Natural history of mild trigonocephalic deformities.

OBJECTIVE: Ridging along the metopic suture line can be a common cause of concern for parents and has been theorized to represent a mild form of trigonocephaly, a cranial deformity associated with risks of negative cosmetic outcomes, if not surgically corrected. Yet the literature contains sparse reports of long-term cosmetic results or expectations for infants with isolated metopic ridging compared with those with severe trigonocephaly, or even what objective metrics discriminate isolated metopic ridging from severe trigonocephaly. Therefore, the authors' goals for this study were to 1) quantify the degree of frontal deformity among patients with metopic ridge, metopic craniosynostosis, and normocephalic head shapes; and 2) document the natural history of frontal deformities in isolated metopic ridge patients in the 1st year of life. METHODS: This was a retrospective cohort study of patients with normocephalic head shapes, metopic ridges, and metopic craniosynostoses who presented at < 1 year of age to the Connecticut Children's neurosurgery clinic from January 2019 to December 2021. Data were collected regarding demographics and photograph-based craniometrics. RESULTS: A total of 212 normocephalic, 34 metopic ridge, and 29 metopic craniosynostosis patients were included. Both the normocephalic and metopic ridge groups had a significantly higher anterior arc angle (AAA) value compared with the metopic craniosynostosis group (p < 0.0001). The AAA did not differ significantly among normocephalic patients and those with ridging. Over the course of 1 year of follow-up, patients with metopic ridging demonstrated a slight decrease in AAA values, but overall remained within the same range as normocephalic patients. CONCLUSIONS: Photograph-based craniometrics suggest that metopic ridge patients with frontal bone angulations > 2.2 radians have a mild degree of frontal constriction that does not significantly worsen over the 1st year of life.

Incorporation of a biparietal narrowing metric to improve the ability of machine learning models to detect sagittal craniosynostosis with 2D photographs.

OBJECTIVE: Sagittal craniosynostosis is the most common form of craniosynostosis and typically results in scaphocephaly, which is characterized by biparietal narrowing, compensatory frontal bossing, and an occipital prominence. The cephalic index (CI) is a simple metric for quantifying the degree of cranial narrowing and is often used to diagnose sagittal craniosynostosis. However, patients with variant forms of sagittal craniosynostosis may present with a "normal" CI, depending on the part of the suture that is closed. As machine learning (ML) algorithms are developed to assist in the diagnosis of cranial deformities, metrics that reflect the other phenotypic features of sagittal craniosynostosis are needed. In this study the authors sought to describe the posterior arc angle (PAA), a measurement of biparietal narrowing that is obtained with 2D photographs, and elucidate the role of PAA as an adjuvant to the CI in characterizing scaphocephaly and the potential relevance of PAA in new ML model development. METHODS: The authors retrospectively reviewed 1013 craniofacial patients treated during the period from 2006 to 2021. Orthogonal top-down photographs were used to calculate the CI and PAA. Distribution densities, receiver operating characteristic (ROC) curves, and chi-square analyses were used to describe the relative predictive utility of each method for sagittal craniosynostosis. RESULTS: In total, 1001 patients underwent paired CI and PAA measurements and a clinical head shape diagnosis (sagittal craniosynostosis, n = 122; other cranial deformity, n = 565; normocephalic, n = 314). The area under the ROC curve (AUC) for the CI was 98.5% (95% confidence interval 97.8%-99.2%, p < 0.001), with an optimum specificity of 92.6% and sensitivity of 93.4%. The PAA had an AUC of 97.4% (95% confidence interval 96.0%-98.8%, p < 0.001) with an optimum specificity of 94.9% and sensitivity of 90.2%. In 6 of 122 (4.9%) cases of sagittal craniosynostosis, the PAA was abnormal while the CI was normal. This means that adding a PAA cutoff branch to a partition model increases the detection of sagittal craniosynostosis. CONCLUSIONS: Both CI and PAA are excellent discriminators for sagittal craniosynostosis. Using an accuracy-optimized partition model, the addition of the PAA to the CI increased model sensitivity compared to using the CI alone. Using a model that incorporates both CI and PAA could assist in the early identification and treatment of sagittal craniosynostosis via automated and semiautomated algorithms that utilize tree-based ML models.

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.

Middle Meningeal Artery Embolization for Primary Treatment of a Chronic Subdural Hematoma in a Pediatric Patient: A Systematic Review of the Literature and Case Report.

BACKGROUND: Middle meningeal artery (MMA) embolization is becoming increasingly studied as a safe, effective treatment for chronic subdural hematoma (cSDH) in adults. Among pediatric patients, however, MMA embolization for cSDH has been rarely described, and the potential benefit of this approach for pediatric patients remains unknown. OBJECTIVE: To systematically review the literature and identify cases of pediatric MMA embolization for cSDH. We also report our experience with pediatric MMA embolization. METHODS: A systematic review of the literature was performed to identify cases of pediatric MMA embolization for cSDH. Inclusion criteria included English language availability and pediatric age defined as less than 18 years. A pediatric patient treated with MMA embolization was also identified at our institution. RESULTS: Five cases of pediatric MMA embolization for cSDH were identified in the literature. Two were associated with arachnoid cysts, 2 with antiplatelet/anticoagulation therapy, and 1 with abusive head trauma. There were no adverse events, and all patients demonstrated clinical and radiological improvement on follow-up. At our institution, a previously healthy 8-year-old male was found to have a right-sided acute-on-chronic SDH during a headache evaluation. A diagnostic angiogram was performed to rule out a dural arteriovenous fistula, and right-sided MMA embolization was performed concurrently. Rapid clinical and radiological improvement was observed, with complete resolution by 6 months. CONCLUSION: MMA embolization may represent a treatment option for pediatric patients with cSDH.

Family-acquired photographs for the evaluation of pediatric head shape via telemedicine: an analysis of photograph quality.

OBJECTIVE: Telemedicine can be an effective tool for the evaluation of the pediatric patient with a cranial deformity, but it increases the reliance of neurosurgical providers on data provided by patients and families. Family-acquired photographs, in particular, can be used to augment the evaluation of pediatric head shape abnormalities via telemedicine, but photographs of sufficient quality are necessary. Here, the authors systematically reviewed the quality and utility of family-acquired photographs for patients referred to their pediatric neurosurgery clinic for telemedicine-based head shape evaluations. METHODS: All telemedicine encounters that were completed for head shape abnormalities at the authors' institution between May 2020 and December 2021 were retrospectively reviewed. Instructions were sent to families prior to each visit with examples of ideal photographs. Three orthogonal views of the patient's head-frontal, lateral, and vertex-were requested. Data were collected regarding demographics, diagnosis, follow-up, and photograph quality. Quality variables included orthogonality of each requested view, appropriate distance, appropriate lighting, presence of distracting elements, and whether hair obscured the head shape. RESULTS: Overall, 565 patients had 892 visits during the study period. A total of 1846 photograph requests were made, and 3335 photographs were received for 829 visits. Of 2676 requested orthogonal views, 1875 (70%) were received. Of these, 1826 (97%) had adequate lighting, 1801 (96%) had appropriate distance, and 1826 (97%) had no distracting features. Hair did not obscure the head shape on the vertex view in 557 visits with orthogonal vertex views (82%). In-person follow-up was requested for further medical evaluation in 40 visits (5%). CONCLUSIONS: The family-acquired photographs in this series demonstrated high rates of adequate lighting and distance, without distracting features. Lack of orthogonality and obscuration of the head shape by hair, however, were more common issues. Family education prior to the visit may improve the quality of family-acquired photographs but requires an investment of time by medical staff. Efforts to further improve photographic quality will facilitate efforts to perform craniometric evaluations through telemedicine visits.

Machine Learning-Driven Clinical Image Analysis to Identify Craniosynostosis: A Pilot Study of Telemedicine and Clinic Patients.

BACKGROUND: The authors have developed pretrained machine learning (ML) models to evaluate neonatal head shape deformities using top-down and facial orthogonal photographs of the patient's head. In previous preliminary analysis, this approach was tested with images from an open-source data bank. OBJECTIVE: To determine the accuracy of pretrained ML models in identifying craniosynostosis among patients seen in our outpatient neurosurgery clinic. METHODS: We retrospectively reviewed top-down and facial orthogonal images of each patient's head and provider clinical diagnosis from the same encounters. Head shape classifications generated from 3 pretrained ML models (random forest, classification and regression tree, and linear discriminant analysis) were applied to each patient's photograph data set after craniometric extraction using a predefined image processing algorithm. Diagnoses were codified into a binary scheme of craniosynostosis vs noncraniosynostosis. Sensitivity, specificity, and Matthew correlation coefficient were calculated for software vs provider classifications. RESULTS: A total of 174 patients seen for abnormal head shape between May 2020 and February 2021 were included in the analysis. One hundred seven patients (61%) were seen in-person and 67 (39%) through telemedicine. Twenty-three patients (13%) were diagnosed with craniosynostosis. The best-performing model identified craniosynostosis with an accuracy of 94.8% (95% CI 90.4-97.6), sensitivity of 87.0% (95% CI 66.4-97.2), specificity of 96.0% (95% CI 91.6-98.5), and Matthew correlation coefficient of 0.788 (95% CI 0.725-0.839). CONCLUSION: Machine learning-driven image analysis represents a promising strategy for the identification of craniosynostosis in a real-world practice setting. This approach has potential to reduce the need for imaging and facilitate referral by primary care providers.

Rate of craniometric change following suture release in patients with metopic and sagittal craniosynostosis.

OBJECTIVE: Craniosynostosis is a congenital disorder resulting from the premature fusion of cranial sutures in the infant skull. This condition results in significant cosmetic deformity and can impede neurodevelopment, if left untreated. Currently, rates of craniometric change following minimally invasive surgery have only been examined for sagittal craniosynostosis. A better understanding of postoperative skull adaptations in other craniosynostosis subtypes is needed to objectively categorize surgical outcomes and guide length of cranial orthosis therapy. METHODS: Eleven patients with sagittal and 8 with metopic craniosynostosis treated using endoscopic strip craniectomy and postoperative helmet orthoses were retrospectively reviewed. Using semiautomated image analysis of top-down orthogonal 2D photographs, the following craniometrics were recorded before surgery and at postoperative visits: cephalic index (CI), cranial vault asymmetry index (CVAI), anterior arc angle (AAA), posterior arc angle (PAA), anterior-middle width ratio (AMWR), anterior-posterior width ratio (APWR), left-right height ratio (LRHR), sagittal Hu moment (Sag-Hu), and brachycephaly Hu moment (Brachy-Hu). These craniometrics were then normalized to photograph-based measurements of normocephalic patients and the rates of change between metopic and sagittal craniosynostoses were compared. RESULTS: Patients with sagittal craniosynostosis exhibited significantly lower CI, lower PAA, higher AMWR, higher APWR, lower Sag-Hu, and higher Brachy-Hu preoperatively compared to patients with normocephalic craniosynostosis. Patients with metopic craniosynostosis exhibited lower AAA and AMWR preoperatively compared to normocephalic subjects. Sagittal and metopic patients had a rapid initial change in normalized CI or AAA, respectively. Craniometric rates of change that significantly differed between metopic and sagittal patients were found in AAA (p < 0.001), AMWR (p < 0.001), and APWR (p < 0.0001). Metopic patients had a prolonged AAA change with a significantly different rate of change up to 6 months postoperatively (median at 3 months = 0.027 normalized units/day, median at 6 months = 0.017 normalized units/day, and median at > 6 months = 0.007 normalized units/day), while sagittal CI rate of change at these time points was not significantly different. CONCLUSIONS: Patients with metopic craniosynostosis have a prolonged rate of change compared to patients with sagittal craniosynostosis and may benefit from longer helmet use and extended postoperative follow-up. Categorizing craniometric changes for other craniosynostosis subtypes will be important for evaluating current treatment guidelines.

Image processing and machine learning for telehealth craniosynostosis screening in newborns.

OBJECTIVE: The authors sought to evaluate the accuracy of a novel telehealth-compatible diagnostic software system for identifying craniosynostosis within a newborn (< 1 year old) population. Agreement with gold standard craniometric diagnostics was also assessed. METHODS: Cranial shape classification software accuracy was compared to that of blinded craniofacial specialists using a data set of open-source (n = 40) and retrospectively collected newborn orthogonal top-down cranial images, with or without additional facial views (n = 339), culled between April 1, 2008, and February 29, 2020. Based on image quality, midface visibility, and visibility of the cranial equator, 351 image sets were deemed acceptable. Accuracy, sensitivity, and specificity were calculated for the software versus specialist classification. Software agreement with optical craniometrics was assessed with intraclass correlation coefficients. RESULTS: The cranial shape classification software had an accuracy of 93.3% (95% CI 86.8-98.8; p < 0.001), with a sensitivity of 92.0% and specificity of 94.3%. Intraclass correlation coefficients for measurements of the cephalic index and cranial vault asymmetry index compared to optical measurements were 0.95 (95% CI 0.84-0.98; p < 0.001) and 0.67 (95% CI 0.24-0.88; p = 0.003), respectively. CONCLUSIONS: These results support the use of image processing-based neonatal cranial deformity classification software for remote screening of nonsyndromic craniosynostosis in a newborn population and as a substitute for optical scanner- or CT-based craniometrics. This work has implications that suggest the potential for the development of software for a mobile platform that would allow for screening by telemedicine or in a primary care setting.

Comparison of Children's Venipuncture Fear and Pain: Randomized Controlled Trial of EMLA® and J-Tip Needleless Injection System®.

PURPOSE: Needle procedures, like venipuncture and intravenous (IV) catheter insertion, are recognized as a common cause of pain and fear for children in hospitals and emergency departments. The purpose of this study was to compare children's self-reported pain and fear related to IV insertion with administration of either the topical local anesthetic EMLA® or 1% buffered lidocaine delivered with the J-Tip Needleless Injection System® (J-Tip®). DESIGN AND METHODS: In this prospective, randomized trial, 150 consecutive pediatric patients 8 to 18years of age undergoing IV insertion were randomly assigned 1:1 to treatment group. Participants self-reported procedural pain using a Visual Analog Scale, and procedural fear using the Children's Fear Scale. RESULTS: Procedural pain scores were significantly lower in the EMLA® group (mean score 1.63+1.659) vs. the J-Tip® group (2.99±2.586; p<0.001). Post-procedure fear scores were significantly lower than pre-procedure fear scores in both treatment groups (p<0.002), but there was no difference in fear scores between the two treatment groups (p=0.314). CONCLUSION: EMLA® provided superior pain relief for IV insertion compared to J-Tip®. PRACTICE IMPLICATIONS: Although EMLA® use resulted in lower self-reported pain scores compared to J-Tip®, pain scores for both treatments were low and fear scores did not differ. When IV insertion can be delayed for 60-90min, EMLA® should be used. When a delay is contraindicated, J-Tip® may be a reasonable alternative to minimize procedural pain of IV insertion.

Incidence and acute complications of asymptomatic central venous catheter-related deep venous thrombosis in critically ill children.

OBJECTIVE: To determined the current incidence and acute complications of asymptomatic central venous catheter (CVC)-related deep venous thrombosis (DVT) in critically ill children. STUDY DESIGN: We performed a prospective cohort study in 3 pediatric intensive care units. A total of 101 children with newly inserted untunneled CVC were included. CVC-related DVT was diagnosed using compression ultrasonography with color Doppler. RESULTS: Asymptomatic CVC-related DVT was diagnosed in 16 (15.8%) children, which equated to 24.7 cases per 1000 CVC-days. Age was independently associated with DVT. Compared with children aged <1 year, children aged >13 years had significantly higher odds of DVT (aOR, 14.1, 95% CI, 1.9-105.8; P = .01). Other patient demographics, interventions (including anticoagulant use), and CVC characteristics did not differ between children with and without DVT. Mortality-adjusted duration of mechanical ventilation, a surrogate for pulmonary embolism, was statistically similar in the 2 groups (22 ± 9 days in children with DVT vs 23 ± 7 days in children without DVT; P = .34). Mortality-adjusted intensive care unit and hospital lengths of stay also were similar in the 2 groups. CONCLUSION: Asymptomatic CVC-related DVT is common in critically ill children. However, the acute complications do not seem to differ between children with and without DVT. Larger studies are needed to confirm these results. Future studies should also investigate the chronic complications of asymptomatic CVC-related DVT.

Emergent endotracheal intubations in children: be careful if it's late when you intubate.

OBJECTIVE: Emergent endotracheal intubations carry a high risk of morbidity and mortality in critically ill adults. Although children may be at higher risk during this procedure as a result of age-related differences in anatomy and physiology, this has not been previously examined. The purpose of this study was to delineate the risks of emergent endotracheal intubations in children. DESIGN: Retrospective cohort study. SETTING: A 122-bed free-standing children's hospital. PATIENTS: We conducted a retrospective review of all intubations occurring outside of the operating room setting between October 2005 and October 2007. Elective intubations were excluded. Intubations were classified as emergent after review of the child's vital signs, blood gas values, and written documentation by nurses, respiratory therapists, and physicians. Intubations occurring on weekends or between 5 pm and 8 am on weekdays were categorized as "off-hours." INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: During the study period, 137 intubations were performed, 77 of which (56%) were emergent. Emergent endotracheal intubations were significantly more likely to occur off-hours (odds ratio, 2.0; 95% confidence interval, 1.1-4.1) and to be associated with a complication (odds ratio, 3.0; 95% confidence interval, 1.4-6.1). Complications occurred in 41% of all intubations. The most common complications were desaturations (29% of all intubations), hypotension (16%), and bradycardia (7%). In a multivariate logistic regression analysis, emergent intubation, off-hours intubation, three or more attempts at intubation, smaller endotracheal tube size, and admission for cardiovascular disease all increased the likelihood of experiencing a complication during intubation. Complications were not associated with an indication for intubation or baseline chronic disease of the child and were not associated with prolonged intensive care unit course or duration of mechanical ventilation. CONCLUSIONS: Emergent endotracheal intubations are commonly performed in children, are two times more likely to occur off-hours, and are associated with three times the risk of complications as nonemergent intubations.

Beta2-adrenergic receptor polymorphisms affect response to treatment in children with severe asthma exacerbations.

BACKGROUND: beta(2)-adrenergic receptor (AR) agonists are the mainstay of treatment for severe asthma exacerbations, one of the most common causes of critical illness in children. Genotypic differences in the beta(2)-AR gene, particularly at amino acid positions 16 and 27, have been shown to affect the response to beta(2)-AR agonist therapy. Our hypothesis is that genotypic differences contribute to patient response to beta(2)-AR agonist treatment during severe asthma exacerbations in children. METHODS: Children admitted to the hospital ICU for a severe asthma exacerbation between 2002 and 2005 were located, and genetic samples were obtained from saliva. Children hospitalized during this period were treated with a protocol that titrated beta(2)-AR therapy (first nebulized, then IV) according to a validated clinical asthma score. RESULTS: Thirty-seven children hospitalized during the study period were enrolled into the study. At amino acid position 16 in the beta(2)-AR gene, 13 children were homozygous for the glycine (Gly) allele (Gly/Gly), 8 were homozygous for the arginine (Arg) allele (Arg/Arg), and 16 were heterozygous (Arg/Gly). Despite similar clinical asthma scores on hospital admission, the children with the Gly/Gly genotype had significantly shorter hospital ICU length of stay and duration of continuously nebulized albuterol therapy and were significantly less likely to require IV beta(2)-AR therapy than those with Arg/Arg or Arg/Gly genotypes. No association existed among polymorphisms at amino acid position 27 and response to beta(2)-AR therapy. CONCLUSIONS: In this cohort of children with severe asthma exacerbations, children whose genotypes were homozygous for Gly at amino acid position 16 of the beta(2)-AR gene had a more rapid response to beta(2)-AR agonist treatment. The beta(2)-AR genotype appears to influence the response to therapy in this population.

Childhood overweight increases hospital admission rates for asthma.

OBJECTIVES: Although childhood overweight has been associated with increased hospital lengths of stay for patients with asthma, the possible relationship between overweight and hospital admission for asthma has not been well studied. We hypothesized that overweight children who presented to the emergency department with asthma exacerbations were more likely to be admitted to the hospital than nonoverweight children. METHODS: A retrospective chart review was conducted of all children who were older than 2 years and presented to the emergency department with an asthma exacerbation in calendar year 2005. Children with chronic medical conditions other than asthma were excluded. Children were classified as nonoverweight (< or = 95% weight-for-age percentile) or overweight (> 95% weight for age). RESULTS: During the study period, there were 884 visits to the emergency department for an asthma exacerbation by 813 children; 238 (27%) were admitted to the hospital, and 33 (4%) were admitted to the ICU. Overall, hospital admission was associated with higher clinical asthma score but not with age, gender, or poverty status (as quantified as home in zip-code areas designated as "impoverished"). Overweight children (n = 202 [23%]) were significantly older (8.5 +/- 4.4 vs 7.3 +/- 4.3 years) and more likely to live in an impoverished area (37% vs 28%). Presenting clinical asthma score and therapeutic interventions in the emergency department were similar for overweight and nonoverweight children; however, overweight children were significantly more likely to be admitted to the hospital. CONCLUSIONS: Overweight children who present to the emergency department with acute asthma exacerbations are significantly more likely to be admitted to the hospital than nonoverweight children. This identifies an important area in which childhood overweight has a significant impact on the health of children with asthma.