Cognitive Outcomes of Children With Sagittal Craniosynostosis Treated With Either Endoscopic or Open Calvarial Vault Surgery.

Importance: Several studies have reported a higher incidence of neurodevelopmental delays and cognitive deficits in patients with single-suture craniosynostosis; however, there are few studies examining the associations of repair type with cognitive outcomes. Objective: To measure differences in neuropsychological outcomes between school-age children who were treated for sagittal craniosynostosis and unaffected controls and explore differences in cognitive function among children with sagittal craniosynostosis who were previously treated with either endoscopic strip craniectomy or open calvarial vault surgery. Design, Setting, and Participants: This cohort study was performed between 2018 and 2022. Eligible participants included patients aged 5 to 17 years who had previously been seen as infants or toddlers (<3 years) at 1 of 3 surgical centers for craniosynostosis repair with either endoscopic surgery or open calvarial vault surgery. A separate cohort of unaffected controls were included for comparison. Data analysis was conducted from November 2023 to February 2024. Exposures: Open calvarial vault surgery or endoscopic repair for single-suture craniosynostosis. Main Outcomes and Measures: The primary outcome was the Differential Ability Scales-II (DAS-II) General Conceptual Ability (GCA) score, an index for overall intellectual ability. Secondary outcomes included DAS-II subscale scores (Verbal Ability, Nonverbal Reasoning, Spatial Ability, Working Memory, and Processing Speed), and Patient-Reported Outcomes Measurement Information System (PROMIS) cognitive function scores. Results: A total of 81 patients with sagittal craniosynostosis (59 male [73%]; 22 female [27%]) and 141 controls (81 male [57%]; 60 female [43%]) were included. Of the 81 participants with sagittal craniosynostosis, 46 underwent endoscopic repair and 35 underwent open repair. Median (range) age at time of follow-up assessment was 7.7 (5.0-14.8) years for children with sagittal craniosynostosis and median age at assessment was 8.5 (7.7-10.5) years for controls. After controlling for age at assessment, sex, and socioeconomic status, there was no statistically significant or clinically meaningful difference in GCA scores between children who underwent endoscopic repair (adjusted mean score, 100; 95% CI, 96-104) and open repair (adjusted mean score, 103; 95% CI, 98-108) (P > .99). We found no significant difference in PROMIS scores between repair types (median [range] for endoscopic repair 54 [31-68] vs median [range] for open repair 50 [32-63]; P = .14). When comparing the treatment groups with the unaffected controls, differences in subscale scores for GCA and working memory were observed but were within normal range. Conclusions and Relevance: In this cohort study, there were no statistically or clinically significant differences in cognitive outcomes among school-age children by and type of surgical procedure used to repair nonsyndromic sagittal craniosynostosis. These findings suggest primary care clinicians should be educated about different options for craniosynostosis surgery to ensure early referral of these patients so that all treatment options remain viable.

Cognitive Development in Single-Suture Craniosynostosis - A Systematic Review.

There is conflicting evidence whether single-suture craniosynostosis (SSC), is linked to adversities of cognitive development. To assess the evidence for a link between SSC and cognition, a systematic literature search was conducted and eligible studies assessed for inclusion by two independent readers. Forty-eight studies met inclusion criteria. Small to medium but persistent effects on both general and some specific cognitive functions across age bands were found in higher quality studies for SSC overall. There was limited evidence for effects related to surgical correction. Methodologies varied substantially and there was a lack of longitudinal studies using broad assessment batteries.

Raising a Child with Craniosynostosis: Psychosocial Adjustment in Caregivers.

OBJECTIVE: While knowledge of the psychosocial impact of craniofacial conditions is growing, literature regarding the impact on parents remains limited. Parents of children born with a health condition may be at risk of experiencing a range of psychosocial challenges. This study conducted an initial investigation of psychosocial adjustment of parents of individuals with craniosynostosis to inform support provision for families. SETTING: An online UK-wide mixed-methods survey was distributed to members of Headlines Craniofacial Support. DESIGN: Quantitative data including standardized measures were analysed using descriptive statistics and independent samples t-tests, and inductive content analysis was used for open-ended questions. PARTICIPANTS: Mothers (n = 109) and fathers (n = 9) of individuals ages 3 months to 49 years with single suture (63%) or syndromic (33%) craniosynostosis participated. RESULTS: Compared to the general population, parents of individuals with craniosynostosis reported higher levels of stress, anxiety, and depression; lower levels of resilience and optimism. Qualitative responses provided insight into parents' experiences of birth, diagnosis, healthcare provision, familial wellbeing, and relationships. Parents reported several unmet information and support needs, alongside a range of positive outcomes. CONCLUSIONS: This study illustrates the potential long-term psychosocial implications for parents raising children with craniosynostosis. There is a need for routine psychological screening for family members and provision of appropriate psychological support for those at risk for distress. Non-specialist health professionals may benefit from additional training about craniofacial conditions so they are better equipped to support and refer families.

Neurocognitive outcomes of children with non-syndromic single-suture craniosynostosis.

While the focus of craniosynostosis surgery is to improve head shape, neurocognitive sequelae are common and are incompletely understood. Neurodevelopmental problems that children with craniosynostosis face include cognitive and language impairments, motor delays or deficits, learning disabilities, executive dysfunction, and behavioral problems. Studies have shown that children with multiple suture craniosynostosis have more impairment than children with single-suture craniosynostosis. Children with isolated single-suture subtypes of craniosynostosis such as sagittal, metopic, and unicoronal craniosynostosis can have distinct neurocognitive profiles. In this review, we discuss the unique neurodevelopmental profiles of children with single-suture subtypes of craniosynostosis.

Sagittal Synostosis and Its Association With Cognitive, Behavioral, and Psychological Functioning: A Meta-analysis.

Importance: Findings on the cognitive, behavioral, and psychological functioning of individuals with sagittal synostosis (SS) are highly disparate, limiting their clinical utility. Objective: To identify and review research on individuals with SS and to determine whether, and to what extent, they experience cognitive, behavioral, and psychological difficulties compared with their healthy peers or normative data for each measure. Data Sources: PubMed, Scopus, Embase, and PsycINFO were searched through January 2021 with no date restrictions. Scopus citation searches and manual checks of the reference lists of included studies were conducted. Study Selection: Studies included participants of any age who had received a diagnosis of single-suture (isolated or nonsyndromic) SS or scaphocephaly and who had been assessed on cognitive, behavioral, and psychological outcomes. Data Extraction and Synthesis: Data were independently extracted by 2 reviewers. Case-control outcomes (individuals with SS vs healthy peers or normative data) were compared using random-effects models with 3 effect sizes calculated: weighted Hedges g (gw), odds ratios (ORs), and mean prevalence rates. This study follows the Meta-analysis of Observational Studies in Epidemiology (MOOSE) reporting guidelines. Main Outcomes and Measures: Findings were categorized by surgical status (conservatively managed, presurgery, postsurgery, or combined); domain (eg, general cognition); type of cognitive, behavioral, or psychological measure (objective or subjective); and source of comparison data (peers or normative data). Results: Data from 32 studies, involving a pooled sample of 1422 children and adults with SS (mean [SD] age at assessment, 5.7 [6.6] years; median [interquartile range] age, 3.3 [0.5-10.3] years), were analyzed. Data on sex were available for 824 participants, and 642 (78%) were male. Individual study results varied substantially. Objective tests identified significant moderate group differences on 3 of 16 examined domains: presurgical motor functioning (3 studies; gw = -0.42; 95% CI, -0.67 to -0.18; P < .001), postsurgical short-term memory (2 studies; gw = -0.45; 95% CI, -0.72 to -0.17; P < .001), and postsurgical visuospatial ability (6 studies; gw = 0.31; 95% CI, 0.18 to 0.44; P < .001). Prevalence estimates and ORs varied widely, with 15 studies showing prevalence estimates ranging from 3% to 37%, and 3 studies showing ORs ranging from 0.31 (95% CI, 0.01 to 6.12) for processing speed in the conservatively managed sample to 4.55 (95% CI, 0.21 to 98.63) for postsurgical visuospatial abilities. Conclusions and Relevance: In this meta-analysis, findings for the functioning of participants with SS were highly disparate and often of low quality, with small samples sizes and control groups rarely recruited. Nonetheless, the findings suggest that some individuals with SS experience negative outcomes, necessitating routine assessment.

Cognitive, behavioral and psychological functioning of children and adults with conservatively managed metopic synostosis.

Children diagnosed with metopic synostosis (MS) commonly experience poor neuropsychological outcomes, with research suggesting that children whose MS is managed conservatively (without surgery) potentially having worse outcomes than their operated peers. However, studies of children whose MS was managed conservatively are scarce. This study therefore examined the cognitive, behavioral, and psychological functioning of children/adults with conservatively managed MS (N = 38) and compares their outcomes to individually matched healthy controls (N = 38) of the same age and sex (matched-pairs design) from the general community. Age-appropriate, validated assessments measuring general cognition, verbal and visuospatial ability, attention and working memory, executive functioning, behavior, depression, anxiety, and satisfaction with appearance were utilized. Group differences were estimated using linear regression for (a) the overall sample and (b) by broad developmental stages: 2&3 yrs; ≥6-≤17. Moderate to large negative effects (g = -0.38 to -1.30) were evident before controlling for socio-economic status (SES), with the MS group performing significantly worse on 8 out of the 10 cognitive domains (general cognition, visuospatial ability, working memory, information processing, executive functioning: semantic & initial letter verbal fluency, switching, inhibition+switching). However, only initial letter verbal fluency (g = -0.99) and switching (g = -1.19) remained significant after adjusting for SES. The MS group displayed more behavioral problems, although this was not significant. Depression, anxiety, and satisfaction with appearance did not differ between the groups. Regular monitoring of cognitive functioning, particularly executive functioning, should be undertaken for those with conservatively managed MS.

Revisiting the Resolution of Chiari Malformation in Nonsyndromic Craniosynostosis: A Case of Posterior Cranial Vault Reconstruction in Secondary Pan-Suture Synostosis.

BACKGROUND: While commonly seen in syndromic craniosynostosis, the incidence of Chiari malformation (CM) in nonsyndromic craniosynostosis has been reported at 5% and there is a lack of understanding of the pathophysiology and management of CM in this patient population. CASE DESCRIPTION: We present a 5-year-old male patient who underwent a sagittal craniosynostosis repair at the age of 5 months and returned at the age of 5 years with daily headaches associated with behavioral changes. He was found to have pan-synostoses and radiographic evidence of increased intracranial pressure, including a Chiari malformation. Neurologic and genetic workup was unremarkable. A cranial vault reconstruction was performed, and subsequent imaging demonstrated resolution of previously noted Chiari malformation. CONCLUSIONS: In our case, we provided a unique window into the underlying pathophysiology for CM in patients with concurrent nonsyndromic craniosynostosis that we hope will add to the current foundation of literature supporting the intricate relation between cranial vault compliance and Chiari malformation or hindbrain herniation. Furthermore, we provide insight into the management of acquired CM and support isolated cranial vault reconstruction in those who do not appear to have symptomatic suboccipital compression.

[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.

Frustration and Emotional Regulation in Nonsyndromic Craniosynostosis: A Functional Magnetic Resonance Imaging Study.

BACKGROUND: Nonsyndromic craniosynostosis may manifest with complex behavioral, attentional, and emotional sequelae. The authors characterized higher level brain connectivity in adolescent nonsyndromic craniosynostosis patients in response to emotional frustration. METHODS: Surgically corrected patients older than 9 years with nonsyndromic craniosynostosis were age/sex/handedness matched to controls. Patients participated in a "go/no-go" task, structured as win/lose/recovery paradigms. BioImage Suite was used to analyze whole-brain intrinsic connectivity between tasks with cluster-corrected group-level t maps. A value of p < 0.05 was significant. RESULTS: Seven unilateral coronal (average age, 12.2 years), six metopic (average age, 11.5 years), and controls were included. Unilateral coronal had worse emotional regulation scores on the Behavior Rating Inventory of Executive Function survey (p = 0.065) and performed poorly on the go/no-go task (p < 0.001). Metopic had four regions of interest, with the majority having decreased activity compared with controls, and few differences between tasks. Unilateral coronal patients had 11 regions of interest; the majority decreased during the win and lose conditions, but all increased during the recovery condition. Metopic patients had decreased blood oxygenation level- dependent signal in the posterior cingulate (p = 0.017) and middle temporal gyrus (p = 0.042). Unilateral coronal had decreased signal in the posterior cingulate (p = 0.023), middle temporal gyrus (p = 0.027), and thalamus (p = 0.033), but increased signal in the cuneus (p = 0.009) and cerebellum (p = 0.009). Right unilateral coronal, but not metopic/controls, had increased right brain activity in the caudate (p = 0.030), thalamus (p = 0.011), temporal lobe (p = 0.012), and cerebellum (p = 0.029). CONCLUSIONS: Unilateral coronal patients may have emotional dysregulation in response to frustration, whereas metopic patients may have attenuated emotional reactions. Evidence of right unilateral coronal brain laterality suggests that the area of suture fusion may contribute to the mechanism of dysfunction. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

[Neuropsychological consequences of craniosynostosis: Non-syndromic scaphocephaly]. / Le retentissement neuropsychologique de la craniosténose : cas de la scaphocéphalie non syndromique.

BACKGROUND: Scaphocephaly increases the rate of some modifications of cognitive and mood profile in a manner that remains to be elucidated. OBJECTIVE: We aimed to describe the impact of scaphocephaly on neuropsychological profile and more particularly on the executive functions. PATIENTS AND METHODS: An experimental group of 19 children older than 5 years, operated on for scaphocephaly, was compared with a control group of 10 children operated on for trigonocephaly, using IQ tasks, attention tasks and mood scales. A group of 6 children from 2 to 4 years old, operated on for scaphocephaly, and a group of 6 children with non-operated scaphocephaly are also described. RESULTS: Both the experimental group and the control group showed unchanged IQ, whereas attention deficit and anxiety disorder were more frequent in the experimental group. Cognitive profiles differed between groups, with a higher rate of impaired inhibitory control of visual processing in the scaphocephaly group, contrasting with a higher rate of impaired auditory verbal working memory in the trigonocephaly group. Comparable profiles were also found in groups of younger or non-operated children with scaphocephaly. CONCLUSIONS: Many children with scaphocephaly must cope with a specific neuropsychological profile throughout development. This study suggests the interest for these children and their families of specific follow-up in reference centers.

[Psychological support for children operated on for craniosynostosis and their family. Some thoughts for the craniofacial surgeon]. / Suivi psychologique des enfants opérés de craniosténoses et de leurs proches. Quelques éléments de réflexions pour le chirurgien craniofacial.

What is the impact on child and family when they receive a diagnosis of craniostenosis? And what is the impact of surgery? What is the role of the clinical psychologist in accompanying the child and family, especially during hospital stay and surgery time? We present a few thoughts that help understand the psychological processes at work in case of craniostenosis, giving a little hint of the impact on the life of the child and family - which surgeons, preoccupied by more technical questions, sometimes tend to overlook.

Understanding the Learning Disabilities Linked to Sagittal Craniosynostosis.

OBJECTIVE: The purpose of this study is to investigate further findings that corroborate similarities between corrected sagittal craniosynostosis and attention deficit hyperactivity disorder (ADHD). The aim is to further characterize the neurocognitive deficits seen in adolescents with corrected craniosynostosis by comparing it to established learning deficits such as ADHD. METHODS: A total of 30 functional magnetic resonance imaging (fMRI) of 10 sagittal nonsyndromic craniosynostosis (sNSC), 10 ADHD-combined, and 10 control adolescents were studied. The fMRI scans were analyzed utilizing Statistical Parametric Mapping (University College London, UK) and analyzed with BioImageSuite (Yale University, New Haven, CT). RESULTS: The ADHD has lower connectivity to Brodmann area (BA) 11 (Montreal Neurological Institution [MNI]: -12,26,-21), BA20 (MNI: 62,-24,-25), and BA21 (MNI: 62,-32,-23) compared to sNSC and controls (P < 0.001). The sNSC has a unique visuospatial defect, compared to ADHD, created by decreased connectivity to BA31 (MNI: -3,-68,37), BA7 (MNI: -4,-68,41), BA19 (MNI: 0,-83,31), visual association cortex (MNI: -4,-78,22), and primary visual cortex (MNI: 7,-74,21) (P < 0.001). CONCLUSION: Patients born with sNSC have different neural connections than children born with ADHD. Patients born with sNSC have decreased connections in areas of visual processing and increased connections in areas of attention and auditory processing than patients with ADHD. Therefore, children with sagittal craniosynsotosis may have learning difficulties that, similar, yet different from ADHD.

Caregiver stress in children with craniosynostosis: a systematic literature review.

OBJECTIVES: We present an overview of the literature on caregiver stress in children with craniosynostosis and report common trends in the literature. INTRODUCTION: Craniosynostosis occurs approximately 1 in 2500 births. As this is a diagnosis most common in infants and often requires surgical treatment, this is a significant and stressful ordeal for caregivers. Caregiver stress impacts various outcomes for the child, and little is understood and known about caregiver stress in the pediatric craniosynostosis population. METHODS: A literature search for all articles pertaining to craniosynostosis and parental/caregiver stress was conducted using PubMed, Embase, PsychINFO, and CINAHL databases. RESULTS: Seven articles on caregiver stress in craniofacial abnormalities patients and three articles on caregiver stress in pediatric craniosynostosis patients specifically were identified. Three articles on parental satisfaction after craniosynostosis repair were also identified and included in the review. Few published studies exist in the literature on caregiver stress in children with craniosynostosis and no clear trends were identified. It is evident that caregiver stress significantly affects the psychosocial outcomes of children with craniosynostosis. However, there are an equal number of studies reporting significant differences in caregiver stress in children with craniosynostosis as those reporting no significant differences. CONCLUSIONS: There is evidence that caregiver stress affects psychosocial outcomes of children with craniosynostosis, but no clear trends of either increased or decreased levels of stress were identified in caregivers of children with craniosynostosis. Additional research is needed to identify risk factors related to caregiver stress.

Infant/toddler motor skills as predictors of cognition and language in children with and without positional skull deformation.

PURPOSE: To estimate associations between early motor abilities (at two age points, 7 and 18 months on average) and cognitive/language outcomes at age 3. To determine whether these associations are similar for children with and without positional plagiocephaly and/or brachycephaly (PPB). METHODS: The Bayley Scales of Infant/Toddler Development 3 were given at all age points to 235 children with PPB and 167 without PPB. Linear regressions assessed longitudinal associations between fine and gross motor scales and cognition/language. Item analyses examined the contributions of specific motor skills. RESULTS: Associations between 7-month motor skills and cognition/language were modest overall (effect sizes [ES] = - 0.08 to 0.10, p = .13 to .95). At 18 months, both fine and gross motor skills were associated with outcomes for children with PPB (ES = 0.21 to 0.41, p < .001 to .01), but among those without PPB, only fine motor skills were associated with outcomes (ES = 0.21 to 0.27, p < .001 to .001). CONCLUSIONS: Toddlers' motor skills were associated with cognition and language at 3 years, particularly among children with PPB. Interventions targeting early motor development in infants and toddlers with PPB may have downstream benefits for other outcomes.

Quality of Life in Adults with Nonsyndromic Craniosynostosis.

BACKGROUND: Although studies have analyzed quality of life in children with nonsyndromic craniosynostosis, to date nobody has investigated long-term quality of life in adults with nonsyndromic craniosynostosis. The purpose of this study was to compare quality of life in adult nonsyndromic craniosynostosis patients with a cohort of unaffected controls. METHODS: The authors queried their institution's prospectively maintained craniofacial registry for nonsyndromic craniosynostosis patients aged 18 years and older, and administered the validated World Health Organization Quality of Life Biomedical Research and Education Foundation questionnaire. Responses were compared, using a two-sample t test, to an age-matched U.S. normative database provided by the World Health Organization. RESULTS: One hundred fifty-one adults met inclusion criteria: 52 were successfully contacted and 32 completed the questionnaire. Average age of respondents was 23.0 ± 6.1 years (range, 18.1 to 42.1 years). Nonsyndromic craniosynostosis patients had a superior quality of life compared with comparative norms in all domains: physical health (17.8 ± 2.7 versus 15.5 ± 3.2; p < 0.001), psychological (16.3 ± 2.9 versus 13.8 ± 3.2; p < 0.001), social (16.9 ± 2.6 versus 13.2 ± 3.6; p < 0.001), and environmental (17.2 ± 2.5 versus 11.7 ± 2.7; p < 0.001). Comparison between single-suture subtypes revealed no difference in any quality-of-life domain (p > 0.05). CONCLUSIONS: Adult patients previously treated for nonsyndromic craniosynostosis perceive their quality of life to be high, superior to that of a normative U.S. SAMPLE: Future work will seek to analyze additional patients and better understand the reasons behind these findings.

What's New in Syndromic Craniosynostosis Surgery?

LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Understand the role of prenatal screening and counseling of parents of unborn children with syndromic craniosynostosis. 2. Recognize the genetic abnormalities, craniofacial phenotype, associated anomalies, and challenges associated with each of the five major forms of syndromic craniosynostosis. 3. Identify the pros and cons associated with timing and types of cranial vault remodeling techniques in this patient population. 4. Understand the risks and benefits associated with midface advancement with Le Fort III, Le Fort II plus zygomatic repositioning, monobloc, and facial bipartition. 5. Understand the important role of psychological counseling throughout childhood in this at-risk group. SUMMARY: Crouzon, Apert, Pfeiffer, Muenke, and Saethre-Chotzen syndromes are the five most common forms of syndromic craniosynostosis. Although each has different genetic underpinnings and associated anomalies, their hallmark finding is turribrachycephaly most often associated with bicoronal craniosynostosis. The role of prenatal screening and counseling is growing, with caregivers becoming involved before birth. Multidisciplinary care from birth onward involves craniofacial plastic surgeons, neurosurgeons, otolaryngologists, ophthalmologists, orthodontists, anesthesiologists, psychologists, speech therapists, and geneticists. Early partial, or regional, craniectomy may be urgently indicated in multisuture cases with signs of increased intracranial pressure. Others may be managed successfully with posterior cranial vault distraction, middle vault expansion, or fronto-orbital advancement. Some authors have advocated early monobloc advancement for those patients who require acute airway intervention and globe protection, although the risks of these procedures are high. Many patients will require midfacial advancement with a Le Fort III, Le Fort II plus zygomatic repositioning, monobloc, or facial bipartition. The indications, risks, and benefits for each midfacial procedure must be considered, as this step in the treatment algorithm may carry the greatest functional and aesthetic benefits but also the potential for the greatest morbidity. At the culmination of facial growth, it is not uncommon for patients to require conventional orthognathic surgery and other bony contouring and soft-tissue procedures. Finally, an understanding of the psychological aspects of craniofacial difference, both in affected individuals and in their families, is essential to a successful, holistic approach.

Sagittal craniosynostosis: a utility outcomes study.

OBJECTIVE Sagittal craniosynostosis results in a characteristic scaphocephalic head shape that is typically corrected surgically during a child's 1st year of life. The authors' objective was to determine the potential impact of being born with sagittal craniosynostosis by using validated health state utility assessment measures. METHODS An online utility assessment was designed to generate health utility scores for scaphocephaly, monocular blindness, and binocular blindness using standardized utility assessment tools, namely the visual analog scale (VAS) and the standard gamble (SG) and time trade-off (TTO) tests. Utility scores were compared between health states using the Wilcoxon and Kruskal-Wallis tests. Univariate regression was performed using age, sex, income, and education as independent predictors of utility scores. RESULTS Over a 2-month enrollment period, 122 participants completed the online survey. One hundred eighteen participants were eligible for analysis. Participants rated scaphocephaly due to sagittal craniosynostosis with significantly higher (p < 0.001) median utility scores (VAS 0.85, IQR 0.76-0.95; SG 0.92, IQR 0.84-0.98; TTO 0.91, IQR 0.84-0.95) than both monocular blindness (VAS 0.60, IQR 0.50-0.70; SG 0.84, IQR 0.68-0.94; TTO 0.84, IQR 0.67-0.91) and binocular blindness (VAS 0.25, IQR 0.20-0.40; SG 0.51, IQR 0.18-0.79; TTO 0.55, IQR 0.36-0.76). No differences were noted in utility scores based on participant age, sex, income, or education. CONCLUSIONS Using objective health state utility scores, authors of the current study demonstrated that the preoperatively perceived burden of scaphocephaly in a child's 1st year of life is less than that of monocular blindness. These relatively high utility scores for scaphocephaly suggest that the burden of disease as perceived by the general population is low and should inform surgeons' discussions when offering morbid corrective surgery, particularly when driven by aesthetic concerns.

[DEMOGRAPHIC AND ANATOMIC CHARACTERISTICS AND SURGERY RESULTS IN CHILDREN WHO WERE TREATED IN SOROKA FOR CRANIOSYNOSTOSIS].

BACKGROUND: Craniosynostosis, the premature fusion of one or more of the cranial sutures, can occur as a part of a syndrome or as an isolated deformity. When not treated properly it may have major physical and social implications. STUDY HYPOTHESIS: We assumed that our center's results are similar to those reported in the literature, in terms of gender and anatomic characteristics, and different in terms of the number of syndromic cases. We also assume that only a few patients needed a second surgery and that this medical problem effects quality of life in term of cognitive impairment and aesthetic results. METHODS: A retrospective chart review was conducted of patients who were treated for craniosynostosis in Soroka University Medical Center between the years 1991-2005. RESULTS: A total of 63 patients were treated in Soroka during those years: 30 (47.6%) were females and 33 (50.8%) were males; 27 (42.9%) had coronal synostosis, 20 (31.8%) had sagital synostosis, 10 (15.8%) had metopic synostosis, 5 (7.9%) had lambdoid synostosis. A total of 5 (7.9%) cases were syndromic and the rest were isolated; 6 patients (9.5%) needed a second surgery for correction of the defect; 8 out of 30 patients (26.7%) 6 years or older at the present time suffer from attention deficit disorder. Parents' satisfaction from aesthetic results on a scale of 1-5 was 4.77 with SD of 0.504, while the doctors' score was 3.93 with SD 0.980 (p = 0.01). CONCLUSIONS: Craniosynostosis is a complex surgical problem, however with prompt treatment it may attain high levels of satisfaction among parents and patients. We found high rates 26.7% vs 10% (in the general population) of ADD in children affected by the disease.