Craniosynostosis Surgery Research Articles

A New Method for Measuring Cognition in Children With Sagittal Craniosynostosis

Objectives: The neurocognitive profile of patients who have undergone spring-assisted surgery (SAS) for craniosynostosis is not well understood, and a variety of different cognitive measures have been used across various studies. Although neuropsychological evaluations can document cognitive strengths and weaknesses across a number of cognitive areas, they can be time consuming. This study aimed to: (1) describe the neurocognitive profile of patients who underwent SAS using both a common intelligence measure and iPad assisted testing measuring several cognitive areas, as well as parent ratings; (2) better understand the feasibility and utility of using iPad assisted assessments in this population. Methods: Thirty-six patients between the ages of 6 and 18 with sagittal synostosis who underwent SAS participated in a brief battery of assessments including a measure of abbreviated IQ, National Institutes of Health Toolbox Cognition Battery (NIH Toolbox), and caregiver ratings on the Behavior Rating Inventory of Executive Function – Second Edition (BRIEF-2). Statistical analyses assessed performance within the group and between measures. Results: Positive correlations were found between all 3 NIH Toolbox Composite scores and abbreviated IQ. Five of 7 subtests were positively correlated with Full Scale IQ, while 4 of 7 were positively correlated with both verbal and visual reasoning indices. Negative correlations between the Crystallized Composite score and BRIEF-2 were found for 8 of 9 BRIEF-2 subscales. Three of 9 BRIEF-2 subscales were negatively correlated with Fluid Composite and Total Composite scores. Conclusions: Cognitive data were generally aligned with the normal distribution curve. Although caregiver ratings yielded several executive functioning concerns, significant correlations were found with composite scores and a few specific subtests. Use of the NIH Toolbox was feasible in this population and shows promise as a tool for measuring a range of different cognitive domains.

Comparison of Surgical Techniques in Managing Craniosynostosis: Systematic Review and Bayesian Network Meta-Analysis

Open total cranial vault reconstruction (CVR) is the common procedure in managing craniosynostosis, yet more techniques have been introduced as alternatives, namely endoscopic suturectomy (ES), endoscopy-assisted craniectomy (EC), spring-assisted surgery (SAS), strip craniectomy with helmet (SC), Pi craniectomy (PiC), Pi plasty (PiP), and Renier’s “H” technique (RH). The aim of this study was to compare the effectiveness of craniosynostosis surgeries in improving the cephalic index of the patients. Studies published until 7 March 2024 reporting CVR, ES, SAS, SC, RH, and PiP as definitive craniosynostosis management with the cephalic index as the outcome were included. Bayesian network meta-analysis and pair-wise meta-analysis were performed using a random-effects model based on standardized mean difference (SMD) and 95% confidence interval (CI). Nine studies published in 2008–2024 recruiting a total of 464 craniosynostosis patients (age: 18–61 months) were included in this meta-analysis. EC (SMD = 0.23 [95%CI: −5.47 to 5.63]; p = 0.935), PiP (SMD = −0.07 [95%CI: −9.27 to 8.79]; p = 0.988), ES (SMD = −0.59 [95%CI: −6.07 to 4.94]; p = 0.834), PiC (SMD = −1.16 [95%CI: −8.89 to 6.35]; p = 0.765), RH (SMD = −0.96 [95%CI: −6.62 to 4.53]; p = 0.736), SAS (SMD = −0.86 [95%CI: −8.25 to 6.18]; p = 0.815), and SC (SMD = −1.79 [95%CI: −9.05 to 5.28]; p = 0.624) were found to be as effective as CVR in improving the cephalic index. Network meta-analysis suggests that PiP is the most effective among these techniques (rank 1 probability = 0.273). According to the rank probabilities of our model the order of techniques from the most to the least effective is as follows: EC > CVR > PiP > ES > SAS > RH > PiC > SC.

Evaluating the Learning Curve and Patient Outcomes in Endoscopically Assisted Craniosynostosis Surgery: A 20-Year Analysis

Evaluating the Learning Curve and Patient Outcomes in Endoscopically Assisted Craniosynostosis Surgery: A 20-Year Analysis

The impact of neurosurgery on the neuropsychological development of patients with craniosynostosis: A scoping review

Introduction: Neuropsychological assessment evaluates the cognitive profile by identifying deficits and enabling monitoring. At HRAC-USP, patients with craniosynostoses undergo this assessment due to impacts on neuropsychomotor development (DNPM). These conditions may cause cranial fusion, restricting brain growth and adversely affecting cognitive functions. Evaluation is crucial for guiding therapeutic and surgical approaches and ensuring better outcomes. Objective: To identify how surgical procedures impact the neuropsychological profile of patients with syndromic and isolated craniosynostoses through a scoping review. Methodology: This scoping review was conducted in March 2024 using PubMed, Embase, BVS/Lilacs, and Scopus databases; 1,215 articles were found and 18 original articles (E1-18) in English were selected that addressed the question: "What is the impact of surgery on neuropsychological aspects in patients with craniosynostoses?" Results: Studies on craniosynostosis highlighted the predominance of isolated cases (83.3%), with surgeries performed before one year of age associated with better cognitive outcomes. The cranial expansion technique (38.8%) was effective in non-syndromic cases, while cranial remodeling showed varied results. After surgery, there were overall improvements in DNPM, but also risks of language delay and motor deficits, especially in patients operated on at older ages. Conclusion: Surgery for craniosynostosis can improve neuropsychological outcomes, especially when performed early and with appropriate surgical techniques. More invasive procedures may lead to better outcomes, including increased Total Intelligence Quotient and better academic performance. It is crucial to consider such factors to optimize outcomes, reduce neurodevelopmental risks, and continuously monitor language and executive functions.

Pre- and postoperative intracranial volumes in children with non-syndromic craniosynostosis using 3D volumetric technique

Introduction: Corrective surgery for craniosynostosis presents several challenges to students during their training, with obtaining practical experience being one of the main obstacles. The neurocranium is formed by the bony structures that surround the brain, thus ensuring its protection. During the first years of life, the baby's skull grows exponentially. Understanding the normal growth and development of the cranial shape is essential for monitoring cranial development, detecting abnormalities, and evaluating the long-term results of craniosynostosis surgery. Objectives: To study the cranial volume gained after surgical treatment of craniosynostosis using 3D printing technology. Methodology: 26 patients who underwent craniosynostosis surgery, from 2019 to 2022 were selected. Preoperative, immediate, and late postoperative (3 months) tomography examination was performed. Reconstructing the exams using the Blender program with cranial volume calculation. 3D printing of skulls using Sethi 3D printer. Evaluation of results using Student's t-test with independent samples. Results: The study involved 26 patients, with ten presenting scaphocephaly treated with Renier's "H" cranial remodeling, five with trigonocephaly, five with plagiocephaly, and two with brachycephaly treated with front-orbital advancement (FOA). Cranial volumes were measured before and after surgery. For patients undergoing Renier's "H" treatment, there was an average increase of 224 cm³ between the late postoperative and preoperative stages, with smaller differences between the immediate postoperative and preoperative stages. For those treated with FOA, the average difference between late postoperative and preoperative stages was 138.8 cm³, while between immediate postoperative and preoperative stages was 129.7 cm³. Conclusion: Cranial volume gained by patients undergoing Renier's h technique and Fronto-orbital advancement is significant, showing that it may be possible to use less invasive techniques to take advantage of patients' natural volumetric gain.

Using A Disentangled Neural Network to Objectively Assess the Outcomes of Midfacial Surgery in Syndromic Craniosynostosis.

Advancements in artificial intelligence and the development of shape models that quantify normal head shape and facial morphology provide frameworks by which the outcomes of craniofacial surgery can be compared. In this work, we will demonstrate the use of the Swap Disentangled Variational Autoencoder (SD-VAE) to objectively assess changes following midfacial surgery. Our model is trained on a dataset of 1405 3D meshes of healthy and syndromic patients which was augmented using a technique based on spectral interpolation. Patients with a diagnosis of Apert and Crouzon syndrome who had undergone sub- or trans-cranial midfacial procedures utilising rigid external distraction were then interpreted using this model as the point of comparison. A total of 56 patients met our inclusion criteria, 20 with Apert and 36 with Crouzon syndrome. By using linear discriminant analysis to project the high-dimensional vectors derived by SD-VAE onto a 2D space, the shape properties of Apert and Crouzon syndrome can be visualised in relation to the healthy population. In this way, we are able to show how surgery elicits global shape changes in each patient. To assess the regional movements achieved during surgery, we use a novel metric derived from the Malahanobis distance to quantify movements through the latent space. Objective outcome evaluation, which encourages in-depth analysis and enhances decision making, is essential for the progression of surgical practice. We have demonstrated how artificial intelligence has the ability to improve our understanding of surgery and its effect on craniofacial morphology.

Using the SCAR-Q to Evaluate Morbidity of Scars in Craniosynostosis Repair.

While previous literature has investigated the psychosocial impact and aesthetic satisfaction associated with post-operative scarring for certain pediatric craniofacial conditions, the impact of the scar burden resulting from craniosynostosis surgery has not been adequately studied. SCAR-Q was shared with patients ages 8 and older. Thirty-two complete patient responses were recorded. SCAR-Q is a PROM that consists of three independent scales - appearance, symptoms, and psychosocial impact - associated with a scar. Mann-Whitney U, linear regression, and Pearson correlation tests were used to evaluate associations between the scales, in addition to patient characteristics such as sex and suture involvement. Mean ages at time of surgery and survey completion were 9.65 ± 10.10 months and 12.10 ± 3.92 years, respectively. Mean scale scores were 81.5 ± 17.9 for appearance, 86.8 ± 12.4 for symptoms, and 79.3 ± 25.7 for psychosocial impact. Higher patient dissatisfaction with scar appearance correlated with more scar-related symptoms (r = 0.389; p = 0.028) and a greater psychosocial impact (r = 0.725; p < 0.001). SCAR-Q scales did not significantly correlate with age at surgery, age at survey completion, type of synostosis, or type of surgery; however, female patients reported lower mean appearance (65.4 vs. 86.0; p = 0.012) and psychosocial impact (57.3 vs. 85.5; p = 0.010) scores when compared to their male counterparts. It is vital that surgeons discuss patients' aesthetic satisfaction following craniosynostosis surgery in order to appropriately address and limit deleterious, long-term physical and psychosocial outcomes.

Sociodemographic Factors Associated with Delayed Presentation in Craniosynostosis Surgery at a Tertiary Children's Hospital.

Craniosynostosis is a common diagnosis requiring early referral to a pediatric plastic surgeon; however, disparities in healthcare may influence presentation timing and affect treatment options and outcomes. This study aimed to explore sociodemographic factors contributing to delay in craniosynostosis surgical consultation. A retrospective cohort study of 694 California-based craniosynostosis patients at a tertiary children's hospital was performed from 2006 to 2023. State-specific Area Deprivation Index (ADI) and distance to the hospital were calculated using ZIP codes. Multivariate linear and logistic regressions considered race, insurance type, syndromic status, suture type, and ZIP code-based socioeconomic factors. Median age of presentation was 4.5 [interquartile range: 2.6-7.6] months with racial/ethnic breakdown of Hispanic/Latinx (41.2%), White (23.6%), Asian (3.7%), Black/African American (2.0%), or other/unreported (29.5%) with 58.4% having public insurance and an average distance to the hospital of 48.3 km. Median ADI was 5.4 [interquartile range: 4.0-7.1]. By linear regression, public insurance (P < 0.001) and higher ADI decile (P < 0.001) independently contributed to an older age of presentation. Patients with public insurance (odds ratio 1.90; P = 0.002) were more likely to present after 4 months of age. Patients who had public insurance or resided in more disadvantaged areas presented later for craniosynostosis surgical consultation. Eliminating disparities in these populations ensures more equitable access to surgical options and can improve patient outcomes.

Endoscopic surgery for craniosynostosis: a bibliometric analysis of primary studies.

Bibliometrics, a statistical method assessing the influence of scholarly works, was employed to analyze the evolution of endoscopic surgery for craniosynostosis. This comprehensive review followed PRISMA guidelines, sourcing data from PubMed, Embase, and Web of Science, focusing on clinical and surgical outcomes up to December 2023. We identified 1409 records, with 83 papers meeting inclusion criteria. The majority of studies originated from the Americas (79%), predominantly from the United States (75.9%). The typical study design was retrospective cohort studies (62%), involving a total of 8,175 patients with median research duration of 8years. Citation metrics indicated an average count of 38.9 per paper, with notable contributions from several key authors. A distinct increase in research was observed in recent years, particularly from 2011 onwards, peaking in 2022 and 2023. The top 10 most cited papers, largely emanating from the U.S., had a higher median patient number (103) compared to the overall median (53), suggesting their significant impact. These papers were primarily published in journals with higher impact factors and citation indicators. The most cited research was notably published in the Journal of Neurosurgery-Pediatrics. This analysis provides a comprehensive view of the field, highlighting the growing trend and clinical importance of endoscopic approaches in craniosynostosis, offering a valuable resource for future research and clinical practice.

Use of piezoelectric instrumentation in craniofacial surgery.

The use of piezoelectric instrumentation is increasingly recognized as an alternative to traditional bone-cutting techniques across a wide array of surgeries. Here, we provide an overview of the technique, including device principles, benefits, and drawbacks. We also review its use in craniofacial surgery. Piezoelectric surgery is a minimally invasive bone-cutting system with lower risk of damage to surrounding soft tissue structures. Indications for its use are rapidly expanding across multiple fields, including craniofacial surgery. To date, piezosurgical techniques have been most widely adopted and studied in the contexts of rhinoplasty, orthognathic surgery, and cranioplasty in craniosynostosis. Piezosurgery can facilitate more precise and consistent osteotomies while decreasing morbidities associated with traditional osteotomy techniques. Primary limitations include cost and concerns regarding increased operative times secondary to operator learning curves and decreased cutting efficiency. Piezoelectric surgery represents an alternative to traditional bone-cutting modalities to improve precision, consistency, and safety of osteotomies. Further research is needed to better understand the efficacy of the technique as well as potential for additional applications.

Application of Computerized Surgical Planning in Craniosynostosis Surgery.

Craniosynostosis, a medical condition characterized by premature fusion of one or multiple cranial sutures, has historically been treated through surgical correction. Computerized Surgical Planning (CSP) and three-dimensional (3D) modeling have gained significant popularity across craniofacial surgery. Through a collaborative effort between surgeons and engineers, it is now possible to virtually execute a surgical plan based on preoperative imaging using computed tomography scans. The CSP workflow involves several elements including virtual 3D modeling, CSP computer-aided surgical guide design, manufacturing of guides and templates, and intraoperative implementation. Through the gradual optimization of this workflow, it has been possible to achieve significant progress in the surgical process including improvements in the preoperative planning of complex craniosynostosis cases and reduction of intraoperative time. Furthermore, CSP and 3D modeling have had a positive impact on surgical simulation and residency training, along with patient education and counseling. This article summarizes the CSP workflow in the treatment of craniosynostosis and the implications of this treatment modality on medical trainee education and patient management.

A Transfusion Regimen With Same-donor Packed Red Blood Cells Reduces Exposure to Multiple Blood Donors in Craniosynostosis Surgery.

In major craniosynostosis surgery with moderate to severe blood loss, patients may be exposed to multiple donors. We have previously reported a method for reducing donor exposure using mixed pediatric units including plasma. To further reduce donor exposure, we used plasma-free divided pediatric units. The study aimed to investigate the feasibility of the new strategy for reducing donor exposure. This prospective observational study recruited children younger than 1 year who were scheduled for nonsyndromic craniosynostosis surgery. One adult red blood cell unit was divided into 4 equal units on the day before the operation for use intra- or postoperatively. Number of donor exposures, estimated blood loss, crystalloid, colloid, and blood product volumes, and coagulation parameters were evaluated. Nineteen infants were included. The mean estimated blood loss was 19 (3) mL/kg and the transfusion volume was 17 (7) mL/kg. The median donor exposure per patient was 1 (range, 1-3). During surgery, all infants received at least one DPU. Two infants received transfusions from more than one donor during the intraoperative period. In the first 24 hours postoperatively, 14 infants received transfusion; 10 received only DPUs, whereas 4 received from multiple donors. In all, multiple donor exposure was prevented in 14 of 19 infants. Postoperative Pk-INR was 1.33 (0.16); no plasma or platelets were transfused. The plasma-free DPU transfusion protocol may be useful to reduce donor exposure in open craniosynostosis surgery in infants.

Incomes to Outcomes: A Global Assessment of Disparities in Cleft and Craniofacial Treatment.

Recent investigations focused on health equity have enumerated widespread disparities in cleft and craniofacial care. This review introduces a structured framework to aggregate findings and direct future research. Systematic review was performed to identify studies assessing health disparities based on race/ethnicity, payor type, income, geography, and education in cleft and craniofacial surgery in high-income countries (HICs) and low/middle-income countries (LMICs). Case reports and systematic reviews were excluded. Meta-analysis was conducted using fixed-effect models for disparities described in three or more studies. N/A. Patients with cleft lip/palate, craniosynostosis, craniofacial syndromes, and craniofacial trauma. N/A. One hundred forty-seven articles were included (80% cleft, 20% craniofacial; 48% HIC-based). Studies in HICs predominantly described disparities (77%,) and in LMICs focused on reducing disparities (42%). Level II-IV evidence replicated delays in cleft repair, alveolar bone grafting, and cranial vault remodeling for non-White and publicly insured patients in HICs (Grades A-B). Grade B-D evidence from LMICs suggested efficacy of community-based speech therapy and remote patient navigation programs. Meta-analysis demonstrated that Black patients underwent craniosynostosis surgery 2.8 months later than White patients (P < .001) and were less likely to undergo minimally-invasive surgery (OR 0.36, P = .002). Delays in cleft and craniofacial surgical treatment are consistently identified with high-level evidence among non-White and publicly-insured families in HICs. Multiple tactics to facilitate patient access and adapt multi-disciplinary case in austere settings are reported from LMICs. Future efforts including those sharing tactics among HICs and LMICs hold promise to help mitigate barriers to care.

D151. Socioeconomic Factors Associated With Delayed Presentation in Craniosynostosis Surgery at a Tertiary Children’s Hospital

D151. Socioeconomic Factors Associated With Delayed Presentation in Craniosynostosis Surgery at a Tertiary Children’s Hospital

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

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. 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. 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. Open calvarial vault surgery or endoscopic repair for single-suture craniosynostosis. 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. 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. 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.

Sports participation after craniosynostosis repair: the critical role of post-operative guidance in parental decision-making.

Despite previous research supporting patient safety in sports after craniosynostosis surgery, parental anxiety remains high. This study sought to evaluate the role of healthcare providers in guiding patients and families through the decision-making process. Parents of children with repaired craniosynostosis were asked to assess sports involvement and parental decision-making in children ages 6 and older. Questions were framed primarily on 5-point Likert scales. Sport categorizations were made in accordance with the American Academy of Pediatrics. Chi-squared, linear regression, and Pearson correlation tests were used to analyze associations between the questions. Forty-three complete parental responses were recorded. Mean ages at surgery and time of sports entry were 7.93 ± 4.73months and 4.76 ± 2.14years, respectively. Eighty-two percent of patients participated in a contact sport. Discussions with the primary surgeon were more impactful on parental decisions about sports participation than those with other healthcare providers (4.04 ± 1.20 vs. 2.69 ± 1.32). Furthermore, children whose parents consulted with the primary surgeon began participating in sports at a younger age (4.0 ± 1.0 vs. 5.8 ± 2.7years, p = 0.034). The mean comfort level with contact sports (2.8 ± 1.4) was lower than that with limited-contact (3.8 ± 1.1, p = 0.0001) or non-contact (4.4 ± 1.3, p < 0.0001) sports. This study underscores the critical role that healthcare professionals, primarily surgeons, have in guiding families through the decision-making process regarding their children's participation in contact sports.

Tranexamic acid reduces intraoperative red blood cell transfusion rates in craniosynostosis surgery: a 6-year retrospective analysis

Surgery for craniosynostosis in children is associated with substantial intraoperative bleeding and the need for blood transfusions. Recent studies have supported the efficacy of tranexamic acid (TXA) in reducing blood loss and transfusion requirements. The records of 72 patients under 18 months of age who had undergone 113 surgeries for non-syndromic craniosynostosis in a single institution for 6 years were retrospectively analyzed. The transfusion requirements in infants with and without TXA administration were compared. The concept of patient blood management before and after TXA implementation in practice was also examined. The rate of intraoperative packed red blood cells (PRBC) transfusion was significantly lower in the TXA than in the non-TXA group (26.7% vs. 83%, p < 0.001). The volume of intraoperatively transfused PRBC was also lower in the TXA group compared to the non-TXA group (mean 68.25 mL vs. 101.91 mL, p < 0.001), as was the weight-adjusted volume (mean 11.2 mL/kg vs. 15 mL/kg, p = 0.002). There were no significant differences in the rate and volumes of postoperative PRBC transfusion between groups. The volume of intraoperatively transfused PRBC was dependent on TXA administration, but not on the affected suture and type of intervention. We found that while in the pre-TXA period all patients were transfused intraoperatively, the frequency of intraoperative transfusions in the post-TXA period was reduced by 40.9% even in the patients who had not received TXA. TXA seems to effectively reduce the intraoperative transfusion requirements in children undergoing craniosynostosis surgery. The optimal blood management in this patient population remains to be further evaluated.

Developmental outcomes of preschool children requiring craniosynostosis surgery in Manitoba: a cohort study.

Craniosynostosis is a congenital abnormality resulting in the premature fusion of one or more cranial sutures and usually requires surgical correction in the first year of life. While the majority of craniosynostosis cases represent isolated, nonsyndromic defects, approximately 25% are linked to a genetic diagnosis. Craniosynostosis has been associated with developmental delay. Therefore, historically in Manitoba, all children with surgically managed craniosynostosis were referred to the Child Development Clinic (CDC) for developmental assessment. There are currently prolonged wait times for neurodevelopmental assessments at CDC, leading to the need to better prioritize children who require neurodevelopmental assessment. The aim of the study was then to determine the developmental outcomes of preschool children requiring craniosynostosis surgery in Manitoba from July 1, 2016 through December 1, 2021. In this retrospective cohort study, clinical records were reviewed to evaluate the developmental outcomes of patients who were assessed at CDC for surgically managed craniosynostosis. Out of 67 children included in this study, 18% demonstrated global developmental delay, 24% demonstrated mild developmental delay, and 58% were developmentally appropriate across all levels. The presence of a genetic syndrome, multisutural craniosynostosis, and parental socioeconomic status were associated with risk of global developmental delay. The findings of this study suggest that while not all children with craniosynostosis present with developmental delay, those with suspected or confirmed syndromic involvement, multisutural cranyosinostosis, and/or low SES, should be referred for neurodevelopmental evaluation.

Tranexamic Acid: Safeguarding Children with Craniosynostosis from Bleeding. A Review Article.

Craniosynostosis is a congenital condition characterized by the premature closure of one or more sutures in the skull after birth, often necessitating urgent surgical intervention. Nevertheless, cranial vault reconstruction surgery can cause rapid and massive blood loss. This procedure requires a blood transfusion, which entails potential hazards. In addition, the hemostatic system in children differs significantly from that in adults, resulting in increased bleeding during surgical procedures. We conducted a comprehensive literature review in the PubMed, Scopus, and Web of Science databases, referring to their inception for studies on the use of tranexamic acid in pediatric craniosynostosis surgery. Selection criteria were based on the relevance of tranexamic acid, its clinical efficacy, and its safety profile in pediatric populations. Authoritative reviews were considered to ensure a comprehensive synthesis of current knowledge and practice trends in the field. We determined that a low loading dose of 10 mg/kg followed by a maintenance dose of 5 mg/kg/h is as effective as a high dose of 50 mg/kg followed by a maintenance dose of 10 mg/kg/h of tranexamic acid when administered after induction of anesthesia through skin closure and can reduce blood loss by up to 72% and total packed red blood cell transfusion by up to 85%. No difference in safety profile is observed. We concluded that a low dose of tranexamic acid, administered as a loading dose followed by a maintenance dose, is beneficial and safe for reducing blood loss and transfusion following craniosynostosis surgery.

Prospective Evaluation of Health-Related Quality-of-Life in Children with Craniosynostosis.

To investigate health-related quality of life (HRQL) in children aged 2 to 7 years, who have undergone surgery for craniosynostosis. Cross-sectional survey. A tertiary pediatric academic medical center. Children with craniosynostosis who underwent surgical correction, and who were 2-7 years old at the time of the study. Children from families that did not speak English were excluded. Caregivers were asked to fill out the Pediatric Quality of Life Inventory (PedsQL) Core Parent Report and the PedsQL Cognitive Functioning Scale. PedsQL: Psychosocial Health Summary Score, Physical Health Summary Score, Total Core Score, Cognitive Functioning Scale Score. Scores range from 0 to 100, with higher scores reflecting greater QoLSubject factors: comorbidities, syndromic status, type of craniosynostosis, type of surgery. The study included 53 subjects, of whom 13.2% had a syndrome. Core and cognitive scores did not depend on presence of a syndrome or suture involved. Subjects who underwent posterior cranial distraction achieved higher Total Core Scores than subjects who underwent open vault remodeling. Among subjects with sagittal craniosynostosis, there was a tendency for higher scores among children who underwent minimally-invasive surgery compared to those who underwent open vault remodeling. This study demonstrates similar HRQL among children with and without a syndrome, higher HRQL among children undergoing posterior cranial distraction than those undergoing open vault remodeling, and trends towards higher HRQL in children with sagittal craniosynostosis who underwent minimally-invasive surgery compared to those who underwent open vault remodeling.