Single-suture Craniosynostosis Research Articles

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.

Data-driven cranial suture growth model enables predicting phenotypes of craniosynostosis

We present the first data-driven pediatric model that explains cranial sutural growth in the pediatric population. We segmented the cranial bones in the neurocranium from the cross-sectional CT images of 2068 normative subjects (age 0–10 years), and we used a 2D manifold-based cranial representation to establish local anatomical correspondences between subjects guided by the location of the cranial sutures. We designed a diffeomorphic spatiotemporal model of cranial bone development as a function of local sutural growth rates, and we inferred its parameters statistically from our cross-sectional dataset. We used the constructed model to predict growth for 51 independent normative patients who had longitudinal images. Moreover, we used our model to simulate the phenotypes of single suture craniosynostosis, which we compared to the observations from 212 patients. We also evaluated the accuracy predicting personalized cranial growth for 10 patients with craniosynostosis who had pre-surgical longitudinal images. Unlike existing statistical and simulation methods, our model was inferred from real image observations, explains cranial bone expansion and displacement as a consequence of sutural growth and it can simulate craniosynostosis. This pediatric cranial suture growth model constitutes a necessary tool to study abnormal development in the presence of cranial suture pathology.

Patient-reported outcome measures more than fifteen years after treatment of sagittal or metopic craniosynostosis: a prospective cohort study

PurposeTo evaluate the long-term anthropometric measurements, cosmetic satisfaction, and other patient-reported outcome measures (PROMs) of patients who underwent surgical treatment or observation only of sagittal or metopic single-suture craniosynostosis (SSC).MethodsA prospective study was designed for all patients diagnosed with non-syndromic sagittal and metopic craniosynostosis at the British Columbia Children’s Hospital, Vancouver, Canada, in the period July 1986 to July 2006. After a minimum of 15 years post-diagnosis, all eligible patients were invited to fill out the Craniofacial Surgery Outcomes Questionnaire (CSO-Q) and to attend a scheduled follow-up appointment for the collection of anthropometric measurements. A descriptive analysis of the cosmetic results was performed. Statistical analyses compared the differences in anthropometric measurements between treated and non-treated patients.ResultsOf the 253 eligible patients, 52 participants were willing to share patient data for use in the study. Of those 52 former patients, 36 (69.2%) filled out and returned the CSO-Q and 23 (44.2%) attended the follow-up appointment. The mean follow-up period between surgical treatment and the CSO-Q was 20.2 ± 2.5 years and between surgical treatment and the follow-up appointment was 20.9 ± 2.7 years. In patients with sagittal SSC, the mean cephalic index (CI) was significantly larger in treated than in non-treated patients (74.6 versus 69.1, p = 0.04), while the mean pupillary distance and forehead to back index were significantly smaller (pupillary distance 6.0 cm versus 6.7 cm [p = 0.04] and forehead to back index 19.6 cm versus 21.1 cm [p = 0.03]). Focusing more on the patient reported outcome measures, overall cosmetic satisfaction was found to be high (80.6%) and no differences were found between sagittal and metopic synostosis patients, nor between treated or non-treated craniosynostosis patients. Overall outcomes regarding self-esteem (RSES) and fear of negative evaluation (FNE) were comparable with population based outcomes.ConclusionThis is the first prospective study of sagittal and metopic craniosynostosis patients regarding long-term anthropometric outcome and patient reported outcome measures, including patients who were treated surgically and those who received observation only. Although study participation two decades after initial diagnosis was difficult to obtain, our data provide a platform from which one can develop an inclusive and uniform approach to assess patients’ subjective cosmetic satisfaction using the CSO-Questionnaire and might be useful in preoperative counseling and psychosocial care for patients and their families.

Can Craniosynostosis be Diagnosed on Physical Examination? A Retrospective Review.

Craniosynostosis is a developmental craniofacial defect in which one or more sutures of the skull fuse together prematurely. Uncorrected craniosynostosis may have serious complications including elevated intracranial pressure, developmental delay, and blindness. Proper diagnosis of craniosynostosis requires a physical examination of the head with assessment for symmetry and palpation of sutures for prominence. Often, if craniosynostosis is suspected, computed tomography (CT) imaging will be obtained. Recent literature has posited that this is unnecessary. This study aims to address whether physical examination alone is sufficient for the diagnosis and treatment planning of single suture craniosynostosis. Between 2015 and 2022, the Divisions of Pediatric Neurosurgery and Pediatric Plastic Surgery at UTHealth Houston evaluated 140 children under 36 months of age with suspected craniosynostosis by physical examination and subsequently ordered CT imaging for preoperative planning. Twenty-three patients received a clinical diagnosis of multi-sutural or syndromic craniosynostosis that was confirmed by CT. One hundred seventeen patients were diagnosed with single suture craniosynostosis on clinical examination and follow-up CT confirmed suture fusion in 109 (93.2%) patients and identified intracranial anomalies in 7 (6.0%) patients. These patients underwent surgical correction. Eight (6.8%) patients showed no evidence of craniosynostosis on CT imaging. Treatment for patients without fused sutures included molding helmets and observation alone. This evidence suggests that physical examination alone may be inadequate to accurately diagnose single suture synostosis, and surgery without preoperative CT evaluation could lead to unindicated procedures.

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.

Discussion: Secondary Corrections for Single-Suture Craniosynostosis: Perioperative Outcomes and Predisposing Factors.

Discussion: Secondary Corrections for Single-Suture Craniosynostosis: Perioperative Outcomes and Predisposing Factors.

Physical and neurocognitive outcomes of children with primary Non-Syndromic Single-Suture craniosynostosis

IntroductionCraniosynostosis is a congenital anomaly disturbing children’s cognitive disability and motor status and appearance. The present study aimed to determine the physical and neurocognitive outcomes of children with primary non-syndromic single-suture craniosynostosis. MethodThe present cohort study started data collection from the Imam Hossein Hospital of Isfahan in 2019 and continued it until 2022. A total of 70 children with primary non-syndromic single-suture craniosynostosis were selected by the census method and then were included in the study. Children were examined before the treatment and up to one year after the surgery (1,3,6,12 and 15 months), and data were collected using a checklist. ResultsA total of 68 children with primary non-syndromic single-suture craniosynostosis were followed up in this study for 15 months. Among them, 72.1% were male, and the skull shapes were Scaphocephaly in 37 children (54.4%). The mean head circumference score increased from one month to 6 months; however, it decreased from 12 months onward. In each examination, the mean HC had a significant difference from the pre-operation stage. The 12-month follow-up indicated that 15 children (25.8%) had cognitive deficits. A total of 7 children (46.7%) had cognitive disability, 3 (20%) had motor problems, 3 (33.3%) had both problems, and 7 (31.8%) improved after the operation. Finally, the cranial index was significantly related to the intra-operative child's age. ConclusionAccording to the results, the cranial index is significantly associated with the intra-operative child age and it is probably a more suitable index than the head circumference to investigate the child's appearance.

Minimally invasive craniectomy and postoperative cranial remolding orthotic treatment in infants with craniosynostosis: A multicenter prospective study

ObjectiveTo investigate the effectiveness of minimally invasive suturectomy (MIS) with cranial remolding orthosis (CRO) for treatment of patients with single-suture craniosynostosis. MethodsIn this multicenter prospective study, all patients included underwent MIS followed by postoperative CRO. The values of the cephalic index (CI) and cranial vault asymmetry index (CVAI) were measured and recorded before the surgery, at initiation of CRO, and at the cessation of CRO. The total cranial volume (TV), anterior hemisphere cranial volume (AV), and AV to TV were also acquired with a non-contact optical scanner. All patients were followed up at least until the end of the twelfth month of age. ResultsA total of 38 patients were included. The average operative time including anesthesia was 95.38 minutes. The average length of hospital admission was 2.5 days. There was a statistically significant difference in CI values for the patients with sagittal craniosynostosis (p < 0.05). The improvement in CVAI values were statistically significant in all three groups of participants (p < 0.001). The AV and TV from initiation to cessation of CRO treatment were statistically significant in all three groups of participants (p < 0.001). Regarding the AV to TV ratio, there was a significant difference from initiation to the cessation of CRO treatment in sagittal and metopic groups of participants (p < 0.05). The average daily hours of CRO wearing was 18.54 hours. ConclusionsThe findings of this investigation complement those of earlier studies and showed that MIS with CRO treatment is a good strategy with satisfactory results for patients with single-suture craniosynostosis.

Surgical Timing and Neurocognitive Development among Patients with Craniosynostosis: Analysis of Confounders.

In 1988, Renier and Marchac asserted that children with craniosynostosis who undergo cranial vault remodeling (CVR) after 12 months of age experience delayed neurocognitive development compared to children who undergo CVR before 12 months of age. The purpose of this study was to identify factors potentially confounding this cause-and-effect relationship. The authors hypothesize that children with socioeconomic disadvantages or comorbid conditions are more likely to undergo CVR after 12 months and may represent a selection bias toward delayed neurocognitive development. Patients with nonsyndromic single-suture craniosynostosis who underwent CVR between 2009 and 2020 at Michigan Medicine were included ( n = 227). Sociodemographic and clinical variables were documented. The sample was dichotomized to compare patients who underwent CVR before (early) and after (late) 12 months of age. Statistical analysis was performed at P < 0.05 significance. The early and late groups contained 157 patients and 70 patients, respectively. Compared to the early group, the late group contained a larger proportion of patients who identified as non-White ( P = 0.03), qualified for need-based financial assistance ( P = 0.03), were born preterm ( P < 0.01), or had a comorbid condition ( P < 0.01). Based on preoperative testing, the late group contained a larger proportion of patients with baseline cognitive ( P < 0.001) and language ( P = 0.008) delays relative to the early group. This study demonstrates that socioeconomic disadvantages and comorbid conditions are prevalent among patients who undergo delayed CVR and may represent a selection bias toward delayed neurocognitive development. Future studies evaluating the relationship between surgical timing and neurocognitive development must control for these factors. Risk, II.

Transcriptomic Signatures of Single-Suture Craniosynostosis Phenotypes

Craniosynostosis is a birth defect where calvarial sutures close prematurely, as part of a genetic syndrome or independently, with unknown cause. This study aimed to identify differences in gene expression in primary calvarial cell lines derived from patients with four phenotypes of single-suture craniosynostosis, compared to controls. Calvarial bone samples (N = 388 cases/85 controls) were collected from clinical sites during reconstructive skull surgery. Primary cell lines were then derived from the tissue and used for RNA sequencing. Linear models were fit to estimate covariate adjusted associations between gene expression and four phenotypes of single-suture craniosynostosis (lambdoid, metopic, sagittal, and coronal), compared to controls. Sex-stratified analysis was also performed for each phenotype. Differentially expressed genes (DEGs) included 72 genes associated with coronal, 90 genes associated with sagittal, 103 genes associated with metopic, and 33 genes associated with lambdoid craniosynostosis. The sex-stratified analysis revealed more DEGs in males (98) than females (4). There were 16 DEGs that were homeobox (HOX) genes. Three TFs (SUZ12, EZH2, AR) significantly regulated expression of DEGs in one or more phenotypes. Pathway analysis identified four KEGG pathways associated with at least one phenotype of craniosynostosis. Together, this work suggests unique molecular mechanisms related to craniosynostosis phenotype and fetal sex.

Secondary Corrections for Single-Suture Craniosynostosis: Perioperative Outcomes and Predisposing Factors.

Many single-suture craniosynostosis corrections end up needing secondary remodeling procedures. The authors sought to determine whether these more complicated operations carry higher complication rates, and to explore potential predisposing factors. The authors performed a retrospective chart review of all patients undergoing primary and secondary remodeling corrections at a single center, between 2010 and 2020. Of 491 sequential single-suture corrections, 380 were primary procedures and 111 were secondary (89.2% initially treated elsewhere); 10.3% of primary procedures received allogeneic blood versus only 1.8% of secondary corrections ( p =0.005). Median hospital lengths of stays were similar between groups [primary, 2.0 days (interquartile range [IQR], 2 to 2); secondary, 2.0 days (IQR, 2 to 2)], as were surgical infection rates (primary, 0%; secondary, 0.9%). With respect to predisposing factors, the affected suture and presence of a genetic mutation did not appear predictive; however, the median age at primary correction was significantly younger for those needing second procedures [6.0 months (IQR, 4 to 9 months) versus 12.0 months (IQR, 11 to 16 months)]. An odds ratio estimate suggests that for every month increase in age, the odds of undergoing a reoperation drops 40%. With respect to surgical indications, concerns for raised intracranial pressure and skull defects were more commonly cited following strip craniectomies than remodeling procedures. This single-center review was unable to identify a higher risk profile for reoperations. In addition, analyses suggest that performing primary corrections at a younger age and, possibly, strip craniectomies were associated with a higher likelihood of a subsequent secondary correction. Therapeutic, III.

Multi-suture craniosynostosis in Sotos Syndrome: A case Report

Study Design Case report. Objective Sotos syndrome is a rare genetic disorder which is characterized by excessive physical growth during the first few years of life. The principal features of this condition include macrocephaly, learning disability and physical overgrowth. The current diagnosis of Sotos Syndrome is based on identification of genetic mutations and deletions in the NSD family (the Nuclear-receptor-binding SET-Domain containing protein). Single suture craniosynostosis has been reported in a few cases of Sotos syndrome. We report an atypical presentation of multi-suture craniosynostosis in a child with Sotos Syndrome. Methods The initial phenotypical presentation was consistent with left coronal craniosynostosis. She underwent strip suturectomy and orthotic helmeting with failure to correct her anterior plagiocephaly and orbital dysmorphology. Additional post-operative investigations revealed a multi-suture craniosynostosis. She subsequently underwent an anterior cranial vault reconstruction and fronto-orbital advancement. Conclusions Sotos Syndrome can be associated with single or multi-suture craniosynostosis. This is likely driven by the overgrowth status of this disease. In our case report, the patient presents with unicoronal craniosynostosis which progressed to multi-suture craniosynostosis. Therefore, a multidisciplinary approach and close monitoring by craniofacial surgeons, neurosurgeons, developmental paediatricians and other allied teams is required in the care of such patients.

Flexible endoscope-assisted suture release and barrel stave osteotomy for the correction of sagittal synostosis.

Early suturectomy with a rigid endoscope followed by orthotic cranial helmet therapy is an accepted treatment option for single-suture craniosynostosis. To the authors' knowledge, flexible endoscope-assisted suture release (FEASR) has not been previously described. Presented herein is their experience with FEASR for the treatment of isolated sagittal craniosynostosis. A retrospective analysis of the health records of patients who had undergone FEASR between March 2018 and December 2020 was performed. Patients under the age of 6 months who had been diagnosed with isolated sagittal synostosis were considered eligible for FEASR. Exclusion criteria included syndromic synostosis or multiple-suture synostosis. The cephalic index, the primary measure of the cosmetic endpoint, was calculated at prespecified intervals: immediately preoperatively and 6 weeks and 12 months postoperatively. Parental satisfaction with the cosmetic outcome was determined throughout the clinical follow-up and documented according to a structured questionnaire for the first 12 months. A total of 18 consecutive patients met the criteria for study inclusion. The mean patient age at the time of surgery was 3.4 months (range 2-6 months). All patients underwent a wide craniectomy with no need to convert to an open procedure. The mean craniectomy width was 3.61 cm. Estimated blood loss ranged from 5 to 30 ml. The mean operative time was 75 minutes. No intraoperative complications were observed. The average length of stay was 2.6 days. The mean cephalic index was 67.7 preoperatively, 77.1 at 6 weeks postoperatively, and 76.3 at 1 year postoperatively. The mean percentage change in the cephalic index from preoperatively to the 12-month follow-up was 10.44 (p < 0.001). The mean follow-up was 17 months (range 12-28 months). All parents were satisfied with the cosmetic outcome of the procedure. No patients developed symptoms of raised intracranial pressure (ICP) or needed invasive ICP monitoring during the follow-up period. No patients required reoperation. In this modest single-hospital series, the authors demonstrated the feasibility of FEASR in treating sagittal synostosis with favorable cosmetic outcomes. The morbidity profile and resource utilization of the procedure appear similar to those of procedures conducted via traditional rigid endoscopy.

Utilizing Computer-Assisted Design and Virtual Surgical Planning for Correction of Unilateral Lambdoid Synostosis.

Unilateral lambdoid synostosis is the rarest form of single-suture craniosynostosis. Although various surgical approaches have been described, cranial vault remodeling remains the predominant approach. To aid in surgical planning, preoperative virtual surgical modeling using a patient's presenting computed tomography scan can be used to increase reconstructive precision and to reduce operative time. Presented is a 7-month-old male with unilateral lambdoid synostosis who underwent medically modeled cranial vault reconstruction.

The Craniofacial Collaboration UK: Developmental Outcomes in 5-Year-Old Children With Metopic Synostosis.

The Craniofacial Collaboration (CC-UK) was setup in 2015 as a joint initiative between the Psychology teams attached to the 4 highly specialized craniofacial centers in the United Kingdom. The CC-UK aims to address key limitations in the existing craniofacial literature by applying strict exclusion criteria and collating clinical data on a homogenous sample of children. This article reports the fourth wave of data collection from the CC-UK, with the analysis of developmental and behavioral outcomes for children with metopic synostosis at 5 years old. Previous data for sagittal synostosis at 3 and 5 years, and metopic synostosis at 3 years, have been presented. This paper offers the first analysis of developmental and behavioral parent-report measures at school age for metopic synostosis. All children in the current data set had primary corrective surgery. Findings highlight similar patterns to that of previous research among children with single-suture craniosynostosis, as well as earlier CC-UK analyses, with the majority falling within 1 standard deviation of the normative mean. However, differences across key behavioral and emotional domains, with some areas reporting heightened concerns compared with those detected among sagittal groups, may suggest that subtle differences between individual diagnostic groups are present. This further highlights the importance of utilizing homogenous samples within the field of craniofacial research. To further build upon this work, and to provide a greater understanding of how these difficulties and concerns may develop, or diminish, over time, further consideration to longitudinal outcomes is needed for individual diagnostic groups. Through this collaboration, the authors seek to achieve this goal in their future work.

Aesthetic results in children with single suture craniosynostosis: proposal for a modified Whitaker classification

ObjectiveAesthetic assessment after surgery for non-syndromic single suture craniosynostosis (SSC) is crucial. Surgeons’ evaluation is generally based on Whitaker classification, while parental impression is generally neglected. The aim of this paper is to compare aesthetic perceptions of parents and surgeons after surgery for SSC, expressed by a 10-item questionnaire that complement Whitaker’s classification.MethodsThe authors submitted a 10-item questionnaire integrating Whitaker’s classification in order to evaluate the degree of satisfaction, the detailed aesthetics results and the need for surgical revision, to surgeons and parents of a consecutive series of patients operated for SSC between January 2007 and December 2018. The results were collected blindly.ResultsA total of 70 patients were included in the study. Scaphocephaly and trigonocephaly were the two most frequent craniosynostosis. Parents and surgeons general aesthetics evaluation and average rating for Whitaker’s classification were 1.86 vs 1.67 (p = 0.69) and 1.19 vs 1.1 (p = 0.45) respectively. Parents’ evaluation for scar perception and alopecia (p < 0.00001), the presence of bony crest (0.002), bony bump (p < 0.00001), or other bone irregularities (p = 0.02) are significantly worse when compared to surgeons’ perception.ConclusionsParents seem to be more sensitive to the detection of some aesthetic anomalies and their opinion should not be neglected. The authors propose a modified Whitaker classification based on their results to better stratify the aesthetic outcome after surgery for SSC.

Shared Decision-Making: Process for Design and Implementation of a Decision Aid for Patients With Craniosynostosis.

To describe the process of developing a craniosynostosis decision aid. We conducted a mixed-methods exploratory study between August 2019 and March 2020 to develop a decision aid about surgical treatment for single suture craniosynostosis. A single tertiary care academic children’s hospital. The decision aid development team consisted of surgeons, research fellows, a clinical nurse practitioner, clinical researchers with expertise in decision science, and a university-affiliated design school. Qualitative interviews (N = 5) were performed with families, clinicians (N = 2), and a helmeting orthotist to provide feedback on decision aid content, format, and usability. After cycles of revisions and iterations, 3 related decision aids were designed and approved by the marketing arm of our institution. Distinct booklets were created to enable focused discussion of treatment options for the 3 major types of single suture craniosynostosis (sagittal, metopic, unicoronal). Three decision aids representing the 3 most common forms of single suture craniosynostosis were developed. Clinicians found the decision aids could help facilitate discussions about families’ treatment preferences, goals, and concerns. We developed a customizable decision aid for single suture craniosynostosis treatment options. This tool lays the foundation for shared decision-making by assessing family preferences and providing clear, concise, and credible information regarding surgical treatment. Future research can evaluate this tool’s impact on patient–clinician discussions about families’ goals and preferences for treatment.

A Retrospective Analysis of the Impact of Health Disparities on Treatment for Single Suture Craniosynostosis Before and During the Pandemic.

Background Candidates for endoscopic treatment for craniosynostosis must be less than 6 months old. Given the narrow window of eligibility, there is potential for barriers to access to impact the type of surgery a patient receives. We hypothesized that COVID may worsen these potential disparities. Methods Charts were reviewed for children with single suture craniosynostosis from January 2014 to March 2020 (pre-COVID cohort) and March 2020 to September 2021 (COVID cohort) from 2 academic centers. Results 110 children were in the pre-COVID group; 56 were treated open and 54 endoscopically. Of the 44 patients in the COVID cohort, 20 were treated endoscopically and 24 were treated open. There was no significant difference in age of presentation or age of surgery across cohorts when considering type of surgery performed. Rate of endoscopy in the pre-COVID versus COVID cohorts was not significantly different. In both the pre-COVID and COVID cohorts there were significant differences between age of presentation, age of surgery, and type of surgery received based on race—black and Hispanic patients presented later, had surgery later, and underwent open surgery more frequently than their white/Asian counterparts (P = .0095, P = .0067). Conclusions The pandemic did not alter age of presentation/age of surgery or rates of patients receiving endoscopic surgery in patients with single suture craniosynostosis. There was no relationship between insurance status and type of surgery received during the pandemic, however Hispanic and black patients presented later and underwent surgery at an older age compared to white/Asian patients as they did pre-pandemic.

Epidemiology, Genetics, and Pathophysiology of Craniosynostosis.

Craniosynostosis, the premature fusion of the infant cranial skulls, can be recognized by characteristic head shape differences that worsen with head growth. Craniosynostosis can be syndromic or nonsyndromic and can involve one suture or multiple sutures. Timely cranial vault surgery is recommended to expand and reshape the skull, with a goal of preventing increased intracranial pressure and providing sufficient space for brain growth. Several gene variants and environmental exposures are known to increase the risk of single suture craniosynostosis (SSC), including in utero constraint, exposure to specific toxins and medications, and medical conditions such as thyroid dysregulation and metabolic bone disorders.

The utility and post-operative evolution of head circumference in nonsyndromic single-suture craniosynostosis: a prospective study in Vietnamese children.

Head circumference (HC) is an important clinical tool for following head growth in children with craniosynostosis (CS). The purpose of this study is to quantify the usefulness of HC along continuum of CS care, from diagnosis to pre- and post-operative (pre-op, post-op) follow-up in Vietnamese children. A prospective cohort of 54 nonsyndromic single-suture CS patients undergoing open surgery from January 2015 to January 2020 was collected at Children's Hospital 2, Vietnam. HC z-score on admission was compared with World Health Organization (WHO) standards to evaluate for utility in initial diagnosis. Pre-op and post-op HC were compared to demonstrate the evolution of head growth following reconstruction. Nonsyndromic single-suture CS was more predominant in males (79.6%) than in females (20.4%). The mean HC z-score was - 0.38 [Formula: see text] 1.29 similar to normal WHO standards regardless of which sutural involvement. The HC z-score increased above + 1 standard deviation (SD) significantly at 3months of follow-up (p < 0.001); however, the trajectory gradually decreased after the first year of surgery. One patient (1.8%, 1/54) demonstrated restenosis and delayed intracranial hypertension (DIH) 4years after reconstruction. The HC in nonsyndromic single-suture CS children presents similarly to the values of healthy children. Additionally, HC reliably increased after reconstruction and gradually normalized over subsequent years. This indicator is consistent in Southeast Asian populations and should be used to follow all patients to assess the normal progression of post-op head growth and as a useful indicator of suspected recurrent synostosis.