Geospatial and Socioeconomic Disparities Influencing the Management of Craniosynostosis.

BACKGROUND: Various social determinants of health have been described as predictors of clinical outcomes for the craniosynostosis population. However, literature lacks a granular depiction of socioeconomic factors that impact these outcomes, and little is known about the relationship between patients' proximity to the care center and management of the condition. METHODS/DESIGN: This study retrospectively evaluated patients with craniosynostosis who presented to a tertiary children's hospital between 2000 and 2019. Outcomes of interest included age at presentation for surgery, incidence of reoperation, and length of follow-up. Patient addresses were geocoded and plotted on two separate shapefiles containing block group information within San Diego County. The shapefiles included percent parental educational attainment (bachelor's degree or higher) and median household income from 2010. The year 2010 was chosen for the shapefiles because it is the median year of data collection for this study. Multivariate linear, logistic, and polynomial regression models were used to analyze the relationship between geospatial and socioeconomic predictors and clinical outcomes. RESULTS: There were 574 patients with craniosynostosis included in this study. The mean ± SD Haversine distance from the patient's home coordinates to the hospital coordinates was 107.2 ± 321.2 miles. After adjusting for the suture fused and insurance coverage, there was a significant positive correlation between distance to the hospital and age at index surgery (P = 0.018). There was no correlation between distance and incidence of reoperation (P = 0.266) or distance and duration of follow-up (P = 0.369). Using the same statistical adjustments, lower parental percent educational attainment and lower median household income correlated with older age at index surgery (P = 0.008 and P = 0.0066, respectively) but were not correlated with reoperation (P = 0.986 and P = 0.813, respectively) or duration of follow-up (P = 0.107 and P = 0.984, respectively). CONCLUSIONS: The results offer evidence that living a greater distance from the hospital and socioeconomic disparities including parental education and median household income may serve as barriers to prompt recognition of diagnosis and timely care in this population. However, the geospatial and socioeconomic factors studied do not seem to hinder incidence of reoperation or length of follow-up, suggesting that, once care has been initiated, longitudinal outcomes may be less impacted.

Factors associated with late surgical correction of craniosynostosis: A decade-long review of the United States nationwide readmission database.

Late-repair craniosynostosis (LRC), defined as craniosynostosis surgery beyond 1 year of age, is often associated with increased complexity and potential complications. Our study analyzed data from the 2010-2019 Nationwide Readmissions Database to investigate patient factors related to LRC. Of 10 830 craniosynostosis repair cases, 17% were LRC. These patients were predominantly from lower-income families and had more comorbidities, indicating that socioeconomic status could be a significant contributor. LRC patients were typically treated at teaching hospitals and privately owned investment institutions. Our risk-adjusted analysis revealed that LRC patients were more likely to belong to the lowest-income quartile, receive treatment at privately owned investment hospitals, and use self-payment methods. Despite these challenges, the hospital stay duration did not significantly differ between the two groups. Interestingly, LRC patients faced a higher predicted mean total cost compared with those who had surgery before turning 1. This difference in cost did not translate to a longer length of stay, further emphasizing the complexity of managing LRC. These findings highlight the urgent need for earlier intervention in craniosynostosis cases, particularly in lower-income communities. The medical community must strive to improve early diagnosis and treatment strategies in order to mitigate the socioeconomic and health disparities observed in LRC patients.

64-Slice ECG-gated computed tomographic angiography for assessment of coronary arteries in brachycephalic dogs with pulmonary stenosis.

BACKGROUND: Brachycephalic dogs with pulmonary stenosis are known to have a higher incidence of concurrent coronary artery abnormalities than non-brachycephalic breeds, which increases risk when performing balloon valvuloplasty. The use of ECG-gated CT angiography has been reported for the evaluation of coronary arteries in normal dogs and dogs with pulmonary stenosis. The purpose of this study was to report findings of coronary artery origination and morphology of main branches using ECG-gated CT angiography in brachycephalic dogs with pulmonary stenosis. METHODS: An ECG-gated CT angiographic protocol was used to image coronary artery anatomy in nine brachycephalic dogs with pulmonary stenosis. Images were assessed for quality as well as coronary artery morphology by one veterinary radiologist, one veterinary cardiologist and one veterinary radiology resident. RESULTS: All nine dogs had good to excellent image quality. Coronary artery anomalies were identified in three of nine dogs: one R2A anomaly, one L2A anomaly and one L2C anomaly. Two dogs were assessed to be poor balloon valvuloplasty candidates based on CT angiographic images. LIMITATION: Coronary artery morphology was not confirmed via postmortem examination in all patients. CONCLUSION: ECG-gated CT angiography is a minimally invasive imaging modality capable of diagnosing various coronary artery anomalies in brachycephalic dogs with pulmonary stenosis and aiding in the determination of patient candidacy for balloon valvuloplasty.

Neurodevelopmental assessment of normocephalic children born to Zika virus exposed and unexposed pregnant people.

BACKGROUND: Studies examining the association between in utero Zika virus (ZIKV) exposure and child neurodevelopmental outcomes have produced varied results. METHODS: We aimed to assess neurodevelopmental outcomes among normocephalic children born from pregnant people enrolled in the Zika in Pregnancy in Honduras (ZIPH) cohort study, July-December 2016. Enrollment occurred during the first prenatal visit. Exposure was defined as prenatal ZIKV IgM and/or ZIKV RNA result at enrollment. Normocephalic children, >6 months old, were selected for longitudinal follow-up using the Bayley Scales of Infant and Toddler Development (BSID-III) and the Ages & Stages Questionnaires: Social-Emotional (ASQ:SE-2). RESULTS: One hundred fifty-two children were assessed; after exclusion, 60 were exposed and 72 were unexposed to ZIKV during pregnancy. Twenty children in the exposed group and 21 children in the unexposed group had a composite score <85 in any of the BSID-III domains. Although exposed children had lower cognitive and language scores, differences were not statistically significant. For ASQ:SE-2 assessment, there were not statistically significant differences between groups. CONCLUSIONS: This study found no statistically significant differences in the neurodevelopment of normocephalic children between in utero ZIKV exposed and unexposed. Nevertheless, long-term monitoring of children with in utero ZIKV exposure is warranted. IMPACT: This study found no statistically significant differences in the neurodevelopment in normocephalic children with in utero Zika virus exposure compared to unexposed children, although the exposed group showed lower cognitive and language scores that persisted after adjustment by maternal age and education and after excluding children born preterm and low birth weight from the analysis. Children with prenatal Zika virus exposure, including those normocephalic and have no evidence of abnormalities at birth, should be monitored for neurodevelopmental delays. Follow-up is important to be able to detect developmental abnormalities that might not be detected earlier in life.

The Enhanced Recovery After Surgery protocol for the surgical management of craniosynostosis: Lausanne experience.

OBJECTIVE: Over the past decade, the Enhanced Recovery After Surgery (ERAS) program has demonstrated its effectiveness and efficiency in improving postoperative care and enhancing recovery across various surgical fields. Preliminary results of ERAS protocol implementation in craniosynostosis surgery are presented. METHODS: An ERAS protocol was developed and implemented for cranial pediatric neurosurgery, focusing on craniosynostosis repair. The study incorporated a pre-ERAS group consisting of a consecutive series of patients who underwent craniosynostosis repair surgery prior to the implementation of the ERAS protocol; the results were compared with a consecutive group of patients who had been prospectively collected since the introduction of the ERAS for craniosynostosis protocol. The safety, feasibility, and efficiency of the ERAS protocol in pediatric neurosurgery was evaluated, through the collection of clinical data from the pre-, intra-, and postoperative phase. Surgery-related complications were evaluated according to the Clavien-Dindo classification. Costs of the stays were obtained using a microcosting approach. RESULTS: A total of 35 pre-ERAS patients and 10 ERAS patients were included. Scaphocephaly was the most common pathology in both groups. The overall compliance with the pre-, intra-, and postoperative criteria significantly increased-from 35.5%, 64.4%, and 54.7%, respectively, in each phase to 94%, 90%, and 84% (p < 0.001). The authors noticed a reduction in the average opioid dose used per patient in the ERAS group (p = 0.004), and they observed a trend toward a decreased mean length of stay from 5.2 days in the pre-ERAS group to 4.6 days in the ERAS group, without an increase of the rate of readmission within 30 days of surgery. The rate of complications decreased but this difference was not statistically significant. The hospital costs lowered significantly: from 21,958 Confederatio Helvetica Francs (CHF) in the pre-ERAS group to 18,936 CHF in the ERAS group (p = 0.02). CONCLUSIONS: The ERAS protocol represents a safe and cost-effective tool for the perioperative management of craniosynostosis. It showed its positive impact on the analgesia provided and on the reduction of in-hospital costs for these patients. ERAS protocols may thus be interesting options in the pediatric neurosurgical field.

BrachySound: machine learning based assessment of respiratory sounds in dogs.

The early and accurate diagnosis of brachycephalic obstructive airway syndrome (BOAS) in dogs is pivotal for effective treatment and enhanced canine well-being. Owners often do underestimate the severity of BOAS in their dogs. In addition, traditional diagnostic methods, which include pharyngolaryngeal auscultation, are often compromised by subjectivity, are time-intensive and depend on the veterinary surgeon's experience. Hence, new fast, reliable assessment methods for BOAS are required. The aim of the current study was to use machine learning techniques to bridge this scientific gap. In this study, machine learning models were employed to objectively analyze 366 audio samples from 69 Pugs and 79 other brachycephalic breeds, recorded with an electronic stethoscope during a 15-min standardized exercise test. In classifying the BOAS test results as to whether the dog is affected or not, our models achieved a peak accuracy of 0.85, using subsets from the Pugs dataset. For predictions of the BOAS results from recordings at rest in Pugs and various brachycephalic breeds, accuracies of 0.68 and 0.65 were observed, respectively. Notably, the detection of laryngeal sounds achieved an F1 score of 0.80. These results highlight the potential of machine learning models to significantly streamline the examination process, offering a more objective assessment than traditional methods. This research indicates a turning point towards a data-driven, objective, and efficient approach in canine health assessment, fostering standardized and objective BOAS diagnostics.

Racial differences in the care of pediatric sagittal craniosynostosis: a single-institution cohort study affecting state Medicaid policy.

OBJECTIVE: Although research has shown the cost-effectiveness of endoscopic versus open repair of sagittal synostosis, few studies have shown how race, insurance status, and area deprivation impact care for these patients. The authors analyzed data from children evaluated for sagittal synostosis at a single institution to assess how socioeconomic factors, race, and insurance status affect the surgical treatment of this population. They hypothesized that race and indicators of disadvantage negatively impact workup and surgical timing for craniosynostosis surgery. METHODS: Medical records of patients treated for sagittal synostosis between 2010 and 2019 were reviewed. Area deprivation index (ADI) and rural-urban commuting area codes, as well as median income by zip code, were used to measure neighborhood disadvantage. Black and White patients were compared as well as patients using Medicaid versus private insurance. RESULTS: Fifty patients were prospectively included in the study. Thirty-one underwent open repair; 19 had endoscopic repair. All 8 (100%) Black patients had open repair, compared to 54.8% of White patients (p = 0.018). Black patients were more likely to use Medicaid compared to White patients (75.0% vs 28.6%, p = 0.019). White patients were younger at surgery (5.5 vs 10.0 months, p = 0.001), and Black patients had longer surgeries (147.5 minutes vs 110.0 minutes, p = 0.021). The median household income by zip code was similar for the two groups. Black patients were generally from areas of greater disadvantage compared to White patients, based on both state and national ADI scores (state: 7.5 vs 4.0, p = 0.013; national: 83.5 vs 60.0, p = 0.013). All (94.7%) but 1 patient undergoing endoscopic repair used private insurance compared to 14 (45.2%) patients in the open repair group (p = 0.001). Patients using Medicaid were from areas of greater disadvantage compared to those using private insurance by both state and national ADI scores (state: 6.0 vs 3.0, p = 0.001; national: 75.0 vs 52.0, p = 0.001). CONCLUSIONS: Because Medicaid in the geographic region of this study did not cover helmeting after endoscopic repair of sagittal synostosis, these patients usually had open repair, resulting in significant racial and socioeconomic disparities in treatment of sagittal synostosis. This research has led to a change in Alabama Medicaid policy to now cover the cost of postoperative helmeting.

Impact of neighborhood deprivation and social vulnerability on long-term outcomes and desire for revision in patients with craniosynostosis.

OBJECTIVE: The authors utilized the area deprivation index (ADI), a validated composite measure of socioeconomic disadvantage, and the social vulnerability index (SVI) to examine whether differences in neighborhood deprivation impact interventions and outcomes among patients with craniosynostosis. METHODS: Patients who underwent craniosynostosis repair between 2012 and 2017 were included. The authors collected data about demographic characteristics, comorbidities, follow-up visits, interventions, complications, desire for revision, and speech, developmental, and behavioral outcomes. National percentiles for ADI and SVI were determined using zip and Federal Information Processing Standard (FIPS) codes. ADI and SVI were analyzed by tertile. Firth logistic regressions and Spearman correlations were used to assess associations between ADI/SVI tertile and outcomes/interventions that differed on univariate analysis. Subgroup analysis was performed to examine these associations in patients with nonsyndromic craniosynostosis. Differences in length of follow-up among the nonsyndromic patients in the different deprivation groups were assessed with multivariate Cox regressions. RESULTS: In total, 195 patients were included, with 37% of patients in the most disadvantaged ADI tertile and 20% of patients in the most vulnerable SVI tertile. Patients in more disadvantaged ADI tertiles were less likely to have physician-reported desire (OR 0.17, 95% CI 0.04-0.61, p < 0.01) or parent-reported desire (OR 0.16, 95% CI 0.04-0.52, p < 0.01) for revision, independent of sex and insurance status. In the nonsyndromic subgroup, inclusion in a more disadvantaged ADI tertile was associated with increased odds of speech/language concerns (OR 4.42, 95% CI 1.41-22.62, p < 0.01). There were no differences in interventions received or outcomes among SVI tertiles (p ≥ 0.24). Neither ADI nor SVI tertile was associated with risk of loss to follow-up among nonsyndromic patients (p ≥ 0.38). CONCLUSIONS: Patients from the most disadvantaged neighborhoods may be at risk for poor speech outcomes and different standards of assessment for revision. Neighborhood measures of disadvantage represent a valuable tool to improve patient-centered care by allowing for modification of treatment protocols to meet the unique needs of patients and their families.

The epidemiology of upper respiratory tract disorders in a population of insured Swedish dogs (2011-2014), and its association to brachycephaly.

Upper respiratory tract (URT) disorders are common in dogs but neither general nor breed-related epidemiological data are widely reported. This study´s aims were to describe the epidemiology of URT disorders in a Swedish population of dogs and to investigate whether brachycephalic breeds were overrepresented among high-risk breeds. A cohort of dogs insured by Agria Djurförsäkring in Sweden (2011-2014) was used to calculate overall and breed-specific incidence rate (IR), age at first URT diagnosis and relative risk (RR) for URT disorders. For breeds with high RR for URT disorders, co-morbidities throughout the dog's insurance period and age at death were investigated. The cohort included approximately 450,000 dogs. URT disorders had an overall IR of 50.56 (95% CI; 49.14-52.01) per 10,000 dog years at risk. Among 327 breeds, the English bulldog, Japanese chin, Pomeranian, Norwich terrier and pug had highest RR of URT disorders. Eight of 13 breeds with high RR for URT disorders were brachycephalic. The median age at first URT diagnosis was 6.00 years (interquartile range 2.59-9.78). French bulldogs with URT diagnoses had a significantly shorter life span (median = 3.61 years) than other breeds with URT diagnosis (median = 7.81 years). Dogs with high risk for URT disorders had more co-morbidities than average.

Clinical metrics and tools for provider assessment and tracking of trigonocephaly.

OBJECTIVE: Quantitative measurements of trigonocephaly can be used to characterize and track this phenotype, which is associated with metopic craniosynostosis. Traditionally, trigonocephaly metrics were extracted from CT scans; however, this method exposes patients to ionizing radiation. Three-dimensional optical scans are another option but are not routinely available in most outpatient settings. Recently, the authors developed semiautomated artificial intelligence algorithms that extract craniometric data from orthogonal 2D photographs. Although 2D photographs are safe, inexpensive, and straightforward to obtain, the accuracy of photograph-based craniometrics in comparison to CT and 3D optical scan correlates has not been established. In this study the authors compared the classification power of 2D photograph-based metrics of trigonocephaly with four CT-based metrics and one 3D optical scan-based metric in a heterogeneous series of patients who presented to an outpatient craniofacial clinic. METHODS: In this study the authors performed retrospective craniometric analyses of patient 2D photographs, 3D optical scans, and CT scans. Imaging-derived craniometrics include the 2D photograph-based anterior arc angle (AAA2D-photo), anterior-posterior ratio (APR2D-photo), and anterior-middle ratio (AMR2D-photo); the CT-based anterior arc angle (AAACT), metopic index (MICT), endocranial-bifrontal angle (eBFACT), and interfrontal angle (IFACT); and the 3D optical scan-based anterior arc angle (AAA3D-optical). Receiver operating characteristics (ROCs) were used to identify craniometrics strongly descriptive of trigonocephaly. Interrater comparisons were made between paired trigonocephaly measurements obtained from photographs and either CT scans or 3D optical scans. RESULTS: There were 13 photograph-based and CT-based pairs and 22 paired measurements from 2D photographs and 3D optical scans. AAA displayed the strongest classification capacity across all three imaging modalities. Significant agreement was observed between AAACT and AAA2D-photo (intraclass correlation coefficient [ICC] = 0.68 [95% CI 0.24-0.89], p = 0.0035), and AAA3D-optical and AAA2D-photo (ICC = 0.70 [95% CI 0.41-0.87], p < 0.0001). There was no significant correlation between APR2D-photo or AMR2D-photo and conventional CT-based metrics describing longitudinal width ratios (MICT). CONCLUSIONS: Photograph-based craniometrics are powerful tools that can be used to quantify the severity of trigonocephaly and exhibit high concordance with standard measurements derived from CT scans and 3D optical scans. The authors developed and freely share a research-use application to calculate trigonocephaly metrics from 2D photographs. Given the availability of digital photography, lack of ionizing radiation, and low cost of photograph-based craniometric derivation, this technique may be useful to supplement routine ambulatory care and objectively track outcomes following treatment.

An Esthetic Scoring System for Scaphocephaly Assessment and Outcomes: A Pilot Study.

Scaphocephaly is the commonest form of craniosynostosis with a varied presentation consisting of many morphological components and a range of possible surgical interventions. However, with regard to esthetic assessment, there is no universally applied assessment system. The aim was to develop a simple assessment tool encompassing multiple phenotypic components of scaphocephaly. This was done by piloting a red/amber/green (RAG) scoring system to judge esthetic outcomes following scaphocephaly surgery using photographs and experienced observers. Standard photographic views of 20 patients who had undergone either passive or anterior 2/3 vault remodelling were scored by 5 experienced assessors. Using a RAG scoring system before and after scaphocephaly correction according to 6 morphological characteristics: visual impression of cephalic index, calvarial height, bitemporal pinching, frontal bossing, posterior bullet, and displacement of the vertex. All 5 assessors were asked to score the preoperative and postoperative views independently. The RAG scores were each assigned a number (1-3) and added to give a composite score (range 6-18) and these were averaged between the 5 assessors. There was a highly statistically significant difference between both preoperative and postoperative composite scores ( P <0.0001). A subgroup analysis of the postoperative composite score between the 2 surgical techniques showed no significant difference ( P =0.759). The RAG scoring system can be used to assess esthetic change following scaphocephaly correction and it provides both a visual analogue and a numerical indicator of change. This assessment method needs further validation but is a potentially reproducible way to score and compare esthetic outcomes in scaphocephaly correction.

Results of training with a low-cost simulation model for endoscope-assisted scaphocephaly repair.

OBJECTIVE: Endoscope-assisted repair of sagittal craniosynostosis is an effective technique that requires a learning curve. Surgical simulation models can be applied to acquire the necessary skills for this procedure. Several models with a wide range of costs have been described for training in this technique. The aim of this work was to present the results of training with a low-cost simulation model for endoscope-assisted sagittal craniosynostosis repair. METHODS: A simulation model for sagittal craniosynostosis was developed using low-cost materials. The model is easily assembled and allows successive uses. Three neurosurgery residents, 3 fellows, and 2 neurosurgeons performed a 4-session training program in sagittal craniosynostosis repair. The Global Rating Scale (GRS) score, number of errors, and the time required to perform the task were reported by 2 independent evaluators using a checklist. Measurements were compared between the first and last training using the Wilcoxon signed-rank test. All participants completed a questionnaire (5-point Likert scale) regarding the realism of the simulation model. RESULTS: A model was developed to recreate the steps required to perform an endoscope-assisted scaphocephaly repair with the patient in a simulated sphinx position. All participants improved their GRS performance between the first and final training. The median time needed to perform the initial training was 47.5 minutes (interquartile range [IQR] 44.5-48 minutes, interrater difference [IRD] p = 0.77), and for the last training was 40.5 minutes (IQR 35.5-43 minutes, IRD p > 0.99). The median number of errors reported in the initial training was 5.5 (IQR 3-7.75 errors, IRD p = 0.8), and in the last training was 1 (IQR 0.75-2.25 errors, IRD p = 0.35). There was a statistically significant difference regarding the time and number of errors between the initial and final training (p < 0.001). More than 85% of the participants found that the surface anatomy, skull and anterior fontanel, fused sagittal suture, and epidural space of the model were realistic and had appropriate detail required to perform the surgery. All respondents agreed or strongly agreed that the endoscope handling was realistic, and that the steps and skills required to complete the task were representative of those required for the real procedure. CONCLUSIONS: A low-cost sagittal craniosynostosis simulation model was developed, allowing successive uses. The acquisition of skills within the simulation was demonstrated for all participants regarding the GRS score and the number of errors and time needed to perform the task. In addition, the model was found to be realistic in terms of anatomical references and the procedural steps required for this minimally invasive technique.

Assessment of the number of mast cells in the soft palate of dogs affected by brachycephalic obstructive airway syndrome.

BACKGROUND: Oedema is described in the soft palate of dogs affected by brachycephalic obstructive airway syndrome (BOAS). Activated mast cells (MCs) release vasoactive mediators that temporarily increase vascular permeability. METHODS: Data and caudal soft palate tissue were prospectively collected from a population of dogs undergoing surgical management of BOAS and a control group of greyhound cadavers with no previous history of respiratory signs. Histological assessment was performed to quantify the number of MCs within the lamina propria of each group. RESULTS: The mean number of MCs in the BOAS group (53 MCs/10 400× high-power fields [HPF]; standard deviation [SD] = 23) was significantly greater than that in the greyhound group (24 MCs/10 400×HPF; SD = 10). LIMITATIONS: The small size of the control group and the heterogeneous nature of the dogs in the BOAS group limit the generalisability of the findings. The use of different surgical techniques in the BOAS group may have also affected the degree of inflammation present within the samples. The cohort was not screened for concurrent disease processes that could potentially increase the number of circulating MCs. CONCLUSION: This study demonstrated a statistically significant difference between the numbers of MCs in the soft palate of brachycephalic dogs with clinically significant BOAS and the greyhound control group.

Three-Dimensional Assessment of Frontal Bossing and Temporal Pinching in Patients with Sagittal Craniosynostosis Using Curvature Analysis.

BACKGROUND: Sagittal craniosynostosis results in varying degrees of frontal bossing and bilateral temporal pinching. This study assessed the three-dimensional changes in these regions using curvature analysis and volumetric analysis before and 1 year after extended sagittal strip craniectomy (ESC) with postoperative helmet therapy. METHODS: A retrospective review of three-dimensional photographs of 50 subjects treated with ESC with postoperative helmet therapy and 50 age-matched controls was performed. Images were collected preoperatively and 1 year postoperatively. Forehead convexity and temple concavity were quantified. Computed tomographic scans of subjects with and without sagittal synostosis were analyzed to assess the percentage of total intracranial volume (ICV) in the anterior cranial fossa before and after ESC with postoperative helmet therapy. RESULTS: Forehead convexity in the ESC with postoperative helmet therapy group preoperatively (24.49 ± 3.16 m -1 ) was significantly greater than controls (22.48 ± 3.84 m -1 ; P = 0.005). Forehead convexity significantly decreased after ESC with postoperative helmet therapy (18.79 ± 2.43 m -1 ; P < 0.001) and did not differ from controls (19.67 ± 3.08 m -1 ; P = 0.115). The ESC group had more concave temples preoperatively (-10.27 ± 4.37 m -1 ) as compared with controls (-6.99 ± 3.55 m -1 ; P < 0.001). Temple concavity significantly decreased after ESC (-4.82 ± 3.17 m -1 ; P < 0.001) and did not differ from controls (-5.64 ± 3.27 m -1 ; P = 0.075). In the ESC group, the percentage ICV in the anterior cranial fossa decreased from 22.03% to 18.99% after surgery, whereas the anterior volume in controls was stable (17.74% to 16.81%). CONCLUSIONS: The ESC group had significantly greater forehead convexity, temple concavity and anterior cranial fossa volume compared with controls. One year after ESC with postoperative helmet therapy, forehead convexity, temple concavity, and percentage ICV in the anterior fossa were comparable to controls. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Longitudinal Assessment of Neurodevelopment in Patients With Nonsyndromic Single-Suture Craniosynostosis: A Retrospective Review of 66 Patients.

The neurodevelopmental consequences of nonsyndromic single-suture (NSS) craniosynostosis are the subject of continued debate. Although the predictive validity of the Bayley Scales of Infant and Toddler Development (Third Edition) (BSID-III) have been questioned, this neurodevelopmental testing battery continues to be widely utilized among multidisciplinary craniofacial teams. The purpose of this study is to evaluate the neurodevelopmental functioning of patients with NSS craniosynostosis before and after surgical correction and the impact of surgical correction on neurodevelopmental trajectory based on BSID-III testing. All patients with NSS craniosynostosis who underwent cranial vault remodeling between 2009 and 2020 were considered for inclusion. Patients who failed to complete BSID-III testing within 2 months of surgery preoperatively and 2 years of surgery postoperatively were excluded. A total of 66 patients met criteria for the study. On language testing, both the preoperative mean score ( P =0.007) and postoperative mean score ( P =0.003) were significantly lower than the population norm. Furthermore, on motor testing, both the preoperative mean score ( P =0.005) and postoperative mean score ( P =0.001) were significantly lower than the population norm. Bayley Scales of Infant and Toddler Development (Third Edition) testing revealed no significant change between preoperative and postoperative neurodevelopmental functioning. Overall, this study suggests that patients with NSS craniosynostosis experience modest delays in language and motor development, which are present before and after cranial vault remodeling. In addition, this study provides evidence that cranial vault remodeling does not significantly impact the neurodevelopmental trajectory. Multicenter st udies and refined neurodevelopmental testing methods are necessary to definitively establish the neurodevelopmental implications of NSS craniosynostosis.

"Validation of Artificial Intelligence Severity Assessment in Metopic Craniosynostosis".

OBJECTIVE: Several severity metrics have been developed for metopic craniosynostosis, including a recent machine learning-derived algorithm. This study assessed the diagnostic concordance between machine learning and previously published severity indices. DESIGN: Preoperative computed tomography (CT) scans of patients who underwent surgical correction of metopic craniosynostosis were quantitatively analyzed for severity. Each scan was manually measured to derive manual severity scores and also received a scaled metopic severity score (MSS) assigned by the machine learning algorithm. Regression analysis was used to correlate manually captured measurements to MSS. ROC analysis was performed for each severity metric and were compared to how accurately they distinguished cases of metopic synostosis from controls. RESULTS: In total, 194 CT scans were analyzed, 167 with metopic synostosis and 27 controls. The mean scaled MSS for the patients with metopic was 6.18 ± 2.53 compared to 0.60 ± 1.25 for controls. Multivariable regression analyses yielded an R-square of 0.66, with significant manual measurements of endocranial bifrontal angle (EBA) (P = 0.023), posterior angle of the anterior cranial fossa (p < 0.001), temporal depression angle (P = 0.042), age (P < 0.001), biparietal distance (P < 0.001), interdacryon distance (P = 0.033), and orbital width (P < 0.001). ROC analysis demonstrated a high diagnostic value of the MSS (AUC = 0.96, P < 0.001), which was comparable to other validated indices including the adjusted EBA (AUC = 0.98), EBA (AUC = 0.97), and biparietal/bitemporal ratio (AUC = 0.95). CONCLUSIONS: The machine learning algorithm offers an objective assessment of morphologic severity that provides a reliable composite impression of severity. The generated score is comparable to other severity indices in ability to distinguish cases of metopic synostosis from controls.

CT-like MR-derived Images for the Assessment of Craniosynostosis and other Pathologies of the Pediatric Skull.

PURPOSE: To evaluate the diagnostic value of CT-like images based on a 3D T1-weighted spoiled gradient echo-based sequence (T1SGRE) for the visualization of the pediatric skull and the identification of pathologies, such as craniosynostosis or fractures. METHODS: In this prospective study, 20 patients with suspected craniosynostosis (mean age 1.26 ± 1.38 years, 10 females) underwent MR imaging including the T1SGRE sequence and 2 more patients were included who presented with skull fractures (0.5 and 6.3 years, both male). Additionally, the skull of all patients was assessed using radiography or CT in combination with ultrasound. Two radiologists, blinded to the clinical information, evaluated the CT-like images. The results were compared to the diagnosis derived from the other imaging modalities and intraoperative findings. Intrarater and interrater agreement was calculated using Cohen's κ. RESULTS: Of the 22 patients 8 had a metopic, 4 a coronal and 2 a sagittal craniosynostosis and 2 patients showed a complex combination of craniosynostoses. The agreement between the diagnosis based on the T1SGRE and the final diagnosis was substantial (Cohen's κ = 0.92, 95% confidence interval (CI) 0.77-1.00 for radiologist 1 and κ = 0.76, CI 0.51-1.00 for radiologist 2). Of the patients with fractures, one presented with a ping pong fracture and one with a fracture of the temporal bone. Both radiologists could identify the fractures using the T1SGRE. CONCLUSION: The visualization of the pediatric skull and the assessment of sutures using a CT-like T1SGRE MR-sequence is feasible and comparable to other imaging modalities, and thus may help to reduce radiation exposure in pediatric patients. The technique may also be a promising imaging tool for other pathologies, such as fractures.

Racial and Socioeconomic Disparities in Prompt Craniosynostosis Workup and Treatment.

The purpose of this study was to identify racial and socioeconomic disparities in craniosynostosis evaluation and treatment, from referral to surgery. Patients diagnosed with craniosynostosis between 2012 and 2020 at a single center were identified. Chart review was used to collect demographic variables, age at referral to craniofacial care, age at diagnosis, age at surgery, and surgical technique (open versus limited incision). Multivariable linear and logistic regression models with lasso regularization assessed the independent effect of each variable. A total of 298 patients were included. Medicaid insurance was independently associated with a delay in referral of 83 days [95% confidence interval (CI) 4-161, P=0.04]. After referral, patients were diagnosed a median of 21 days later (interquartile range 7-40), though this was significantly prolonged in patients who were not White (ß 23 d, 95% CI 9-38, P=0.002), had coronal synostosis (ß 24 d, 95% CI 2-46, P=0.03), and had multiple suture synostosis (ß 47 d, 95% CI 27-67, P<0.001). Medicaid insurance was also independently associated with diagnosis over 3 months of age (risk ratio 1.3, 95% CI 1.1-1.4, P=0.002) and undergoing surgery over 1 year of age (risk ratio 3.9, 95% CI 1.1-9.4, P=0.04). In conclusion, Medicaid insurance was associated with a 3-month delay in referral to craniofacial specialists and increased risk of diagnosis over 3 months of age, limiting surgical treatment options in this group. Patients with Medicaid also faced a 4-fold greater risk of delayed surgery, which could result in neurodevelopmental sequelae.

[Assessment of clinical signs of brachycephalic obstructive airway syndrome and other breed-specific diseases in pug dogs - an online survey]. / Einschätzung von Symptomen des brachyzephalen obstruktiven Atemwegssyndroms und anderen rassetypischen Erkrankungen bei Mops-Hunden ­ eine Online-Umfrage.

OBJECTIVE: The aim of the study was to investigate the awareness for the breed-related brachycephalic obstructive airway syndrome (BOAS) and the occurrence of other breed-typical diseases within the framework of an online survey for pug owners. MATERIAL AND METHODS: A digital questionnaire for owners was created, distributed via social media and subsequently evaluated. RESULTS: The questionnaire was completed by 1220 pug owners. According to the owners, 32 % (344/1073) of the animals that did not undergo airway dilatation surgery show slight and 3 % (34/1073) show distinct breathing sounds when at rest. 86 % (326/378) of the owners perceive these breathing sounds as "normal, breed-specific" and 14 % (51/378) consider them as sign of "disease". 20 % (210/1073) of the animals are considered "somewhat" and 5 % (57/1073) "frequently tired and quickly short of breath" after a small amount of time. 24 % (245/1220) of all animals suffer from ocular diseases, 10 % (122/1220) from skin diseases and 11 % (134/1220) from spinal diseases, among others. CONCLUSION: The survey shows that with 67 % (814/1220) more than half of the pug owners perceive clinical signs of BOAS and/or other breed-specific diseases in their animals, however, a large proportion consider these as being non-problematic. CLINICAL RELEVANCE: The present study reveals that the animals' clinical limitations associated with brachycephaly are oftentimes not perceived as being pathologic and are hence underestimated by the owners.

A Low-Cost Simulation Model for Endoscopic-Assisted Sagittal Craniosynostosis Repair.

OBJECTIVE: The objective is to introduce a low-cost simulation model for endoscopic-assisted sagittal craniosynostosis repair in which bleedings can be simulated and to present the initial experience using the model as a learning tool. METHODS: A 3-dimensional synthetic skull was printed using polylactic acid. The brain, dura mater, sagittal sinus, and skin were constructed using low-cost materials. The simulation was performed at Garrahan Pediatric Hospital's simulation center employing a rigid endoscope (Pes Pilot HD 0° 4 mm). RESULTS: A low-cost simulation model for sagittal craniosynostosis was built in order to recreate the steps needed to perform a strip craniectomy with lateral extensions. The initial estimated cost is 10 U.S. dollars, and subsequent uses cost approximately 2 U.S. dollars. Four training sessions were performed, and a reduction in surgical time was recorded from 125 to 80 minutes. CONCLUSIONS: We introduce a low-cost simulation model for scaphocephaly endoscopic-assisted surgery in which bleeding can be reproduced. The initial experience shows the possibility of using the model as a learning tool.