealed no differences between responding and non-responding groups regarding background variables. One identified difference between the SS (n = 28) and MS (n = 23) groups involved significantly better performance by the SS group in the hit-reaction time (HRT) test relative to the MS group (p < 0.05). Compared with the norms, the SS group showed significantly worse response style, detectability, omissions, commissions, perseverations, HRT response speed (HRT-SD), HRT inter-stimulus interval change (HRT-iC) (p < 0.01 for all), and variability (p < 0.05). The MS group showed significantly worse detectability, HRT-SD, variability (p < 0.01 for all), commissions, perseverations and HRT-iC (p < 0.05 for all) as compared with norms. No differences regarding attention was detected for the two different surgical techniques used for correction of sagittal synostosis. There were shortcomings in sustained attention and vigilance as compared with the norms in the SS and MS groups, although the deviations were small.

The Cognitive Profile of Children with Nonsyndromic Craniosynostosis.

BACKGROUND: Long-term neuropsychological and cognitive outcomes in patients with nonsyndromic craniosynostosis have proven difficult to evaluate objectively because of methodologic problems with published studies based on their small and biased samples of patients, wide age ranges, and testing with unacceptable psychometric properties. This study evaluated the Full-Scale Intelligence Quotient and its subscales in a cohort with a small selection bias. METHODS: Patients aged 7 to 16 years, born with nonsyndromic craniosynostosis and surgically treated, were tested using the Wechsler Intelligence Scale for Children, Fourth Edition. Ninety-one patients were invited, and 73 patients were tested. RESULTS: There was no difference in Full-Scale Intelligence Quotient score between patients who had undergone operations for sagittal synostosis or metopic synostosis and norms provided by the test. Patients operated on for sagittal synostosis showed a significantly higher perceptual reasoning intelligence quotient, but also significantly lower working-memory intelligence quotient and processing-speed intelligence quotient compared with the norms. Patients operated on for metopic synostosis showed no differences in any intelligence quotient index compared with the norm. In addition, attrition analysis showed no differences in background factors between responders and nonresponders. CONCLUSIONS: These results derived from a group of patients with uniform age range, and tested using an established tool, revealed that nonsyndromic children having undergone surgery for craniosynostosis exhibited average intellectual ability. However, the analysis indicated possible issues with working memory and processing speed in patients operated on for sagittal synostosis, highlighting impairments potentially associated with neuropsychological problems and that might contribute to learning disabilities.

The reliability of the Health Related Quality Of Life questionnaire PedsQL 3.0 Diabetes Module™ for Swedish children with type 1 diabetes.

AIM: The overall aim of the study was to assess reliability and accomplish a limited validation of the Pediatric Quality of Life Inventory 3.0 Diabetes Module Scales (PedsQL 3.0), Swedish version in a sample of Swedish children diagnosed with Type 1 diabetes (T1DM). A secondary aim was to assess whether the children's Health Related Quality of Life (HRQOL) was associated with children's gender and age and whether the child self- and parent proxy reports were consistent. METHODS: One hundred and thirty families from four diabetes centres participated in this study. The Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL 4.0) and the PedsQL 3.0 were administered to 108 children (aged 5-18 years) with T1DM and 130 parents (of children with T1DM aged 2-18 years). RESULTS: The internal consistency of the PedsQL 3.0, Swedish version, reached or exceeded Cronbach's alpha values of 0.70 for both child self- and proxy reports- and parent proxy-reports. The PedsQL 4.0 and PedsQL 3.0 were highly correlated (r = 0.76), indicating convergent validity. The parents reported lower diabetes-specific HRQOL than the children themselves (p < 0.01). The girls in the study reported lower psychological functioning and treatment adherence compared with the boys (p < 0.05). The oldest children (between 13 and 18 years of age) reported significantly lower diabetes-specific HRQOL, as compared with younger children (p < 0.05). CONCLUSIONS: PedsQL 3.0 Diabetes Module can be used as a valuable tool for measuring diabetes-specific HRQOL in child populations, both in research and in clinical practice.