Tertiary Pediatric Medical Center Research Articles

Factors Associated with Failed Closed Reduction in Flexion and Gartland Type III Pediatric Supracondylar Humerus Fractures

BackgroundMost displaced supracondylar humerus fractures (SCHF) are treated with closed reduction and percutaneous pinning (CRPP). While there are only a few possible indications for converting to an open reduction, a failed closed reduction is a common cause. This study aims to elucidate possible risk factors for failed closed reductions of SCHF. MethodsA retrospective review of SCHF from 2010 to 2020 at a pediatric tertiary medical center, who underwent operative fixation, was conducted. Exclusion criteria were open fractures and reasons for open reduction other than failed closed reduction. Rates of open reduction were assessed by pre-operative fracture classification and assessed for respective associations with the factors of interest using Student’s T-test, Chi-square, or fisher exact tests as indicated. Results716 patients (age range 1-15 years old) met inclusion criteria. Failed closed reductions were more likely in flexion-type fractures (15/37) compared to type III extension fractures (31/480) (OR: 9.88, 95% CI: 4.66-20.92). For flexion-type fractures, failed closed reduction occurred at a lower rate for anteriorly displaced fractures (5/22) when compared to other displacement directions (10/15) (OR: 0.15, 95%CI: 0.034-0.637). Age, race, social deprivation index, BMI, associated injuries, comminution, and nerve palsy were not significant. For type III extension fractures, older age (>8 years) (OR: 5.22, 95% CI: 1.56-17.43) and nerve injury (OR: 2.23, 95% CI: 1.00-5.10) were associated with failed closed reduction. No other factors of interest were significant. ConclusionFlexion type SCHF have significantly higher rates of failed closed reduction compared to extension type fractures. For flexion type fractures, anterior displacement predicts a lower rate of failed closed reduction compared to other displacement directions. For type III extension fractures, risk factors include older age and a nerve injury on pre-operative exam. Key Concepts(1).Most operative supracondylar humerus fractures can be treated with closed reduction and percutaneous pinning.(2).Surgeons need to be aware of possible reasons for having to convert to open reduction of pediatric supracondylar humerus fractures.(3).Flexion type fracture patterns had a higher rate of an open procedure compared to extension type fractures.(4).Patients who sustained an extension type injury were more likely to require an open reduction if they had a nerve injury or were older at the time of injury or pinning (>8 years old). Level of EvidenceLevel III, Retrospective Cohort Study

Barriers to Meeting National Early Hearing Detection and Intervention Guidelines in a Diverse Patient Cohort.

To determine our audiology clinics status in meeting the Joint Committee on Infant Hearing recommended 1-3-6 benchmarks for identification and intervention for congenital sensorineural hearing loss and identify those factors contributing to delay in identification and intervention. This is a retrospective case series. Children with sensorineural hearing loss who underwent auditory brainstem response (ABR) testing, hearing aid evaluation, or cochlear implant mapping at our tertiary pediatric medical center between January 2018 and December 2021 were included. Simple and multiple linear regression analyses were used to identify social, demographic, and health factors associated with primary outcomes, defined as age at hearing loss identification, age at intervention (here defined as amplification start), and interval between identification and intervention. Of 132 patients included, mean age was 2.4 years, 48% were male, and 51% were Hispanic. There was significant association between each Hispanic ethnicity (p = 0.005, p = 0.04, respectively), insurance type (p = 0.02, p = 0.001, respectively), and later age at identification and intervention. In multivariable analyses, Hispanic ethnicity was significantly associated with both delays in identification and intervention (p = 0.03 and p = 0.03, respectively), and public insurance was associated with delays in intervention (p = 0.01). In addition, the total number of ABRs was significantly associated with both older age of identification and intervention (p < 0.001, p < 0.001, respectively). Mediator analysis demonstrated that the effect of ethnicity on age at identification is mediated by the total number of ABRs performed. A significant association between total number of ABRs and age at identification and intervention for children with hearing loss exists. Hispanic ethnicity was associated with delays in meeting milestones, further mediated by the number of ABRs, providing a potential avenue for intervention in addressing this disparity.

Repeat tracheal aspirate cultures in pediatric intensive care patients: Frequency, resistance, and antimicrobial use.

To evaluate the clinical impact and features associated with repeat tracheal aspirate (TA) cultures in children admitted to the intensive care unit. Retrospective cohort study. A 338-bed freestanding, tertiary pediatric academic medical center with pediatric medical intensive care unit (PICU) and cardiac intensive care units (CICU). Children ≤18 years of age who were admitted to either the PICU or CICU who had ≥2 TA cultures in a single intensive care admission. Patients with ≥2 TA cultures between 2018 and 2019 were included in this study. The following information was collected: patient demographics, clinical data summarizing patient condition at the time of culture collection, number of TA cultures per patient, antibiotic usage, and microbiologic data. Descriptive statistics established the frequency of TA collection, time between culturing, clinical reasoning for collection, antibiotic exposure, and development of multidrug-resistant organisms (MDRO). Sixty-three patients had repeat TA cultures and accounted for 252 TA cultures during the study period. Most patients with repeat TA cultures were admitted to the PICU (71%) and were male (65%). A median of 3 TA cultures per patient were obtained with 50% of repeat cultures occurring within 7 days from the previous culture. Sixty-six percent of patients had the same organism cultured on ≥2 TA cultures. Most antibiotics were not modified or continued to treat the results of the TA culture. Repeat TA cultures frequently show the same pathogens, and results do not often influence antibiotic selection or usage. Repeat TA cultures did demonstrate the development of MDROs.

Predictors of syrinx presentation and outcomes in pediatric Chiari malformation type I: a single institution experience of 218 consecutive syrinx patients.

Chiari I malformation (CM-I) in pediatric patients can impose substantial neurologic and functional impairment. Additionally, the presence of syrinx is often a harbinger of clinical compromise, but little attention has been devoted to identifying features associated with syrinx development and the clinical impact of syrinx resolution. Therefore, this study aims to identify clinical and radiographic variables associated with preoperative syrinx presence and postoperative syrinx reduction in pediatric patients with CM-I and determine the relationship between postoperative syrinx reduction and clinical symptom improvement. The authors performed a retrospective analysis of 435 consecutive pediatric patients who underwent surgical treatment of CM-I from 2001 to 2021 at a single tertiary pediatric medical center. All patients underwent pre- and postoperative MRI, and clinical and radiographic variables were recorded and subject to inferential analysis. Syrinx at presentation was independently associated with symptoms of spinal cord dysfunction at presentation (OR 2.17 (95% CI 1.05-4.48); p = 0.036), scoliosis (OR 5.33 (2.34-10.86); p = 0.001), and greater pB-C2 (posterior basion to C2 distance) measurement length (OR 1.14 (95% CI 1.01-1.30); p = 0.040). Syrinx at presentation was inversely associated with tussive headaches at presentation (OR 0.27 (95% CI 0.16-0.47); p = 0.001) and cranial nerve deficits at presentation (OR 0.49 (95% CI 0.26-0.92); p = 0.025). Postoperatively, patients with radiographic evidence of syrinx improvement had greater rates of symptom improvement (93.1% vs 82.1%; p = 0.049), better CCOS scores (15.4 vs 14.2; p = 0.001), and decreased rates of readmission (6.0% vs 25.0%, p = 0.002) and reoperation (0.5% vs 35.7%; p = 0.001). The difference in syrinx resolution was similar but not statistically significant (10.3% vs 16.7%; p = 0.251). AO joint anomaly (OR 0.20, 95% CI 0.04-0.95; p = 0.026) and foramen magnum diameter (OR 1.12, 95% CI 1.00-1.25; p = 0.049) were the only independent predictors of syrinx improvement, and surgical technique was the only predictor for syrinx resolution (OR 2.44, 95% CI 1.08-5.50; p = 0.031). Patients that underwent tonsil reduction surgery whose syrinx improved had a wider foramen magnum diameter than those whose did not improve (34.3 vs 31.7; p = 0.028). Radiographic syrinx improvement is associated with greater rates of symptom improvement and less readmissions and reoperations for CM-I. AO joint anomalies and narrower foramen magnums were independent risk factors for the lack of syrinx improvement. These novel insights will help guide preoperative patient counseling, pre- and intraoperative surgical decision-making, and postoperative clinical prognostication in the treatment of pediatric CM-I.

Identification of neurological complications in childhood influenza: a random forest model

BackgroundAmong the neurological complications of influenza in children, the most severe is acute necrotizing encephalopathy (ANE), with a high mortality rate and neurological sequelae. ANE is characterized by rapid progression to death within 1–2 days from onset. However, the knowledge about the early diagnosis of ANE is limited, which is often misdiagnosed as simple seizures/convulsions or mild acute influenza-associated encephalopathy (IAE).ObjectiveTo develop and validate an early prediction model to discriminate the ANE from two common neurological complications, seizures/convulsions and mild IAE in children with influenza.MethodsThis retrospective case-control study included patients with ANE (median age 3.8 (2.3,5.4) years), seizures/convulsions alone (median age 2.6 (1.7,4.3) years), or mild IAE (median age 2.8 (1.5,6.1) years) at a tertiary pediatric medical center in China between November 2012 to January 2020. The random forest algorithm was used to screen the characteristics and construct a prediction model.ResultsOf the 433 patients, 278 (64.2%) had seizures/convulsions alone, 106 (24.5%) had mild IAE, and 49 (11.3%) had ANE. The discrimination performance of the model was satisfactory, with an accuracy above 0.80 from both model development (84.2%) and internal validation (88.2%). Seizures/convulsions were less likely to be wrongly classified (3.7%, 2/54), but mild IAE (22.7%, 5/22) was prone to be misdiagnosed as seizures/convulsions, and a small proportion (4.5%, 1/22) of them was prone to be misdiagnosed as ANE. Of the children with ANE, 22.2% (2/9) were misdiagnosed as mild IAE, and none were misdiagnosed as seizures/convulsions.ConclusionThis model can distinguish the ANE from seizures/convulsions with high accuracy and from mild IAE close to 80% accuracy, providing valuable information for the early management of children with influenza.

Validity evidence for a team-leading assessment tool in pediatric emergency resuscitations using video review.

Effective leadership of health care action teams has demonstrated positive influence on team performance and patient care, but there is no consensus on how to assess these skills. We developed a novel team leadership assessment tool for leaders of interprofessional pediatric resuscitation teams and collected validity evidence for this tool using video review. This was a prospective cohort study from November 2021 to October 2022. A novel team leadership assessment tool was developed using literature review and local expertise and then piloted and refined using medical simulation. Pediatric emergency medicine (PEM) fellows from a single tertiary care pediatric medical center were enrolled, and videos of one medical resuscitation and one trauma resuscitation were collected per fellow each month. Three reviewers underwent reviewer training and then scored the videos using the assessment tool. Raters provided feedback on feasibility and ease of use using a 5-point Likert scale. Inter-rater reliability for the assessment tool using Gwet's agreement coefficient and the association between performance and clinical level of training using generalized linear mixed model were calculated. Twelve PEM fellows enrolled and 146 videos were reviewed. The inter-rater reliability for each domain ranged from 0.45 (p < 0.0001) to 0.59 (p < 0.0001), with the inter-rater reliability of the total score being 0.49 (p < 0.0001). The reviewers' mean ratings of the elements of the tool were as follows: clarity of the domains (4.6/5), the independence of each domain from each other (3.9/5), the ease of use of the 5-point Likert scale (4.5/5), the usefulness of the provided examples for each domain (4.6/5), and the ability to assess each domain without having to rewatch (4.5/5). The tool differentiated between levels of clinical training for two of the six domains (p < 0.02). We developed a novel team leadership assessment tool for pediatric resuscitation team leaders that demonstrated moderate inter-rater reliability. The tool was easy to use and feasible for educators to assess the performance of PEM trainees in complex high-stakes clinical situations.

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

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

Implementation of a tier system for IVIG indications to address IVIG shortage at a tertiary care pediatric medical center.

Drug shortages are a common issue that healthcare systems face and can result in adverse health outcomes for patients requiring inferior alternate treatment. The United States recently experienced a national drug shortage of intravenous immunoglobulin (IVIG). Several reported strategies to address the IVIG and other drug shortages have been proposed; however, there is a lack of evidence-based methods for protocol development and implementation. To evaluate the efficacy of introducing a multidisciplinary task force and tier system of indications and to minimize adverse effects during a shortage of IVIG. Faculty members across disciplines with expertise in IVIG use were invited to participate in a task force to address the shortage and ensure adequate supply for emergent indications. A tier system of IVIG indications was established according to the severity of diagnosis, urgency of indication, and quality of supporting evidence. Based on inventory, indications in selected tiers were auto-approved. Orders that could not be automatically approved were escalated for task force review. Overall, there were 342 distinct requests for IVIG during the study period (August 1, 2019 to December 31, 2019). All Tier 1 indications were approved. Of all requests, only 2.6% (9) of requests were denied, none of which resulted in adverse effects based on retrospective chart review. Seven patients who regularly receive IVIG had possible adverse effects due to dose reduction or spacing of treatment; however, each complication was multifactorial and not attributed to the shortage or tier system implementation alone. Implementation of a multidisciplinary task force and tier system to appropriately triage high-priority indications for limited pharmaceutical agents should be considered in health institutions faced with a drug shortage.

Low Risk of Inappropriate Shock Among Pediatric Patients With an Implantable Cardioverter Defibrillator: A Single Center Experience.

Implantable cardioverter-defibrillators (ICD) are increasingly being used among the pediatric population for indications of both primary and secondary prevention. There is limited long-term data on the outcomes of pediatric patients following ICD implantation. The aim of this study was to investigate the characteristics of this population, burden of appropriate and inappropriate shock and complication rate in a large tertiary pediatric medical center. Included were children under the age of 18years who underwent ICD implantation and had clinical follow up at our center. Data were retrospectively collected between study period 2005-2020. Primary outcome was the incidence of ICD shock appropriate and inappropriate. Secondary outcome was defining our patient population characteristics. Our cohort included 51 patients who underwent ICD implantation. Mean age at implantation was 10.9 ± 4.7years and average follow-up time was 67months. Diagnoses of implanted patients were: 28 (55%) patients with syndromes with risk for sudden death, cardiomyopathy in 14 patients (27%) and congenital heart disease (CHD) in 9 patients (18%). Forty-two (82%) patients had an ICD implanted for secondary prevention after experiencing a life-threatening arrhythmia and 9 (18%) for primary prevention. An endocardial system was implanted in 39 (76%) patients and an epicardial systems in 12 (24%) patients. A total of 20 (39%) patients received appropriate shocks for ventricular fibrillation(VF). 5 patients received inappropriate shocks, 4 due to sinus tachycardia and 1 due to rapidly conducted atrial fibrillation. Those who received an inappropriate shock had a significantly shorter ICD-programmed VF detection cycle length compared to those who did not receive an inappropriate shock (320ms versus 270ms, p = 0.062). This single center study demonstrates a high rate of appropriate ICD shocks (39%) and a low rate of inappropriate ICD shocks. Accurate programming of ICD devices in the pediatric population is paramount to avoid inappropriate ICD shocks.

Development of a Novel Massage Therapy Outcome Measure for Children and Young Adults Receiving Hematopoietic Cell Transplant.

Children receiving hematopoietic stem cell transplantation (HCT) often experience an unfortunate sequalae of negative effects including pain, deconditioning, and anxiety. Massage therapy (MT) has demonstrated effective non-pharmacological management of fatigue, pain, and anxiety in patients undergoing cancer treatment. Existing studies have been limited by the lack of available MT-specific outcome measures to track responses to interventions. This study aimed to describe the creation of a novel MT-specific outcome measure to be utilized in the pediatric acute-care setting and establish construct validity for this measure to assess clinical effectiveness of MT interventions. An oncology ward at a large pediatric tertiary medical center in the United States. A total of 58 children and young adults undergoing HCT. Retrospective Cohort Study. A panel of massage therapists created a novel outcome measure, OMPREP, for use in MT sessions and performed a literature review to ensure face validity of the tool. This outcome measure was administered to patients and data were collected retrospectively to assess construct validity. A total of 1,333 MT sessions were completed (80.7% completion rate) with the novel OMPREP outcome measure utilized on 100% of visits. Mean engagement (p<.001), response (p<.001), and pain (p<.001) scores were all significantly greater at evaluation and discharge compared to the lowest observed scores post-HCT. The novel MT-specific outcome measure, OMPREP, was feasible and demonstrated construct validity when implemented in a pediatric acute-care setting by massage therapists. This new tool may offer a quantitative measure of MT-interventions and assist in tracking patient outcomes.

Severe early-onset Wilson disease caused by a common pathogenic variant in the Bukharan Jewish population in Israel

BackgroundWilson disease is caused by pathogenic variants in the ATP7B gene which encodes a copper-transporting ATPase. AimsDescribe a common founder pathogenic variant among Bukharan Jews and to assess its prevalence, clinical features, and outcome. MethodsThe cohort consisted of patients of Bukharan Jewish descent diagnosed with Wilson disease at a tertiary pediatric medical center in 2013–2018. Clinical and genetic data were collected and analyzed. ResultsSix patients from 4 unrelated families who were homozygous for the c.3784G > T p.(Val1262Phe) pathogenic variant in ATP7B were identified. Five presented with elevated aminotransferase levels, and one, with acute liver failure. Mean age at diagnosis was 8.7 years (5–12.5). Serum ceruloplasmin level was extremely low in all patients (1.9–7 mg/dL; mean 3.2(. The variant was identified in a heterozygous state in 5/153 Bukharan Jews; 2/33 from our local exome database and 3/120 healthy unrelated Bukharan Jews in another cohort, for an estimated carrier frequency of ∼1:30. ConclusionsWe report a common founder pathogenic variant in the ATP7B gene among Bukharan Jews associated with severe early-onset Wilson disease. Given the clinical severity, high frequency of the variant, and being a treatable disease, its inclusion in pre-symptomatic screening in the Bukharan Jewish community should be considered. Furthermore, WD should be part of future genetic newborn screening programs in Israel and worldwide, to enable early treatment and prevention of future life-threatening complications.

Risk factors for developing calcinosis in juvenile dermatomyositis: subcutaneous and myofascial edema in initial magnetic resonance imaging.

Calcinosis is a sequela of Juvenile Dermatomyositis (JDM) with significant morbidity. A retrospective study observing risk factors for JDM calcinosis, including a possible association between higher intensity of subcutaneous and myofascial edema in initial magnetic resonance imaging (MRI) and development of calcinosis was performed at a tertiary pediatric medical center. Data from the past 20years on JDM patients with MRIs at the time of JDM diagnosis were obtained. MRIs were individually evaluated by two pediatric musculoskeletal radiologists who blindly graded the intensity of edema on a 0-4 Likert scale. Clinical data and edema scores were compared between patients who developed calcinosis and who did not. Forty-three patients (14 with calcinosis and 29 without calcinosis) were identified. The calcinosis group contained more racial and ethnic minorities, younger ages of JDM onset and longer time to reach JDM diagnosis. Muscle enzyme levels at JDM diagnosis were lower in the calcinosis group, especially Creatinine Kinase (CK) (p = 0.047) and Alanine Aminotransferase (ALT) (p = 0.015). The median score for edema in both groups was 3 (p = 0.39) with an inter-rater reliability of 95%. There was no association between increased subcutaneous and myofascial edema in MRIs at the time of JDM diagnosis and development of calcinosis. Earlier age of JDM onset, racial and ethnic minority, and delay in JDM diagnosis could be risks for developing calcinosis. The calcinosis group presented with lower muscle enzyme levels at the time of JDM diagnosis, especially CK and ALT with statistical significance. This could reflect delay in diagnosis and treatment.

Association between wildfire smoke exposure and Seattle, Washington Pediatric Hospital services, 2006–2020

Pacific Northwest wildfire smoke events have been increasing in prevalence and severity over the past three decades, resulting in documented negative health outcomes in adults. However, there is less evidence demonstrating the effect of wildfire smoke in pediatric populations. To evaluate the association between wildfire smoke exposure and healthcare utilization in a pediatric tertiary medical center in Seattle, WA. We utilized a case–crossover study to determine the odds of pediatric emergency department (ED) visit/ hospital admission at Seattle Children’s Hospital on wildfire smoke days versus non-wildfire smoke days during wildfire season (June to September), 2006–2020. The health outcomes dataset reports hospital encounters in two categories: ED visits or admissions that are for inpatient or observational purposes. The health outcomes dataset reports hospital encounters in two categories: ED visits or admissions that are for inpatient or observational purposes. The reported encounter types are mutually exclusive. We stratified analyses by individual-level characteristics and examined associations for lagged exposures 0–7 d prior to admission. In adjusted analyses, smoke exposure was associated with a 7.0% (95% CI: 3.0%–12.0%) increase in odds of all-cause hospital admissions and a 0.0% (95% CI: −3.0%, 3.0%) change in odds of all-cause ED visits. We also observed increases in the odds of all-cause hospital admissions ranging from 4.0% to 8.0%, for lagged exposure on days 1–7. When stratified by health outcomes, we found a 9.0% (95% CI: 1.0%–17.0%) and an 11.0% (95% CI:1.0%–21.0%) increase in the odds of ED visits for respiratory and respiratory infection-related concerns, respectively. Our results demonstrate associations between wildfire smoke and negative health effects in children. Similar to other studies, we found that wildfire smoke exposure was associated with an increase in respiratory-related ED visits and all-cause hospital admissions in a pediatric population. These results will help inform patient education and motivate interventions to reduce pediatric morbidity during wildfire season.

Co-occurring conditions in children with Down syndrome and autism: a retrospective study

BackgroundDown syndrome (DS) is one of the most common genetic causes of intellectual disability, and it is associated with an increased incidence of numerous co-occurring conditions. Autism spectrum disorder (ASD) is common in persons with DS, with rates reported as high as 39%. However, little is known regarding co-occurring conditions in children with both DS and ASD.MethodsA single-center retrospective review of prospective longitudinally collected clinical data was performed. Any patient with a confirmed diagnosis of DS evaluated at a large, specialized Down Syndrome Program in a tertiary pediatric medical center between March 2018 and March 2022 was included. A standardized survey which included demographic and clinical questions was administered during each clinical evaluation.ResultsIn total, 562 individuals with DS were included. The median age was 10 years (IQR: 6.18–13.92). Of this group, 72 (13%) had a co-occurring diagnosis of ASD (DS+ASD). Individuals with DS+ASD were more likely to be male (OR 2.23, CI 1.29–3.84) and had higher odds of a current or prior diagnosis of constipation (OR 2.19, CI 1.31–3.65), gastroesophageal reflux (OR 1.91, CI 1.14–3.21), behavioral feeding difficulties (OR 2.71, CI 1.02–7.19), infantile spasms (OR 6.03, CI 1.79–20.34) and scoliosis (OR 2.73, CI 1.16–6.40). There were lower odds of congenital heart disease in the DS+ASD group (OR 0.56, CI 0.34–0.93). There was no observed difference in prematurity or Neonatal Intensive Care Unit complications between groups. Individuals with DS+ASD had similar odds of having a history of congenital heart defect requiring surgery to those with DS only. Furthermore, there was no difference in rates of autoimmune thyroiditis or celiac disease. There was also no difference in rates of diagnosed co-occurring neurodevelopmental or mental health conditions in this cohort, including anxiety disorders and attention-deficit/hyperactivity disorder.ConclusionsThis study identifies a variety of medical conditions which are more frequent in children with DS+ASD than DS alone, providing important information for the clinical management of these patients. Future research should investigate the role of some of these medical conditions in the development of ASD phenotypes, and whether there may be distinct genetic and metabolic contributions towards these conditions.

Unplanned Intensive Care Unit Admission Following Cleft Palate Repair by Head and Neck Surgeons.

To examine the incidence of pediatric intensive care unit (PICU) admission following primary repair of cleft palate by otolaryngologist-head and neck surgeons at 2 tertiary centers. To identify potential diagnoses associated with admission or unanticipated PICU transfer. Multi-institutional case series with chart review. Two tertiary pediatric medical centers. Children who underwent primary repair of cleft palate at 2 cleft centers over a 10-year period were identified. Charts were reviewed for demographics, comorbidities, and whether PICU admission was required. From 2009 to 2019, 464 patients underwent primary repair of a cleft palate by 1 of 6 otolaryngologist-head and neck surgeons with subspecialty training in cleft surgery; 459 patients had sufficient postoperative documentation and 443 children met inclusion criteria. The incidence of PICU admission was 9.3% (41/443), with 33 (7.4%) planned admissions and 8 (1.8%) unexpected PICU transfers. Syndromic conditions were associated with both planned and unanticipated PICU admissions. The incidence of unanticipated postoperative PICU admission following cleft palate repair by otolaryngologist-head and neck surgeons was low. Risk stratification by surgeons with expertise in airway management may inform decisions regarding postoperative disposition of patients with medical or airway complexity who are undergoing cleft palate repair.

The contribution of the Neurobehavioral Screening Tool to identifying fetal alcohol spectrum disorders in children at high risk of prenatal alcohol exposure and neurobehavioral deficits

BackgroundFetal alcohol spectrum disorders (FASD) describe various conditions resulting from prenatal alcohol exposure. The diagnosis of FASD can be challenging and complex. The Neurobehavioral Screening Tool (NST), derived from Achenbach's Child Behavior Checklist, has been suggested as a tool for identifying FASD. AimsTo assess the external validity of the NST and to identify additional characteristics of FASD in a cohort of Israeli children and young adults referred to a neurology and child developmental clinic at a tertiary pediatric medical center in Israel. Study designAn observational study based on medical records. Subjects151 children and young adults, of whom 40 were diagnosed with FASD according to updated clinical guidelines. Outcome measuresNST results, as well as demographic and neurobehavioral variables, were compared between those who were and were not diagnosed with FASD. ResultsThe NST demonstrated 72 % to 73 % sensitivity, and 34 % to 36 % specificity, in identifying FASD. Items 4 and 5 (‘Lies or cheats’, ‘Lacks guilt after misbehaving’) were the most predictive items in the NST. Other variables that were characteristic of the FASD group included: emotional regulation difficulties (p value <0.01), being born and adopted in Israel (vs. other countries) (p value <0.01) and younger age at the first visit to the clinic (p value <0.01). ConclusionsOur findings regarding the screening capabilities of the NST were less promising than those of most previous studies. Further research is needed to establish a valid neurobehavioral tool with the possible focus on antisocial behaviors and emotional regulation problems.

Pediatric temporomandibular joint (TMJ) arthritis, an elusive complication of acute mastoiditis

IntroductionSeptic arthritis of the Temporomandibular joint (TMJ) is a rare complication of acute middle ear infection. Presentation is elusive and could be easily missed. Often diagnosis is made only with consequential development of TMJ ankylosis. This study intends to characterize patients and course of disease and suggest a diagnostic and therapeutic strategy. MethodsRetrospective review of all children diagnosed with TMJ arthritis and/or TMJ ankylosis secondary to acute middle ear infection, treated in a tertiary pediatric medical center between the years 2005 and 2021. ResultSeven patients were identified with otogenic TMJ arthritis. Median age at presentation was 1.14 years (IQ range 1.1–1.5). All seven were diagnosed with acute mastoiditis. CT scans demonstrated TMJ related collections in 5/7 and intracranial complications in 3/7. Treatment included cortical mastoidectomy for 5/7. One patient had a concomitant surgical washout of the TMJ. Two patients had drainage only of subperiosteal collections.Six of the seven patients went on to develop TMJ ankylosis that presented within a median of 2.8 years (IQ range 2.6–3.9) after the episode of acute mastoiditis. All six patients presented with trismus and facial growth anomalies, and all but one required surgery to release the ankylosis. ConclusionOtogenic TMJ arthritis develops mostly in young children with acute mastoiditis and intratemproal and/or intracranial suppuration. Imaging is helpful in making the diagnosis, with TMJ related collections on CT being the most common finding. TMJ ankylosis can develop within a few years and present with trismus and abnormal facial growth. Cortical mastoidectomy does not seem to prevent ankylosis. It remains unclear whether focused treatment to the TMJ or physiotherapy could be beneficial in that.

Dominant Ventricular Morphology and Early Postoperative Course After the Fontan Procedure.

BackgroundSingle ventricle heart disease comprises a wide variety of critical heart defects that lead to the provision of systemic cardiac output by one dominant ventricle. It requires staged surgical palliation that culminates in Fontan circulation. Dominant ventricular morphology in single ventricle patients reportedly has an impact on postoperative morbidity and mortality with varying results. The objectives of this study were to examine the association between ventricular morphology and the early postoperative course after the Fontan procedure.MethodsA retrospective cohort study in a tertiary referral pediatric medical center that included 98 consecutive patients who underwent Fontan procedure between October 2009 and May 2016. Postoperative outcomes were compared between patients with left ventricular morphology and those with right ventricular morphology (crude effect and regression analysis).ResultsPatients with right ventricular morphology had longer postoperative hospitalizations compared to patients with left ventricular morphology (26.5 days vs 18.2 days, respectively, P = .028), higher postoperative maximal vasoactive-inotropic scores (25.6 vs 12.4, P = .02), higher serum lactate levels (7.7 mmol/L vs 6.4 mmol/L, P = .03), higher proportions of ventilation throughout 24 h or more (16 patients [38%] vs 8 patients [14%], P = .009), higher proportions of ventricular dysfunction (12 patients [29%] vs 5 patients [9%], P = .0001), and lower blood oxygen saturation levels at discharge (87% vs 92%, P = .03).ConclusionsThe Fontan procedure in patients with right ventricular morphology is associated with longer postoperative hospitalization and worse early postoperative characteristics (ventricular dysfunction and atrioventricular valve regurgitation) as well as higher rates of early, transient signs of sub-optimal postoperative hemodynamics compared to those with left ventricular morphology.

"Parenteral" Diarrhea Associated With Pediatric Urinary Tract Infections: A Retrospective Chart Review.

The association of noninfectious diarrhea with extraintestinal infections such as otitis media, pneumonia, or febrile urinary tract infections (UTIs) is commonly known as parenteral diarrhea. Although this association has been described for over a century and parenteral diarrhea is mentioned in current reference literature, available evidence for this association seems to be limited. The primary research question was to determine if there is an association between UTIs and reports of diarrhea. A retrospective chart review was performed using the medical records from October 1, 2017, to March 29, 2019 at our tertiary pediatric medical center. We searched for all cases of afebrile and febrile UTIs evaluated in the pediatric emergency department or admitted directly to the hospital for treatment. All children younger than 5 years were eligible for inclusion. Exclusion criteria included children with recent urological procedures, known urinary tract disease, immune suppression, sepsis, or known gastrointestinal diseases. The medical records of each of the pediatric patients with culture-positive UTIs were reviewed for reports of concurrent diarrhea or diarrhea-like illness. In addition, using a comparative quantitative design, we performed a retrospective chart review of all children younger than 5 years with 1 of 2 noninfectious chief complaints, head trauma, and extremity fractures, presenting during the same period to assess the background rate of reported diarrhea.This research project received the approval of the University of South Alabama's Institutional Review Board. A total of 236 children with a culture-positive UTI presented to our pediatric medical center from October 1, 2017 to March 29, 2019. Reports of diarrhea were documented in the medical record for 44 of the 236 UTIs (18.6%). Escherichia coli was the predominant infectious agent in 32 of the 44 children (72.7%) with culture-positive UTIs and diarrhea. Of the 368 comparison group patients who visited the pediatric emergency department for a noninfectious complaint, diarrhea was reported in 2 patients (0.01%). Our retrospective review of pediatric patients younger than 5 years with culture-proven UTIs found an association of reported diarrhea. It is our opinion based on our single-center retrospective chart review that the current available evidence suggests an association between UTIs and extraintestinal diarrhea. Future prospective studies are recommended to confirm this association.

Congenital cholesteatoma: Clinical features and surgical outcomes

ObjectivesA typical presentation of congenital cholesteatoma (CC) is asymmetric conductive hearing loss (CHL). As CHL is usually associated with middle ear effusion, diagnosis of CC is frequently delayed. This study aimed to describe the clinical characteristics, treatment and outcomes of children with CC. MethodsThe medical files of children diagnosed with CC at a large tertiary pediatric medical center during 2000–2019 were reviewed. The primary outcome measures were: presenting symptoms, surgical findings, stage of disease, recurrence rate and hearing outcome. Imaging findings and the size of mastoid air cells were assessed in CT scans. ResultsThirty-nine children were diagnosed with CC. The presenting symptom was unilateral CHL in 85%, with an average speech reception threshold of 41.5 ± 13.7 dB in the affected ear. The mean time from first symptoms to diagnosis was 1.3 years. The surgical approach was exploratory tympanotomy in 25% and canal wall up mastoidectomy in 69%. Seventy percent of the children presented with Potsic stage III-IV. The mean postoperative speech reception threshold was 26.4 ± 12.2 dB (P = 0.002). Recurrence of cholesteatoma occurred in 38% of the patients, mostly in stage III-IV. Mastoid air cell size was significantly smaller on the affected than the unaffected side. ConclusionsIn children with persistent unilateral or asymmetric conductive hearing loss, CC should be suspected. Late diagnosis of CC is associated with a high recurrence rate. This highlights the need to promote awareness to the disease among primary physicians in the community health care system.