Resolution of refractory hypotension and anuria in a premature newborn with loss-of-function of ACE.

We present the investigation and management of a premature, hypotensive neonate born after a pregnancy complicated by anhydramnios to highlight the impact of early and informed management for rare kidney disease. Vasopressin was used to successfully treat refractory hypotension and anuria in the neonate born at 27 weeks of gestation. Next generation sequencing of a targeted panel of genes was then performed in the neonate and parents. Subsequently, two compound heterozygous deletions leading to frameshift mutations were identified in the angiotensin 1-converting enzyme gene ACE; exon 5:c.820_821delAG (p.Arg274Glyfs*117) and exon24: c.3521delG (p.Gly1174Alafs*12), consistent with a diagnosis of renal tubular dysgenesis. In light of the molecular diagnosis, identification, and treatment of associated low aldosterone level resulted in further improvement in renal function and only mild residual chronic renal failure is present at 14 months of age. Truncating alterations in ACE most often result in fetal demise during gestation or in the first days of life and typically as a result of the Potter sequence. The premature delivery, and serendipitous early treatment with vasopressin, and then later fludrocortisone, resulted in an optimal outcome in an otherwise lethal condition.

Bringing Enhanced Recovery After Surgery to the NICU: An Implementation Trial.

INTRODUCTION: Enhanced Recovery After Surgery (ERAS) guidelines are bundled evidence-informed recommendations implemented to improve quality and safety of perioperative care. This study aims to determine feasibility of NICU implementation of an ERAS Guideline for Intestinal Resection, describing clinical outcomes and adherence to recommendations following light-touch implementation. METHODS: Infants <28 days undergoing laparotomy for intestinal resection in a closed-NICU were prospectively enrolled. Exclusion criteria included prematurity (<32wks), instability, or major comorbidity. Clinical data reflecting 13 ERAS recommendations were collected through chart review. Descriptive statistics are presented as median [interquartile range]. Thirty-day post-discharge outcomes include NICU and hospital length of stay (LOS), ventilator days, surgical site infection (SSI), re-intubation, readmission, reoperation, and mortality. Adherence was calculated as the percentage of patients eligible for each recommendation whose care was adherent. RESULTS: Ten infant-parent dyads were enrolled (five females; GA 37 weeks [35, 38.8]; birthweight 2.97 kg [2.02, 3.69]). Surgical diagnoses included intestinal atresia/web (n = 6), anorectal malformation (n = 3), and segmental volvulus (n = 1). NICU LOS was 16 days [11, 21], hospital LOS 20 days [18, 30], and 2.5 ventilator days/patient [2, 3]. There was reduced opioid use, no SSIs, one re-intubation, three readmissions, three reoperations, and no mortalities. Adherence to ERAS recommendations ranged 0-100 % with a pooled adherence rate of 73 %. CONCLUSION: It is feasible to introduce ERAS to the NICU with acceptable overall adherence. Assessing adherence was challenging for some measures. There were promising early clinical findings including a reduction in opioid use. This implementation trial will inform development of an ERAS protocol for surgical NICUs. LEVEL OF EVIDENCE: IV (Cohort Study).

Endotracheal Tube Size Adjustments Within Seven Days of Neonatal Intubation.

BACKGROUND AND OBJECTIVES: Neonatal endotracheal tube (ETT) size recommendations are based on limited evidence. We sought to determine data-driven weight-based ETT sizes for infants undergoing tracheal intubation and to compare these with Neonatal Resuscitation Program (NRP) recommendations. METHODS: Retrospective multicenter cohort study from an international airway registry. We evaluated ETT size changes (downsizing to a smaller ETT during the procedure or upsizing to a larger ETT within 7 days) and risk of procedural adverse outcomes associated with first-attempt ETT size selection when stratifying the cohort into 200 g subgroups. RESULTS: Of 7293 intubations assessed, the initial ETT was downsized in 5.0% of encounters and upsized within 7 days in 1.5%. ETT downsizing was most common when NRP-recommended sizes were attempted in the following weight subgroups: 1000 to 1199 g with a 3.0 mm (12.6%) and 2000 to 2199 g with a 3.5 mm (17.1%). For infants in these 2 weight subgroups, selection of ETTs 0.5 mm smaller than NRP recommendations was independently associated with lower odds of adverse outcomes compared with NRP-recommended sizes. Among infants weighing 1000 to 1199 g: any tracheal intubation associated event, 20.8% with 2.5 mm versus 21.9% with 3.0 mm (adjusted OR [aOR] 0.62, 95% confidence interval [CI] 0.41-0.94); severe oxygen desaturation, 35.2% with 2.5 mm vs 52.9% with 3.0 mm (aOR 0.53, 95% CI 0.38-0.75). Among infants weighing 2000 to 2199 g: severe oxygen desaturation, 41% with 3.0 mm versus 56% with 3.5mm (aOR 0.55, 95% CI 0.34-0.89). CONCLUSIONS: For infants weighing 1000 to 1199 g and 2000 to 2199 g, the recommended ETT size was frequently downsized during the procedure, whereas 0.5 mm smaller ETT sizes were associated with fewer adverse events and were rarely upsized.

Factors that impact second attempt success for neonatal intubation following first attempt failure: a report from the National Emergency Airway Registry for Neonates.

OBJECTIVE: To determine the factors associated with second attempt success and the risk of adverse events following a failed first attempt at neonatal tracheal intubation. DESIGN: Retrospective analysis of prospectively collected data on intubations performed in the neonatal intensive care unit (NICU) and delivery room from the National Emergency Airway Registry for Neonates (NEAR4NEOS). SETTING: Eighteen academic NICUs in NEAR4NEOS. PATIENTS: Neonates requiring two or more attempts at intubation between October 2014 and December 2021. MAIN OUTCOME MEASURES: The primary outcome was successful intubation on the second attempt, with severe tracheal intubation-associated events (TIAEs) or severe desaturation (≥20% decline in oxygen saturation) being secondary outcomes. Multivariate regression examined the associations between these outcomes and patient characteristics and changes in intubation practice. RESULTS: 5805 of 13 126 (44%) encounters required two or more intubation attempts, with 3156 (54%) successful on the second attempt. Second attempt success was more likely with changes in any of the following: intubator (OR 1.80, 95% CI 1.56 to 2.07), stylet use (OR 1.65, 95% CI 1.36 to 2.01) or endotracheal tube (ETT) size (OR 2.11, 95% CI 1.74 to 2.56). Changes in stylet use were associated with a reduced chance of severe desaturation (OR 0.74, 95% CI 0.61 to 0.90), but changes in intubator, laryngoscope type or ETT size were not; no changes in intubator or equipment were associated with severe TIAEs. CONCLUSIONS: Successful neonatal intubation on a second attempt was more likely with a change in intubator, stylet use or ETT size.

Associations between family presence and neonatal intubation outcomes: a report from the National Emergency Airway Registry for Neonates: NEAR4NEOS.

OBJECTIVE: Describe the current practice of family presence during neonatal tracheal intubations (TIs) across neonatal intensive care units (NICUs) and examine the association with outcomes. DESIGN: Retrospective analysis of TIs performed in NICUs participating in the National Emergency Airway Registry for Neonates (NEAR4NEOS). SETTING: Thirteen academic NICUs. PATIENTS: Infants undergoing TI between October 2014 and December 2017. MAIN OUTCOME MEASURES: Association of family presence with TI processes and outcomes including first attempt success (primary outcome), success within two attempts, adverse TI-associated events (TIAEs) and severe oxygen desaturation ≥20% from baseline. RESULTS: Of the 2570 TIs, 242 (9.4%) had family presence, which varied by site (median 3.6%, range 0%-33%; p<0.01). Family member was more often present for older infants and those with chronic respiratory failure. Fewer TIs were performed by residents when family was present (FP 10% vs no FP 18%, p=0.041). Among TIs with family presence versus without family presence, the first attempt success rate was 55% vs 49% (p=0.062), success within two attempts was 74% vs 66% (p=0.014), adverse TIAEs were 18% vs 20% (p=0.62) and severe oxygen desaturation was 49% vs 52%, (p=0.40). In multivariate analyses, there was no independent association between family presence and intubation success, adverse TIAEs or severe oxygen desaturation. CONCLUSION: Family are present in less than 10% of TIs, with variation across NICUs. Even after controlling for important patient, provider and site factors, there were no significant associations between family presence and intubation success, adverse TIAEs or severe oxygen desaturation.

Cardiac arrest with pulseless electrical activity rhythm in newborn infants: a case series.

The 2015 neonatal resuscitation guidelines added ECG to assess an infant's heart rate when determining the need for resuscitation at birth. However, a recent case report raised concerns about this technique in the delivery room. We report four cases of pulseless electrical activity during neonatal cardiopulmonary resuscitation in levels II-III neonatal intensive care units in Canada (Edmonton [n=3] and Winnipeg [n=1]).Healthcare providers should be aware that pulseless electrical activity can occur in newborn infants during cardiopulmonary resuscitation. We propose an adapted neonatal resuscitation algorithm to include pulseless electrical activity. Furthermore, in compromised newborns, heart rate should be assessed using a combination of methods/techniques to ensure accurate heart rate assessment. When ECG displays a heart rate but the infant is unresponsive, pulseless electrical activity should be suspected and chest compression should be started.

Infective endocarditis caused by Streptococcus agalactiae in a native aortic valve, complicated by meningitis and cerebral embolism.

We present a case of infective endocarditis associated with community-acquired Streptococcus agalactiae in an immune competent patient. The endocarditis affected the native aortic valve with perforation of the coronary cusp and was complicated by a cerebral embolism. The use of intravenous ampicillin produced a satisfactory clinical and echocardiographic recovery despite not receiving a valve replacement. In addition to reporting an extremely rare case, this paper confirms that the opportune identification of endocarditis caused by S. agalactiae and the selection of appropriate antibiotics can prevent the necessity of cardiac surgery, usually required in such cases.