OBJECTIVE:To evaluate genetic testing utilization and diagnostic yield in infants with esophageal atresia (EA)/tracheoesophageal fistula (TEF) over the past 12 years to inform future practices and individualize prognostication and management. STUDY DESIGN:A retrospective cohort study was performed for all infants with EA or EA/TEF hospitalized between January 2011 and January 2023 at a quaternary children's hospital. For each infant, demographic information, prenatal and postnatal history, and genetic testing were reviewed. RESULTS:There were 212 infants who were classified as follows: 1) complex/syndromic with EA/TEF plus an additional major anatomic anomaly (n = 114, of which 74 met VACTERL criteria); 2) isolated/nonsyndromic EA/TEF (n = 88) and 3) isolated/nonsyndromic EA (n = 10). A range of genetic tests were sent with varying diagnostic rates including karyotype analysis in 12 (all with complex/syndromic phenotypes and all positive), chromosomal microarray analysis in 189 (114 of whom were complex/syndromic with an overall diagnostic rate of 3/189), single gene testing for CHD7 in 18 (4 positive), and exome analysis in 37 complex/syndromic patients (8 positive). CONCLUSIONS:EA/TEF with and without additional anomalies is genetically heterogeneous with a broad range of associated phenotypes. While the genetic etiology of EA/TEF with or without VACTERL remains largely unknown, genome wide testing (exome or genome) including copy number analysis is recommended over chromosomal microarray testing. We anticipate that expanded genetic/genomic testing modalities such as RNA sequencing and tissue specific molecular testing are needed in this cohort to improve our understanding of the genomic contributors to EA/TEF.

Background Recognition of the patient and family's diverse backgrounds and language preference is critical for communicating effectively. In our hospital's electronic health record, a patient or family's identified language for communication is documented in a discrete field known as “preferred language.” This field serves as an interdepartmental method to identify patients with a non-English preferred language, creating a bolded banner for non-English speakers easily identifiable by health care professionals. Despite the importance of documenting preferred language to facilitate equitable care, this field is often blank. Objectives Using the Institute for Healthcare Improvement's Model for Improvement, our team sought to increase preferred language documentation within the neonatal intensive care unit (NICU) from a baseline of 74% in September 2021 to above 90% within 6 months. Methods A multidisciplinary team was assembled to address preferred language documentation. Our team incorporated guidance regarding preferred language documentation into a novel clinical decision support (CDS) tool aimed at addressing various safety and quality measures within the NICU. Our primary outcome metric was documentation of family's preferred language. Process measures included overall compliance with the CDS tool. A secondary outcome was the assessment of preferred language documentation accuracy. Results The average rate of preferred language documentation increased from a baseline of 74 to 92% within 6 months and is currently sustained at 96%. Moreover, our follow-up assessments found that 100% of a random sample of contacted patients (n = 88) had their language preferences documented correctly. Overall compliance with the CDS tool remained at 85% throughout the project. Conclusion Using a quality improvement framework coupled with a CDS initiative, our team was able to successfully and accurately improve preferred language documentation in our NICU. Future projects include strategies for more equitable care for patients with non-English preferences such as improved interpreter usage and discharge instructions in their preferred language.

Bacterial meningitis causes significant morbidity and mortality in infants. Lumbar punctures are often deferred until the results of blood cultures are known and sometimes not considered, making this population susceptible to a missed diagnosis. There are few studies describing the epidemiology of neonatal meningitis in quaternary neonatal intensive care unit settings. We describe the epidemiology of meningitis in a level IV neonatal intensive care unit; compare pathogens and rates of concordant bacteremia between infants with and without neurosurgical (NS) devices. Retrospective review of infants < 1 year of age in the Children's Hospital of Philadelphia neonatal intensive care unit with bacterial meningitis (June 2007-October 2021). Analysis included summary statistics, Wilcoxon rank sum, Chi square, and Fisher exact tests. We identified 101 episodes of bacterial meningitis (95 infants). 9 infants died. At diagnosis, 26 infants (27%) had NS devices. Group B streptococcus (GBS) and Escherichia coli (E coli) were most common pathogens, however, coagulase-negative staphylococci and Staphylococcus aureus (S aureus) predominated among infants with NS devices. While 86% had positive blood cultures in the absence of a NS device, only 14% of episodes with NS devices had concomitant bacteremia (P < .0001). Although Group B streptococcus and E coli remain most prevalent overall, coagulase-negative staphylococci and S aureus were common pathogens in NS patients. Infants with NS devices rarely had concomitant bacteremia. Meningitis was diagnosed in the absence of a positive blood culture in 36% of episodes, underscoring the importance of developing guidance for lumbar punctures in infants evaluated for sepsis.

Background Thrombocytopenia (<150,000 platelets/ul blood) is frequent among neonatal intensive care unit (NICU) patients, including 70% of extremely low birth weight infants. Severe thrombocytopenia is often treated with prophylactic transfusions of donated adult platelets in an effort to prevent bleeding. However, current transfusion practices actually increase bleeding, neurocognitive impairment, respiratory complications, and death in preterm neonates. This is likely due to physiologic differences in adult vs neonatal platelets. Thus, reducing adult platelet exposure will optimize neonatal outcomes and also conserve limited donated platelet resources. Prior clinical studies have demonstrated that 10mL/kg platelet transfusions can effectively treat thrombocytopenia. Objective We aimed to standardize 10 ml/kg prophylactic platelet transfusions in our quaternary neonatal intensive care unit (NICU) to >80% of ordered platelet transfusions in non-bleeding patients. Methods We identified 1) preconceptions about platelet safety, 2) default order sets with our electronic ordering system, and 3) cultural standards related to transfusion dosing as key drivers of current practices. Infants with procedures/surgeries within 12 hours, on anticoagulation, bleeding, or on ECMO were excluded. The primary outcome measure was compliant 10 ml/kg platelet transfusions orders. Our balancing measure were 1) repeat platelet transfusions within 36 hours and 2) frequency of major bleeding within 72 h of transfusion. Results We created a graphic dashboard based on our electronic medical record to monitor platelet ordering in real time. To define baseline practices, we tracked transfusions in the year prior to initiating our study. Our baseline compliance for 10 ml/kg platelet transfusion orders was 14%. Instead, most prophylactic transfusions were dosed at 15-20 ml/kg. Our first plan-do-study-act (PDSA) cycle targeted education through messaging at staff meetings, on NICU-wide computer screensavers, and laminated reminder cards posted at unit workstations where platelet transfusions were ordered. A second PDSA cycle reinforced platelet dosing guidelines among clinicians through newsletters and staff huddle discussions. A third PDSA cycle targeted clinical decision support within the medical record that eased ordering through defaults to recommended dosing. After consensus agreement from neonatology, cardiology, emergency medicine, oncology, and pediatric intensive care unit clinicians, we implemented electronic order set changes hospital-wide to most effectively spread practice change related to neonatal platelet transfusion dosing across all hospital units. We monitored 165 transfusions among 55 neonates (2022-2024). Following our PDSA cycle interventions, we achieved >80% dosing compliance, noting special cause variation with sustained process change that resulted in a center line shift from 14% to 88% ‘compliant’ (10 ml/kg) transfusions. There was no change in the rate of subsequent platelet transfusions within 36 hours, nor an increase in major bleeding complications within 72 hours post-transfusion. Coupled with more restrictive platelet transfusion thresholds across our network, as described in network-wide transfusion guidelines (Gilmore et al, Transfusion 2024), platelet transfusions dropped by 44% in the first 6 months after electronic order changes (p<0.05 by two tailed t test). Conclusion A standardized prophylactic platelet transfusion dose of 10mL/kg was established through hospital-wide collaborative efforts. We used clinician education and electronic medical record technologies to improve platelet transfusion practices within our NICU, reducing platelet exposures that have been linked with adverse outcomes in preterm neonates. Standardized 10ml/kg platelet transfusion dosing will optimize safety and efficacy of neonatal platelet transfusions and conserve limited donated platelet supplies in line with current literature.

BACKGROUND:There are currently no commonly accepted standardized guidelines for management of cervical vessels at neonatal extracorporeal membrane oxygenation (ECMO) decannulation. This study investigates neonatal ECMO decannulation practices regarding management of the carotid artery and internal jugular vein, use of post-repair anticoagulation, and follow-up imaging. METHODS:A survey was distributed to the 37 institutions in the Children's Hospitals Neonatal Consortium. Respondents reported their standard approach to carotid artery and internal jugular vein management (ligation or repair) at ECMO decannulation by their pediatric surgery and cardiothoracic (CT) surgery teams as well as post-repair anticoagulation practices and follow-up imaging protocols. RESULTS:The response rate was 95%. Pediatric surgeons performed most neonatal respiratory ECMO cannulations (88%) and decannulations (85%), while all neonatal cardiac ECMO cannulations and decannulations were performed by CT surgeons. Pediatric surgeons overwhelmingly ligate both vessels (90%) while CT surgeons typically repair both vessels at decannulation (83%). Of the responding centers that repair, 28% (7) have a standard anticoagulation protocol after neck vessel repair. While 52% (13) of centers routinely image cervical vessel patency at least once post repair, most do not subsequently repeat neck vessel imaging. CONCLUSIONS:Significant practice differences exist between pediatric and CT surgeons regarding the approach to cervical vessels at neonatal ECMO decannulation. For those centers that do repair the vessels there is little uniformity in post-repair anticoagulation or imaging protocols. There is a need to develop standardized cervical vessel management guidelines for neonatal ECMO patients and to study their impact on both short- and long-term outcomes. LEVEL OF EVIDENCE:IV.

INTRODUCTION:Neonates have a high incidence of respiratory and cardiac perioperative events. Disease severity and indications for surgical intervention often dovetail with an overall complex clinical course and predispose these infants to adverse long-term neurodevelopmental outcomes and increased length of stay. Our aims were to describe severe and nonsevere early postoperative complications to establish a baseline of care outcomes and to identify subgroups of surgical neonates and procedures for future prospective studies.METHODS:Electronic health record data were examined retrospectively for a cohort of patients who had general anesthesia from January 26, 2015 to August 31, 2018. Inclusion criteria were full-term infants with postmenstrual age less than 44 weeks or premature infants less than 60 weeks postmenstrual age undergoing nonimaging, noncardiac surgery. Severe postoperative complications were defined as mortality, reintubation, positive blood culture, and surgical site infection. Nonsevere early postoperative outcomes were defined as hypoglycemia, hyperglycemia, hypothermia, hyperthermia, and readmission within 30 days.RESULTS:About 2569 procedures were performed in 1842 neonates of which 10.9% were emergency surgeries. There were 120 postoperative severe complications and 965 nonsevere postoperative outcomes. Overall, 30-day mortality was 1.8% for the first procedure performed, with higher mortality seen on subgroup analysis for patients who underwent exploratory laparotomy (10.3%) and congenital lung lesion resection (4.9%). Postoperative areas for improvement included hyperglycemia (13.9%) and hypothermia (7.9%).DISCUSSION:The mortality rate in our study was comparable to other studies of neonatal surgery despite a high rate of emergency surgery and a high prevalence of prematurity in our cohort. The early outcomes data identified areas for improvement, including prevention of postoperative glucose and temperature derangements.CONCLUSIONS:Neonates in this cohort were at risk for severe and nonsevere adverse postoperative outcomes. Future studies are suggested to improve mortality and adverse event rates.

Pediatric extreme thrombocytosis (EXT, platelet count > 1000 x 103/µL) is rare. In a single center retrospective analysis of hospitalized children with EXT, infants with congenital diaphragmatic hernia (CDH) were overrepresented. In general pediatric patients, EXT is usually secondary to infection or inflammation, but most of the 14 CDH patients with EXT had no identifiable inciting factor. Instead, there was evidence that splenic dysfunction and bone marrow hyperactivity underlied EXT in CDH patients. None were associated with bleeding or thrombosis. Our findings identify mechanisms underlying EXT, and aid clinical interpretation and management of EXT in the pediatric population.

Background: Extreme thrombocytosis (EXT, platelet count > 1000 x 10(3)/mu L) is an uncommon but potentially clinically significant finding. Primary EXT in the setting of myeloproliferative disorders is linked to thrombotic and/or bleeding complications more frequently than secondary EXT, which typically occurs in reaction to infection, inflammation, or iron deficiency. However, comorbidities have been reported in adults with secondary EXT. Clinical implications of EXT in children are not well defined, as prior studies targeted small and/or specialized pediatric populations. Objectives: Our objectives were to determine etiologies and sequelae of EXT in a hospitalized general pediatric patient population. Patients and Methods: We retrospectively analyzed EXT cases from a single-center pediatric cohort of similar to 80 000 patients over 8 years. Results: Virtually all cases (99.8%) were secondary in nature, and most were multifactorial. Many cases of EXT occurred in children under 2 years old (47%) and/or during critical illness (55%). No thrombotic or bleeding events directly resulted from EXT, confirming a paucity of clinical complications associated with EXT in pediatric patients. There were indications that neonatal hematopoiesis and individual genetic variation influenced some cases, in addition to certain diagnoses (eg, sickle cell anemia) and clinical contexts (eg, asplenia). Conclusion: Our findings confirm that thrombotic events related to EXT are rare in pediatric patients, which can inform the use of empiric anti-platelet therapy.

Extreme thrombocytosis (ET, platelet count >1000 × 10 3 /ul) is an uncommon clinical finding 1. Primary ET is associated with myeloproliferative disorders, such as essential thrombocythemia 2. Secondary ET is more common and occurs in reaction to infection, inflammation, or iron deficiency. Bleeding and thrombotic complications more frequently arise in primary ET cases 1, but have been reported with secondary ET in adults 3. Etiologies and complications associated with ET in children are less well-defined, as prior pediatric studies have been relatively small or restricted to specialized patient populations 4,5. We aimed to characterize ET in a large, single-center pediatric cohort.

Recent debate has focused on the significance of platelets generated in lung tissue. Here, we retrospectively analyzed platelet count changes in neonates requiring inhaled nitric oxide (iNO) pulmonary vasodilation therapy for pulmonary hypertension. There were no clinically significant changes in platelet count upon iNO initiation or during iNO therapy. Unexpectedly, platelet counts increased after iNO cessation. These findings argue against a clinically meaningful untapped pulmonary repository of megakaryocytes and platelets in this context, although acute platelet count increases might be expected after discontinuing iNO in some patients. Further work is needed to clarify the underlying etiology for these observations, and to better delineate the mechanisms for platelet count increases after recovery from lung injury.

SESSION TITLE: Airways 4 SESSION TYPE: Original Investigation Poster PRESENTED ON: Wednesday, October 26, 2016 at 01:30 PM - 02:30 PM PURPOSE: Health systems are faced with the daunting task of adapting and implementing a large array of transitional care interventions designed to ensure the coordination and continuity of care as patients transfer between different locations and levels of care. The purpose of this study, based within one medical center of a large integrated health system with a robust electronic medical record, was to describe lessons learned from a two-year, performance improvement project that aimed to optimize care for patients admitted with a COPD exacerbation. METHODS: We used a rapid Plan, Do, Study, Act cycle to stagger the implementation of a bundled intervention based on established COPD practice guidelines and published literature on successful care transition interventions. Individual components were prioritized for roll-out based on expected impact and ease of implementation: 1) COPD trained respiratory therapists serving as a transition coach/navigator, bridging inpatient and outpatient settings and responsible for assessing patients’ medical, psycho-social, and palliative care needs; 2) completion of a pulmonary consult during the hospitalization; 3) coupling interactive patient education videos and one-on-one teach backs during the hospitalization with continued reinforcement in the outpatient clinic; 4) arranging a follow up visit with the same inpatient pulmonologist within 7-14 days of discharge; and 5) enrolling patients into pulmonary rehabilitation. RESULTS: Facilitators to implementation of the bundle included high level executive and administrative support, physician champions who were actively involved in case finding and troubleshooting, respiratory therapists who practiced at the top of their license, data systems that allowed for real-time identification of patients who were admitted for a COPD exacerbation or pneumonia, and the project team’s vigilance in coordinating with other concurrent efforts within the health system to minimize redundancies and confusion for patients. Having adequate staffing remains a key barrier. CONCLUSIONS: We identified key facilitators and barriers to implementing a care transition bundle for COPD. CLINICAL IMPLICATIONS: Successful adaptation and implementation of evidence-based transition care interventions requires sustained alignment of institutional priorities and resources. DISCLOSURE: The following authors have nothing to disclose: Augusto Cam, Hector Garcia, Rene Patino, Luis Moreta-Sainz, Matthew Devine, Oscar Salazar, Huong Nguyen No Product/Research Disclosure Information