Pediatric Tertiary Care Center Research Articles

Virtual Learning and Youth-Onset Type 2 Diabetes during the COVID-19 Pandemic

Introduction: Cases and severity of presentation of youth-onset type 2 diabetes (Y-T2D) increased during the COVID-19 pandemic, yet the potential drivers of this rise remain unknown. During this time public health mandates paused in-person education and limited social interactions, resulting in radical lifestyle changes. We hypothesized that the incidence and severity of presentation of Y-T2D increased during virtual learning amidst the COVID-19 pandemic. Materials and Methods: We conducted a single-center retrospective chart review to identify all newly diagnosed cases of Y-T2D (n = 387) at a pediatric tertiary care center in Washington, DC during three predetermined learning periods as defined by learning modality in Washington, DC Public Schools: pre-pandemic in-person learning (March 11, 2018–March 13, 2020), pandemic virtual learning (March 14, 2020–August 29, 2021), and pandemic in-person learning (August 30, 2021–March 10, 2022) periods. Results: Incident cases were stable during pre-pandemic in-person learning (3.9 cases/month, 95% CI: 2.8–5.4 cases/month), increased to a peak during virtual learning (18.7 cases/month, 95% CI: 15.9–22.1 cases/month), and declined with return to in-person learning (4.3 cases/month, 95% CI: 2.8–6.8 cases/month). Y-T2D incidence was 16.9 (95% CI: 9.8–29.1, p < 0.001) and 5.1-fold higher (95% CI: 2.9–9.1, p < 0.001) among non-Hispanic Black and Latinx youth, respectively, throughout the study period. Overall COVID-19 infection rates at diagnosis were low (2.5%) and were not associated with diabetes incidence (p = 0.26). Conclusions: This study provides timely insights into an important and modifiable correlate of Y-T2D incidence, its disproportionate impact on underserved communities, and the need to consider the effects on long-term health outcomes and preexisting healthcare inequities when designing public policy.

Trends in Histrelin Implantation at a Pediatric Tertiary Care Center

IntroductionDetermine procedural outcomes and identify changing trends of utilization among patients undergoing histrelin implantation at a large pediatric tertiary care center over 15 y. MethodsRetrospective review of all patients undergoing histrelin implantation between January 2008 and April 2022. ResultsA total of 746 patients underwent 1794 unique procedures (1364 placements/replacements, 430 removals). Procedures were performed in the clinic (1071, 60%), sedation unit (630, 35%), and operating room (93, 5%). A total of 14 (0.8%) complications were identified, including two patients that required early implant removal and one patient requiring antibiotics. Implants were placed for central precocious puberty (CPP, 579) or gender dysphoria (GD, 167). Cohort included 25.9% males and 74.1% females with mean age of implantation of 9.48 y (SD: 2.34, range: 1.05-17.34). The GD group is comprised of 52.4% males and 47.6% females, compared to 18.3% males and 81.7% females in the CPP. Significant difference was identified for mean age at placement by indication (CPP 8.65 y versus GD 12.34, P < 0.001). New patient referrals and implant procedures increased significantly over 14 y. Yearly frequency of patients receiving implants for CPP and GD increased significantly (P < 0.001), with proportion of GD patients increasing from 7% to 32%. ConclusionsHistrelin procedures have increased in frequency overall with the greater increase noted in the GD cohort. The development of a streamlined process and a dedicated team have enabled histrelin procedures to be safely performed in the clinic setting for most, with a very low complication rate.

1280-P: The Maturity-Onset Diabetes of the Young (MODY) Calculator Overestimates MODY Probability in Hispanic Youth

The applicability of the MODY probability calculator (Shields et al., Diabetologia, 2012) to non-European populations remains unknown. We aimed to test its utility in Hispanic youth. We performed a retrospective chart review of Hispanic youth (&amp;lt;23 years) with diabetes (n=2033) in a large, pediatric tertiary care center in the U.S. We calculated MODY probability for all subjects, splitting into two cohorts in line with the two equations underlying the MODY calculator: Individuals who were started on insulin within 6 months of diabetes diagnosis (Cohort 1) and those who were not (Cohort 2). MODY genetic testing was carried out on limited individuals based on clinicians’ suspicion of MODY. Cohort 1 and Cohort 2 comprised 1566 (mean age: 15.8 ± 4.7 years, 49% female) and 467 youth (mean age: 17.2 ± 3.5 years, 62% female), respectively. Of 1566 early insulin treated individuals in cohort 1, the mean MODY probability was 6% suggesting an expected 6% (94 individuals) with MODY. This is higher than previous prevalence estimates (1-4%) and much higher than the number identified from genetic testing (6 individuals out of 30 tested). For the 467 non-insulin treated individuals (cohort 2), the mean probability was 62% suggesting an expected 289 individuals with MODY, which again was much higher than previous prevalence estimates and the number identified from genetic testing (10 individuals out of 12 tested). Individuals with a confirmed pathogenic variant in a MODY-associated gene were more likely to have a younger age at diagnosis and lower non-HDL (cohort 1) or lower HbA1c (cohort 2). However, both cohorts had similar MODY probabilities compared to the remaining individuals in each cohort. The MODY calculator likely overestimates the probability of MODY in Hispanic youth, particularly those who are not early insulin treated, where young-onset type 2 diabetes is the more likely diagnosis. The calculator needs updating for use in this population. Disclosure G.Alarcon: None. A.D.Nguyen: None. A.Jones: None. M.J.Redondo: None. B.Shields: None. M.Tosur: Advisory Panel; Provention Bio, Inc.

Aetiologies of low alkaline phosphatase in a Canadian Paediatric Tertiary Care Centre.

Increasingly, laboratories flag low serum alkaline phosphatase (sALP) that are age-and sex-specific in paediatrics. The aim of this study was to report clinical manifestations of paediatric patients with age-and sex-specific low sALP, thereby increasing awareness of its potential aetiologies. This retrospective Canadian tertiary care paediatric hospital study assessed all sALP of ambulatory patients aged less than 18 years from 2015 to 2017. The hospital used a Beckman Coulter AU assay to measure sALP and compared values to the Canadian age-and sex-specific reference intervals from CALIPER. All children who had at least one subnormal age-and sex-specific sALP were evaluated. A review of medical charts of included patients was performed and demographic characteristics, medical history and diagnosis were collected, and categorized under groups of medical disorders. Of 11,874 included patients, 1,001 patients (9.2%) had low sALP. Of those, 48% (485/1,001) had transient low sALP activity and 9.6% (96/1,001) had persistently low sALP. Prolonged immobilization and inflammatory bowel disease represented the main aetiologies for persistently low sALP. Interestingly, 13.5% (13/96) of patients with persistently low sALP had no apparent aetiology. Our results report aetiologies of low sALP in a Canadian paediatric population using age-and sex-specific Canadian reference ranges. This study highlights that healthcare providers should be aware that a low sALP may have clinical significance and should be repeated if warranted based on further clinical assessment.

Diagnostic accuracy of upper gastrointestinal series in children with suspected intestinal malrotation

Intestinal malrotation (IM) results from an altered or incomplete rotation of the fetal midgut around the superior mesenteric artery axis. The abnormal anatomy of IM is associated with risk of acute midgut volvulus which can lead to catastrophic clinical consequences. The upper gastro-intestinal series (UGI) is addressed as the gold standard diagnosis procedure, but a variable failure degree has been described in literature. The aim of the study was to analyze the UGI exam and describe which features are the most reproducible and reliable in diagnosing IM. Medical records of patients surgically treated for suspected IM between 2007 and 2020 at a single pediatric tertiary care center were retrospectively reviewed. UGI inter-observer agreement and diagnostic accuracy were statistically calculated. Images obtained with antero-posterior (AP) projections were the most significant in terms of IM diagnosis. Duodenal-Jejunal Junction (DJJ) abnormal position resulted to be the most reliable parameter (Se = 0.88; Sp = 0.54) as well as the most readable, with an inter-reader agreement of 83% (k = 0.70, CI 0.49–0.90). The First Jejunal Loops (FJL), caecum altered position and duodenal dilatation could be considered additional data. Lateral projections demonstrated an overall low sensitivity (Se = 0.80) and specificity (Sp = 0.33) with a PPV of 0.85 and a NPV of 0.25. UGI on the sole AP projections ensures a good diagnostic accuracy. The position of the third portion of the duodenum on lateral views showed an overall low reliability, therefore it was not helpful but rather deceiving in diagnosing IM.

Macitentan in the Young-Mid-term Outcomes of Patients with Pulmonary Hypertensive Vascular Diseasetreated in a Pediatric Tertiary Care Center.

Pulmonary hypertension (PH) is a severe hemodynamic condition with high morbidity and mortality. Approved targeted therapies are limited for pediatric subjects, and treatments are widely adopted from adult algorithms. Macitentan is a safe and effective drug used for adult PH, but data on pediatric patients are limited. In this prospective single-center study, we investigated mid- and long-term effects of macitentan in children with advanced pulmonary hypertensive vascular disease. Twenty-four patients were enrolled in the study for treatment with macitentan. Efficacy was determined by echo parameters and brain natriuretic peptide levels (BNP) at 3months and 1year. For detailed analysis, the entire cohort was subgrouped into patients with congenital heart disease-related PH (CHD-PH) and non-CHD-PH patients, respectively. Mean age of the patients was 10.7±7.6 years; median observation period was 36months. Twenty of 24 patients were on additional sildenafil and/or prostacyclins. Two of 24 patients discontinued because of peripheral edema. Within the entire cohort, BNP levels and all echo measures such as right ventricular systolic pressure (RVSP), right ventricular end-diastolic diameter (RVED), tricuspid annular plane systolic excursion (TAPSE), pulmonary velocity time integral (VTI), and pulmonary artery acceleration time (PAAT) improved significantly after 3months (p ≤ 0.01), whereas in the long term significant improvement persisted for BNP levels (-16%), VTI (+14%) and PAAT (+11%) (p<0.05). By subgroup analysis, non-CHD PH patients showed significant improvements in BNP levels (-57%) and all echo measures (TAPSE +21%, VTI +13%, PAAT +37%, RVSP -24%, RVED -12%) at 3months (p≤0.01), whereas at 12months, improvements persisted (p<0.05) except for RVSP and RVED (nonsignificant). In CHD-PH patients, none of the measures changed (nonsignificant). 6-MWD (distance walked in 6 minutes)slightly increased but was not statistically evaluated. Data presented herein account for the largest cohort of severely affected pediatric patients receiving macitentan. Overall, macitentan was safe and associated with significant beneficial effects and sustained positive signals after 1year, albeit in the long term disease progression remains a major concern. Our data suggest limited efficacy in CHD-related PH, whereas favorable outcomes were mainly driven by improvements in patients with PH not related to CHD. Larger studies are needed to verify these preliminary results and to prove efficacy of this drug in different pediatric PH entities.

Who is the real team leader? Comparing leadership performance of the team leader and CPR Coach during simulated cardiac arrest

PurposeTo describe the leadership performance of team leaders and CPR Coaches, and to determine if there is a correlation between leadership scores and CPR performance during management of simulated pediatric cardiac arrest events. MethodsThis is a secondary analysis of data from a prior randomized controlled trial. We observed the performance of both team leaders and CPR coaches during the management of an 18-minute simulated cardiac arrest scenario which was run for 20 resuscitation teams comprised of CPR-certified professionals from four pediatric tertiary care centers. CPR Coaches were responsible for providing real-time verbal feedback of CPR performance to compressors. Two raters were trained to use the Behavioral Assessment Tool (BAT) to assess leadership performance of the team leader and CPR Coach. BAT scores of team leaders and CPR coaches were compared and linked with objective CPR performance. ResultsThere was no significant difference between the BAT scores of team leaders and CPR coaches (median score 27/40 vs 28.8/40, p = 0.16). Higher BAT scores of team leaders were significantly associated with higher percentage of excellent CPR (r = 0.52, p = 0.02), while higher BAT scores of CPR coaches were significantly associated with higher chest compression fraction (r = 0.48, p = 0.03). ConclusionsBoth team leaders and CPR coaches have similarly high leadership performance during the management of simulated cardiac arrest. Leadership behaviors were associated with quality of CPR performance.Clinical Trial Registration: Registration ID: NCT02539238; https://www.clinicaltrials.gov.

An 11-Year Review of Lactobacillus Bacteremia at a Pediatric Tertiary Care Center.

To inform clinical decisions on the use of probiotics in a pediatric inpatient setting, we sought to determine the number of cases of Lactobacillus bacteremia as well as associated patient characteristics in a tertiary-care pediatric hospital over an 11-year period. Cases of Lactobacillus bacteremia among admitted patients were identified through positive blood culture reports. The clinical chart for each case was reviewed for presenting symptoms and risk factors such as probiotic use, presence of a central venous catheter, immunocompromised state, impaired intestinal function, and age below 3 months. Concurrent total inpatient probiotic administration was assessed. Over an 11-year period, 8 cases of Lactobacillus bacteremia were identified among 127 845 hospital admissions. All cases were associated with systemic signs of infection. Lactobacillus bacteremia patients most frequently had underlying impaired intestinal function and a central venous catheter. Three cases had a history of probiotic use. The peak number of annual cases did not coincide with the peak number of inpatients who received probiotics. Lactobacillus bacteremia is uncommon and did not correlate with doses of probiotics-administered in the hospital. However, certain populations may be at higher risk and require extra consideration in clinical decision-making regarding use of probiotics.

Treatment Guideline Nonadherence Pretransport Associated With Need for Higher Level of Care in Children Transferred to a Pediatric Tertiary Care Center for Status Epilepticus.

We sought to investigate the association between adherence to the American Epilepsy Society (AES) 2016 guidelines for management of convulsive status epilepticus (SE) and clinical outcomes among children requiring interhospital transport for SE. We hypothesized that pretransport guideline nonadherence would be associated with needing higher level of care posttransfer. This was a retrospective cohort study of children aged 30 days to 18 years transferred to our pediatric tertiary center from 2017 to 2019 for management of SE. Their care episodes were classified as 2016 American Epilepsy Society guideline adherent or nonadherent. There were 40 referring hospitals represented in this cohort. Of 260 care episodes, 55 (21%) were guideline adherent, 184 (71%) were guideline nonadherent, and 21 (8%) had insufficient data to determine guideline adherence. Compared with the adherent group, patients in the nonadherent care group had longer hospitalizations (32 hours [17-68] vs 21 hours [7-48], P = 0.006), were more likely to require intensive care unit admission (47% vs 31%), and less likely to be discharged home from the emergency department (16% vs 35%; χ 2 test, P = 0.01). Intubation rates did not differ significantly between groups (25% vs 18%, P = 0.37). When we fit a multivariable model to adjust for confounding variables, guideline nonadherence was associated with need for higher level of care (odds ratio, 2.04; 95% confidence interval, 1.04-3.99). Treatment guideline adherence did not improve over the 3-year study period (2017: 22%, 2018: 19%, 2019: 29% [χ 2 test for differences between any 2 years, P = 0.295]). Guideline nonadherence pretransport was associated with longer hospitalizations and need for higher level of care among children transferred for SE at our institution. These findings suggest a need to improve SE guideline adherence through multifaceted quality improvement efforts targeting both the prehospital and community hospital settings.

ANESTHETIC MANAGEMENT OF LIVER RESECTION IN PEDIATRIC HEPATOBLASTOMA PATIENTS

Background – Children who present for liver resection are a challenge to anesthesiologists. They require adequate preoperative assessment and planning to improve their postoperative outcomes. A balanced technique providing organ protective anesthesia and hemodynamic stability ensures optimal surgical results. Anaesthesia for hep Result: atic resection has seen great changes with improvements in surgical technique and a better understanding of the underlying physiology. Good communication and interdisciplinary strategization between the pediatric oncologist, surgeon and the anesthesiologist also facilitate a good outcome. A retrospective review was performed on 20 cases of pediatric hepatoblastoma planned for partial hepatectomy in a pediatric tertiary care center in South India, over a 3-year period. Conclusion: In this study, general anesthesia with epidural analgesia and goal directed uid management with monitoring of central venous pressure and mean blood pressure during liver resection was found to be acceptable for pediatric liver resection without any signicant complications

Web-Based Multistate Disaster Rehearsal of Concepts Exercises

Introduction:The Mountain Plains Regional Disaster Health Response System (Mountain Plains RDHRS) works to build disaster capacity across US Federal Region VIII, a rural western six-state region. It conducts an annual rehearsal of concepts and exercises to identify gaps and inform policy development. In 2022, a multi-state exercise was conducted involving responders from individual hospitals coordinating with Healthcare Coalitions and State Public Health. These responses rolled up to a multi-state emergency operations center overseen by the Mountain Plains RDHRS.Method:A fictitious mass multi-state botulism incident generated a pediatric surge across the region. Individual patient cards with demographic information were given to a set of hospitals in participating states. The communication pathways within states were identified. Communication between local and regional pediatric transfer centers were assessed. Overall situational awareness was tracked.The exercise format was incident occurrence and notifications by normal channels and a Zoom conference call held on day one. Situational awareness and patient movement occurred in multiple Zoom rooms on day two. An after-exercise review occurred by Zoom on day three including all participants from the exercise.Results:There was generally good information flow within states, but minimal information exchange across states There was poor regional situational awareness with a lack of complete patient lists and transfers. The Mountain Plains RDHRS planned to exceed the hospital’s patient capacities with a large number of pediatric patients to practice patient movement across state lines. Instead the hospitals showed a surprising willingness to keep and manage critical pediatric patients instead of transferring to tertiary care pediatric centers. This was identified as a consequence of the COVID-19 experience.Conclusion:Web-based exercises vertically spanning responses from individual hospitals to multi-state regional entities are feasible. This exercise demonstrated multiple gaps in regional disaster response.

Decrease in vancomycin MICs and prevalence of hGISA in MRSA and MSSA isolates from a German pediatric tertiary care center

PurposeResistance of Staphylococcus aureus to vancomycin includes a general increase of minimal inhibitory concentrations (MIC) within the susceptible range over time (Vancomycin MIC Creep) and the presence of a subset of the bacterial population that expresses resistance (heterogeneous glycopeptide-intermediate S. aureus; hGISA). Increased MICs have been associated with adverse clinical outcomes. However, the vancomycin MIC creep is not a uniform trend suggesting the importance of regional surveys.MethodsWe performed a retrospective analysis at a German pediatric tertiary care hospital. Isolates from 2002 to 2017 were selected which were newly identified methicillin-resistant S. aureus (MRSA) or samples from invasive methicillin-susceptible S. aureus (MSSA) or MRSA infections. Vancomycin and oxacillin MICs as well as GISA/hGISA were measured using MIC test strips and resistance was evaluated over time.ResultsA total of 540 samples were tested, 200 from the early (2002–2009) and 340 from the later period (2010–2017). All samples were vancomycin susceptible, but the MIC was higher for the earlier samples as compared to the later ones (1.11 vs 0.99; p < 0.001). 14% of the samples were hGISA, GISA strains were not detected. Again, vancomycin resistance decreased over time with 28 vs. 6% hGISA (p < 0.001). There was no significant difference between MRSA and MSSA samples with respect to vancomycin MIC and hGISA prevalence.ConclusionThis study shows a decreasing trend for both MIC values and presence of hGISA strains highlighting the importance of monitoring local susceptibilities. Vancomycin remains a first-line treatment option for suspected severe infection with Gram-positive cocci and proven infection with MRSA.

Utility of Intraoperative Radiographs in Pediatric Cochlear Implant Surgery.

To evaluate the role of intraoperative radiographs to confirm electrode position following pediatric cochlear implantation (CI). Retrospective chart review. Single tertiary care pediatric center. A retrospective chart review was conducted, including all pediatric patients undergoing CI at UPMC Children's Hospital of Pittsburgh over a 13-year period. We identified 326 patients undergoing 492 procedures. Across the cohort, there were 7 cases that required intraoperative electrode reinsertion due to malposition or presumed malposition. For 6 of the 7 cases, intraoperative X-ray identified electrode malposition. Neural response telemetry (NRT) testing was also abnormal for 4 of these cases prior to reinsertion. Implantation of Cochlear's Slim Modiolar electrode was associated with an abnormal perioperative X-ray (odds ratio [OR]: 9.2, p = 0.03) and increased change in management (OR: 9.2, p = 0.03) compared to Cochlear's Contour Advance (CA). Incidence of abnormal X-rays was 1.24% overall, 4% in the Slim Modiolar group, and 0.3% in the CA group. The Slim Modiolar electrode accounted for 4 of 7 cases requiring reinsertion, and in all 4 of these cases, electrode fold-over was identified on the X-ray. NRT was normal in 1 of these 4 cases. The use of Cochlear's Slim Modiolar electrode was associated with a significantly increased risk of abnormal intraoperative X-ray compared to the CA electrode. Given the risk of fold-over with routine insertion and normal electrical testing using the Slim Modiolar electrode, we recommend routine use of intraoperative skull X-ray to confirm electrode position.

Thoracolumbar Scoliosis in Pediatric Patients With Loeys-Dietz Syndrome: A Case Series.

Background Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder that predominantly affects cardiovascular, skeletal, and craniofacial structures. Associated thoracolumbar scoliosis in LDS can be challenging to manage, though other etiologies of pediatric scoliosis have better-defined management guidelines. We examined our institutional experience regarding the treatment of pediatric patients with LDS and scoliosis. Methodology In this retrospective study, all patients seen at our pediatric tertiary care center from 2004 through 2018 with a diagnosis of LDS were reviewed, and those with radiographic diagnoses of scoliosis (full-length scoliosis X-rays) were included. Demographic, clinical, and radiographic parameters were collected, and management strategies were reported. Results A total of 39 LDS patients whose ages ranged between seven and 13 years were identified.A total of nine patients were radiographically diagnosed with scoliosis, but three patients were excluded due to incomplete medical records, leaving six patients. The median age at scoliosis diagnosis was 11.5 years, with a median follow-up of 51 months. Two patients were successfully managed with observation (average initial Cobb angle (CA): 14°, average final CA: 20.5°). Two were braced, one successfully (initial CA: 15°, final CA: 30°) and one with a progressive disease requiring surgery (initial CA: 40°, final CA: 58°). Of the two who were offered surgical correction, one underwent surgery with a durable correction of spinal deformity (CA: 33° to 19°). One patient underwent a recent correction of aortic root dilatation and was not a candidate for scoliosis surgery. Conclusions Principles of adolescent idiopathic scoliosis management such as bracing for CA of 20-50° and surgery for CA of >50° can be applied to LDS patients with good outcomes. This augments our understanding of the treatment algorithm for pediatric patients with LDS.

A94 EXPLORING THE SOCIOECONOMIC BURDEN OF PEDIATRIC INFLAMMATORY BOWEL DISEASE: A SURVEY OF FAMILIES AND PEDIATRIC PROVIDERS

Abstract Background Rates of pediatric inflammatory bowel disease (IBD) have been increasing significantly over recent decades, contributing to the rising chronic disease burden across pediatrics. Currently, there is very limited literature exploring the financial burden on families of children with IBD. In fact, there is only one Canadian study in this area despite Canada having one of the highest rates of pediatric IBD in the world. Purpose The goal of this study is to better understand the socioeconomic burden of pediatric IBD at our institution and compare institutional practices across the country. Method The study took place at a large, tertiary care pediatric centre in Edmonton, Alberta, Canada between October 2022 and January 2023. Two separate electronic surveys were developed and distributed to all families of children with IBD at our institution (N= ~400) and pediatric IBD providers across the country (N= ~45) respectively. Surveys explored demographic information, financial impacts of IBD diagnosis and perceptions around pediatric IBD care and financial support. Result(s) Interim results (N=3) indicate dietary therapy costs, missed time off work and school and time off for IBD treatment as considerable burdens on families. Across the country, initial provider data (N=6) suggests significant variability in clinical practice, allied health support and financial support for families. There is overwhelming agreement among providers that the socioeconomic burdens on families is significant. Further data and regression analysis is ongoing. Conclusion(s) This is the first study in Canada to directly explore the socioeconomic burden on families of children with IBD. Results indicate good correlation between provider awareness and the increased financial burden on families but also considerable variation in practice across the country. Data suggest further research and advocacy is required to better support patients, however various quality improvement opportunities exist both locally and beyond. Please acknowledge all funding agencies by checking the applicable boxes below None Disclosure of Interest None Declared

Awareness of Periconceptional Folic Acid in Parents of Children Affected by Spina Bifida - A Pilot Study.

Spina bifida is a congenital malformation involving an open vertebral column resulting from failure in neural tube closure. It is among the most frequently occurring birth defects, observed in 1-3 cases per 1,000 live births worldwide. Conventionally requiring surgical repair, it can cause severe neurologic and musculoskeletal complications. However, consumption of prophylactic folic acid in mothers, at least 3 months before to 12 weeks after conception (periconceptional) has been shown to reduce the incidence of spina bifida by approximately 75%. This makes ascertaining parental understanding of such benefits critical. Therefore, this study provides baseline information on the awareness of periconceptional folic acid among parents whose children previously underwent surgical repair of spina bifida defects. The study subjects constituted 80 parents whose biological children had undergone surgical repair of spina bifida defects from 2014 to 2021 at a large paediatric tertiary care centre in South India. Upon providing informed consent, the subjects answered a cross-sectional telephonic survey containing 21 questions aimed at exposing their understanding of folic acid and its association with spina bifida. None of the mothers had consumed folic acid before conception. However, 75% of them had consumed it as prescribed by their obstetrician during the first trimester of pregnancy. Finally, only 35% of them were aware of its role in preventing spina bifida. The awareness of periconceptional folic acid and its preventive role in spina bifida was low amongst parents whose children were once treated for same congenital abnormality.

Right ventricle speckle tracking in bronchopulmonary dysplasia: one-year follow-up

BackgroundBronchopulmonary dysplasia (BPD) is still a major concern in preterm infants and adequate screening methods for secondary right ventricle (RV) failure are needed. Early detection could be aided by taking measurements of RV deformation using speckle tracking echocardiography. A prospective longitudinal study was carried out over 28 months at a tertiary care pediatric cardiology center. Preterm infants < 32 weeks gestational age (GA) were eligible for the study. Enrolled patients were separated in two groups: NO-BPD or BPD. At three timepoints over the first year of life, echocardiogram measurements were performed. Right ventricle strain was studied using speckle tracking analysis and compared to conventional function parameters.ResultsFifty patients were enrolled in the study, 22 in the NO-BPD group and 28 in the BPD group. RV strain showed no statistical differences between groups. However, the BPD group showed worse RV function than the NO-BPD group, using speckle tracking analysis and other conventional parameters. During the study follow-up, an improvement trend is shown in RV strain.ConclusionsRV longitudinal strain and strain rate derived by speckle tracking is feasible in preterm infants. Although there seems to be a good correlation between RV strain and BPD severity, the results of this study were not conclusive. More studies should be carried out to investigate the optimum echocardiographic screening model of RV dysfunction in BPD patients.

Prevalence of Cochlear Nerve Deficiency and Hearing Device Use in Children With Single-Sided Deafness.

This study aimed to assess the prevalence of cochlear nerve deficiency (CND) in a cohort of pediatric patients with single-sided deafness (SSD). A secondary objective was to investigate trends in intervention and hearing device use in these children. Case series with chart review. Pediatric tertiary care center. Children ages 0 to 21 years with SSD (N = 190) who underwent computerized tomography (CT) and/or magnetic resonance imaging (MRI) were included. Diagnostic criteria for SSD included unilateral severe-to-profound sensorineural hearing loss with normal hearing sensitivity in the contralateral ear. Diagnostic criteria for CND included neuroradiologist report of an "aplastic or hypoplastic nerve" on MRI or a "stenotic cochlear aperture" on CT. The prevalence of CND was 42% for children with CT only, 76% for children with MRI only, and 63% for children with both MRI and CT. Of the children with MRI and CT, there was a 90% concordance across imaging modalities. About 36% of children with SSD had hearing devices that routed sound to the normal hearing ear (ie, bone conduction hearing device/contralateral routing of signal), while only 3% received a cochlear implant. Approximately 40% did not have a hearing device. Hearing device wear time averaged 2.9hours per day and did not differ based on cochlear nerve status. There is a high prevalence of CND in children with SSD. Cochlear nerve status should be confirmed via MRI in children with SSD. The limited implementation and use of hearing devices observed for children with SSD reinforce the need for increased support for early and continuous intervention.

Speech Outcomes Following Operative Management of Velopharyngeal Dysfunction (VPD) in Non-Syndromic Post-Palatoplasty Cleft Palate Patients.

Approximately 30% of patients with a history of repaired cleft palate (CP) go on to suffer from velopharyngeal dysfunction (VPD). This study discusses the operative management of VPD and postoperative speech outcomes in a cohort of CP patients. An academic tertiary pediatric care center. Retrospective cohort study. Patients with history of repaired CP (Veau I-IV) who underwent operative management of VPD between January 1st, 2010 and December 31st, 2020. Operative modalities were posterior pharyngeal flap (PPF), sphincter pharyngoplasty (SPP), Furlow palate re-repair, and buccal myomucosal flap palate lengthening (PL). The primary outcome measure is postoperative speech improvement evaluated by the Pittsburgh Weighted Speech Scale (PWSS). 97 patients met inclusion criteria. 38 patients with previous straight-line primary palatoplasty underwent Furlow re-repair; these patients were significantly younger (7.62 vs 11.14, P < .001) and were more likely to have severe VPD per PWSS (OR 4.28, P < .01, 95% CI 1.46-12.56) when compared to VPD patients with previous Furlow repair. 21.1% of these patients required an additional non-revisional VPD procedure. The remaining patients underwent a non-revision procedure (26 PPF, 22 SPP, 11 PL); all experienced significant (P < .001 on paired t-test) reductions in PWSS total and subgroup VPD severity scores without difference in improvement between operation types. SPP was statistically associated with all-cause complication (OR 2.79, 95% CI 1.03-7.59, P < .05) and hyponasality (OR 3.27, 95% CI 1.112-9.630, P < .05). Furlow re-repair reduced need for additional VPD operations. Speech outcomes between non-revisional operations are comparable, but increased complications were seen in SPP.

Catastrophic and Critical Intraoperative Events during Pediatric Craniofacial Surgery.

The data reviewed included the operative procedures of two senior craniofacial surgeons over an 18-year period at a tertiary care pediatric craniofacial center. Catastrophic or critical intraoperative events were defined as events requiring the activation of an emergency code during surgery. The operative details and acute outcomes were reviewed and analyzed. This study reviewed 7214 procedures performed between January 2002 and January 2019. There were 2072 (29%) cases classified as major craniofacial procedures (transcranial, mixed trans-and-extracranial, or major extracranial facial osteotomies), and code events occurred in 14 cases (0.67%; one in 148 patients): venous air embolism (n = 4), cardiac complications (n = 3), major hemorrhage (n = 3), trigeminocardiac reflex (n = 2), acute intracranial hypertension (n = 1), and acute airway obstruction (n = 1). Two cases (14%) experienced a critical event that was anesthesia-related. Cardiac arrest requiring compressions and/or defibrillation was necessary for eight patients. There were no mortalities. Surgery was curtailed in seven cases and successfully completed in seven patients. Catastrophic life-threatening events during pediatric craniofacial surgery are, fortunately, rare. In our institution, experienced teams in the management of catastrophic and critical intraoperative events during major pediatric craniofacial procedures resulted in no mortalities.