Management of fetal head and neck masses: Evaluation of prenatal factors associated with airway obstruction and decision for definitive airway and ex-utero intrapartum treatment at birth.

OBJECTIVE: Fetal head and neck masses can result in critical airway obstruction. Our study aimed to evaluate prenatal factors associated with the decision for a definitive airway, including ex-utero intrapartum treatment (EXIT), at birth among at-risk fetuses. METHODS: A single-institution retrospective review evaluated all fetal head and neck masses prenatally diagnosed from 2005 to 2023. The primary outcome was the decision for a definitive airway at birth, including intubation, tracheostomy, or EXIT. RESULTS: Thirty four patients were included, with 23 deliveries occurring at our institution. 8/23 (35%) patients received a definitive airway at birth, six underwent an EXIT procedure, and two required intubation only. Patients who received a definitive airway had higher rates of polyhydramnios (50% vs. 7%, p = 0.03), tracheal narrowing on ultrasound (US) (50% vs. 0%, p = 0.01), tracheal displacement on US (63% vs. 0%, p < 0.01), abnormal fetal breathing on US (50% vs. 0%, p = 0.01), tracheal narrowing or displacement on magnetic resonance imaging (MRI) (75% vs. 7%, p < 0.01), and larger mass maximum diameter (7.9 vs. 4.3 cm, p = 0.02). In our series, 100% of patients with polyhydramnios, tracheal narrowing or displacement on either US or MRI, and abnormal fetal breathing on US received a definitive airway at birth. CONCLUSION: Prenatal findings of tracheal narrowing or displacement, polyhydramnios, and abnormal fetal breathing are strongly associated with the decision for a definitive airway at birth and warrant mobilization of appropriate resources.

Thoracoscopic Excision of Mediastinal Bronchogenic Cysts in Children: A Case Series.

Background: Bronchogenic cysts result from a congenital anomalous budding of the tracheobronchial tree. Resection is usually recommended to avoid complications. Mediastinal bronchogenic cysts present a unique challenge due to their proximity to vital structures. The purpose of this study is to review our experience with mediastinal bronchogenic cysts. Methods: A single-institution retrospective review evaluated all mediastinal bronchogenic cyst excisions between January 2012 and November 2022. Patient demographics were assessed, including age at diagnosis, presenting symptoms, imaging workup, and cyst characteristics. Operative approach, complications, and surgical pathology were reported. Results: Five patients were identified. Age at diagnosis ranged from 18 to 27 months. No patient was diagnosed prenatally. All patients had symptoms at the time of diagnosis, including cough, wheezing, and respiratory distress. Three cysts were paratracheal, and two were paraesophageal. Age at surgery ranged from 26 to 30 months. All bronchogenic cysts were successfully resected thoracoscopically. Individual technical challenges included narrowing of the mainstem bronchus preventing lung isolation, significant mediastinal inflammation, the necessity for cyst evacuation to delineate the extent of the cyst, adherence of cyst wall to bronchus or trachea requiring cold dissection, and a stalk of tissue with an intimate connection to the carina that was amputated. No intraoperative or postoperative complication occurred. Surgical pathology was consistent with a bronchogenic cyst in all cases. Median length of hospital stay was two days. Conclusion: Thoracoscopy is a safe and effective procedure for mediastinal bronchogenic cyst excision in children. Certain technical maneuvers are highlighted, which may facilitate resection.

Delphi Process for Validation of Fluid Treatment Algorithm for Critically Ill Pediatric Trauma Patients.

INTRODUCTION: While intravenous fluid therapy is essential to re-establishing volume status in children who have experienced trauma, aggressive resuscitation can lead to various complications. There remains a lack of consensus on whether pediatric trauma patients will benefit from a liberal or restrictive crystalloid resuscitation approach and how to optimally identify and transition between fluid phases. METHODS: A panel was comprised of physicians with expertise in pediatric trauma, critical care, and emergency medicine. A three-round Delphi process was conducted via an online survey, with each round being followed by a live video conference. Experts agreed or disagreed with each aspect of the proposed fluid management algorithm on a five-level Likert scale. The group opinion level defined an algorithm parameter's acceptance or rejection with greater than 75% agreement resulting in acceptance and greater than 50% disagreement resulting in rejection. The remaining were discussed and re-presented in the next round. RESULTS: Fourteen experts from five Level 1 pediatric trauma centers representing three subspecialties were included. Responses were received from 13/14 participants (93%). In round 1, 64% of the parameters were accepted, while the remaining 36% were discussed and re-presented. In round 2, 90% of the parameters were accepted. Following round 3, there was 100% acceptance by all the experts on the revised and final version of the algorithm. CONCLUSIONS: We present a validated algorithm for intavenous fluid management in pediatric trauma patients that focuses on the de-escalation of fluids. Focusing on this time point of fluid therapy will help minimize iatrogenic complications of crystalloid fluids within this patient population.

Pilot randomized controlled trial of restricted versus liberal crystalloid fluid management in pediatric post-operative and trauma patients.

BACKGROUND: Intravenous (IV) fluid therapy is essential in the treatment of critically ill pediatric surgery and trauma patients. Recent studies have suggested that aggressive fluids may be detrimental to patients. Prospective studies are needed to compare liberal to restricted fluid management in these patients. The primary objective of this pilot trial is to test study feasibility-recruitment and adherence to the study treatment algorithm. METHODS: We conducted a two-part pilot randomized controlled trial (RCT) comparing liberal to restricted crystalloid fluid management in 50 pediatric post-operative (1-18 years) and trauma (1-15 years) patients admitted to our pediatric intensive care unit (PICU). Patients were randomized to a high (liberal) volume or low (restricted) volume algorithm using unblinded, blocked randomization. A revised treatment algorithm was used after the 29th patient for the second part of the RCT. The goal of the trial was to determine the feasibility of conducting an RCT at a single site for recruitment and retention. We also collected data on the safety of study interventions and clinical outcomes, including pulmonary, infectious, renal, post-operative, and length of stay outcomes. RESULTS: Fifty patients were randomized to either liberal (n = 26) or restricted (n = 24) fluid management strategy. After data was obtained on 29 patients, a first study analysis was performed. The volume of fluid administered and triggers for intervention were adapted to optimize the treatment effect and clarity of outcomes. Updated and refined fluid management algorithms were created. These were used for the second part of the RCT on patients 30-50. During this second study period, 54% (21/39, 95% CI 37-70%) of patients approached were enrolled in the study. Of the patients enrolled, 71% (15/21, 95% CI 48-89%) completed the study. This met our a priori recruitment and retention criteria for success. A data safety monitoring committee concluded that no adverse events were related to study interventions. Although the study was not powered to detect differences in outcomes, after the algorithm was revised, we observed a non-significant trend towards improved pulmonary outcomes in patients on the restricted arm, including decreased need for and time on oxygen support and decreased need for mechanical ventilation. CONCLUSION: We demonstrated the feasibility and safety of conducting a single-site RCT comparing liberal to restricted crystalloid fluid management in critically ill pediatric post-operative and trauma patients. We observed trends in improved pulmonary outcomes in patients undergoing restricted fluid management. A definitive multicenter RCT comparing fluid management strategies in these patients is warranted. TRIAL REGISTRATION: ClinicalTrials.gov, NCT04201704 . Registered 17 December 2019-retrospectively registered.

Neonate with congenital pulmonary airway malformation concurrent with enteric duplication cyst: a case report of a rare anomaly.

A congenital pulmonary airway malformation (CPAM) occurring concurrently with an enteric duplication cyst is a rare anomaly. Definitive management for both abnormalities is usually surgical resection. We present the uncommon case of a neonate with a CPAM and ileal duplication cyst, including pre-natal and post-natal workup. The patient was brought to the operating room for laparoscopic duplication cyst excision at 3 months of age. The patient returned to the operating room for a thoracoscopic right lower lobectomy at five months of age. This case presents a rare congenital anomaly with the concurrent presentation of a CPAM and enteric duplication cyst, with both being successfully excised minimally invasively.

Congenital Heart Disease with Congenital Diaphragmatic Hernia: Surgical Decision Making and Outcomes.

OBJECTIVE: To describe the types of congenital heart disease (CHD) in a congenital diaphragmatic hernia (CDH) cohort in a large volume center and evaluate surgical decision making and outcomes based on complexity of CHD and associated conditions. STUDY DESIGN: A retrospective review of patients with CHD and CDH diagnosed by echocardiogram between 01/01/2005 and 07/31/2021. The cohort was divided into 2 groups based on survival at discharge. RESULTS: Clinically important CHD was diagnosed in 19% (62/326) of CDH patients. There was 90% (18/20) survival in children undergoing surgery for both CHD and CDH as neonates, and 87.5 (22/24) in those undergoing repair initially for CDH alone. A genetic anomaly identified on clinical testing was noted in 16% with no significant association with survival. A higher frequency of other organ system anomalies was noted in nonsurvivors compared with survivors. Nonsurvivors were more likely to have unrepaired CDH (69% vs 0%, P < .001) and unrepaired CHD (88% vs 54%, P < .05), reflecting a decision not to offer surgery. CONCLUSIONS: Survival was excellent in patients who underwent repair of both CHD and CDH. Patients with univentricular physiology have poor survival and this finding should be incorporated into pre and postnatal counseling about eligibility for surgery. In contrast, patients with other complex lesions including transposition of the great arteries have excellent outcomes and survival at 5 years follow-up at a large pediatric and cardiothoracic surgical center.

Prenatal ultrasound-and MRI-based imaging predictors of respiratory symptoms at birth for congenital lung malformations.

BACKGROUND: Congenital lung malformations (CLM) are rare developmental anomalies of the fetal lung with a minority of patients exhibiting symptoms around the time of birth. Although ultrasound remains the gold standard, fetal MRI has recently been incorporated as an adjunct imaging modality in the workup and prenatal counseling of patients with CLM as it is thought to more accurately delineate lesion boundaries and diagnose lesion type. We evaluate what prenatal variables correlate with postnatal respiratory symptoms. METHODS: We performed a retrospective review of patients with prenatal diagnosis of CLM treated at our institution between 2006-2020. Fetal ultrasound and magnetic resonance imaging (MRI) parameters including maximal congenital pulmonary airway malformation volume ratio (CVR), absolute cyst volume, and observed to expected normal fetal lung volume (O/E NFLV) were correlated with outcomes including postnatal respiratory symptoms, need for supplementary oxygen or mechanical ventilation, delay in tolerating full feeds, resection in the neonatal period. RESULTS: Our study included 111 patients, all of whom underwent fetal ultrasound with 64 patients additionally undergoing fetal MRI. Postnatal respiratory symptoms were noted in 22.5% of patients, 19.8% required supplemental oxygen, 2.7% mechanical ventilation and two patients requiring urgent resection. Ultrasound parameters including absolute cyst volume and maximal CVR correlated with need for mechanical ventilation (p=0.034 and p=0.024, respectively) and for urgent resection (p=0.018 and p=0.023, respectively) and had a marginal association with postnatal respiratory symptoms (p=0.050 and p=0.052). Absolute cyst volume became associated with postnatal respiratory symptoms (p=0.017) after multivariable analysis controlling for maternal steroid administration and gestational age. O/E NFLV did not correlate with perinatal outcomes. CONCLUSION: We have found that ultrasound-based measurements correlate with postnatal respiratory symptoms, while MRI derived O/E NFLV does not. Further studies are needed to elucidate the role of MRI in the prenatal workup of congenital lung malformations. TYPE OF STUDY: Study of Diagnostic Test. LEVEL OF EVIDENCE: Level I.

Hybrid lung lesions in children with segmental infantile hemangiomas, a new association?

Infantile hemangiomas (IHs) are the most common tumors of infancy and, in rare instances, can present in the setting of congenital structural anomalies or as part of syndromic disorders. In this study, we present three cases of children with segmental IHs born with concurrent pulmonary anomalies: congenital pulmonary airway malformations and bronchopulmonary sequestration. To date, no known association between these entities and hemangiomas has been described.

Postpyloric Feeding Access in Infants and Children: A State of the Art Review.

Achieving postpyloric feeding access is a clinical challenge faced by the pediatric gastroenterologist in everyday practice. Currently, there is limited literature published on the topic. This article provides a practical summary of the literature on the different methods utilized to achieve postpyloric feeding access including bedside, fluoroscopic, endoscopic and surgical options. Indications and complications of these methods are discussed as well as a general approach to infants and children that require intestinal feeding.

Association of Economic Recession and Social Distancing With Pediatric Non-accidental Trauma During COVID-19.

INTRODUCTION: There has been concern that the incidence of non-accidental trauma (NAT) cases in children would rise during the COVID-19 pandemic due to the combination of social isolation and economic depression. Our goal was to evaluate NAT incidence and severity during the pandemic across multiple US cities. METHODS: Multi-institutional, retrospective cohort study comparing NAT rates in children <18 y old during the COVID-19 pandemic (March-August 2020) with a recent historical data (January 2015-February 2020) and during a previous economic recession (January 2007-December 2011) at level 1 Pediatric Trauma Centers. Comparisons were made in local and national macroeconomic indicators. RESULTS: Overall rates of NAT during March-August 2020 did not increase compared to historical data (P = 0.8). Severity of injuries did not increase during the pandemic as measured by Glasgow Coma Scale (GCS) (P = 0.97) or mortality (P = 0.7), but Injury Severity Score (ISS) slightly decreased (P = 0.018). Racial differences between time periods were seen, with increased proportions of NAT occurring in African-Americans during the pandemic (P < 0.001). NAT rates over time had low correlation (r = 0.32) with historical averages, suggesting a difference from previous years. Older children (≥3 y) had increased NAT rates during the pandemic. Overall NAT rates had low inverse correlation with unemployment (r = -0.37) and moderate inverse correlation with the stock market (r = -0.6). Significant variation between sites was observed. CONCLUSIONS: Overall NAT rates in children did not increase during the COVID-19 pandemic, but rates were highly variable by site and increases were seen in African-Americans and older children. Further studies are warranted to explore local influences on NAT rates.

Extracorporeal Membrane Oxygenation (ECMO) and its complications in newborns with congenital diaphragmatic hernia.

BACKGROUND: Extracorporeal Membrane Oxygenation (ECMO) is offered to patients with congenital diaphragmatic hernia (CDH) who are in severe respiratory and cardiac failure. We aim to describe the types of complications among these patients and their impact on survival. METHODS: A single-center, retrospective review of CDH patients cannulated onto ECMO between January 2005 and November 2020 was conducted. ECMO complications, as categorized by the Extracorporeal Life Support Organization (ELSO), were correlated with survival status. Descriptive statistics were used to compare observed complications between survivors and non-survivors. RESULTS: In our cohort of CDH neonates, 21% (54/258) were supported with ECMO, of whom, 61% (33/54) survived. Survivors and non-survivors were similar in baseline characteristics except for birthweight z-score (p = 0.043). Seventy percent of CDH neonates experienced complications during their ECMO run, with the most common categories being metabolic (48.1%) and mechanical (38.9%), followed by hemorrhage (22.2%), neurological (18.5%), renal (11.1%), pulmonary (7.4%), and cardiovascular (7.4%). The median number of complications per patient was higher in the non-survivor group  (2 (IQR: 1-4) vs 1 (IQR: 0-2), p = 0.043). In addition, mechanical (57.1% vs 27.3%, p = 0.045) and renal (28.6% vs 0%, p = 0.002) complications were more common among non-survivors compared to survivors. CONCLUSION: Complications occur frequently among ECMO-treated newborns with CDH, some of which have serious long-term consequences. Survivors had higher birth weight z-scores, shorter ECMO runs, and fewer complications per patient. Mechanical and renal complications were independently associated with mortality, emphasizing the utility of more focused strategies to target fluid balance and renal protection and to prevent circuit and cannula complications.

Long-term outcomes of congenital diaphragmatic hernia: A single institution experience.

BACKGROUND/PURPOSE: As survival rates for patients with congenital diaphragmatic hernia (CDH) increase, long-term sequelae become increasingly prevalent. We present the outcomes of patients who underwent CDH repair at our institution and discuss standardization of follow-up care in our long-term multidisciplinary follow-up clinic. METHODS: A retrospective review of patients followed in multidisciplinary clinic after CDH repair at our institution from January 1, 2005 to December 1, 2020. RESULTS: A total of 193 patients met inclusion criteria, 73 females (37.8%) and 120 males (62.2%). Left-sided defects were most common (75.7%), followed by right-sided defects (20.7%). Median age at repair was 4 days (IQR 3-6) and 59.6% of all defects required patch repair. Median length of stay was 29 days (IQR 16.8-50.0). Median length of follow up was 49 months (IQR 17.8-95.3) with 25 patients followed for more than 12 years. Long-term outcomes included gastroesophageal reflux disease (42.0%), diaphragmatic hernia recurrence (10.9%), asthma (23.6%), neurodevelopmental delay (28.6%), attention deficit hyperactivity disorder (7.3%), autism (1.6%), chest wall deformity (15.5%), scoliosis (11.4%), and inguinal hernia (6.7%). CONCLUSION: As survival of patients with CDH improves, long-term care must be continuously studied and fine-tuned to ensure appropriate surveillance and optimization of long-term outcomes.

High-Frequency Positive Pressure Ventilation as Primary Rescue Strategy for Patients with Congenital Diaphragmatic Hernia: A Comparison to High-Frequency Oscillatory Ventilation.

OBJECTIVE: The aim of this article was to evaluate high-frequency positive pressure ventilation (HFPPV) compared with high-frequency oscillatory ventilation (HFOV) as a rescue ventilation strategy for patients with congenital diaphragmatic hernia (CDH). HFPPV is a pressure-controlled conventional ventilation method utilizing high respiratory rate and low positive end-expiratory pressure. STUDY DESIGN: Seventy-seven patients diagnosed with CDH from January 2005 to September 2019 who were treated with stepwise progression from HFPPV to HFOV versus only HFOV were included. Fisher's exact test and the Kruskal-Wallis test were used to compare outcomes. RESULTS: Patients treated with HFPPV + HFOV had higher survival to discharge (80 vs. 50%, p = 0.007) and to surgical intervention (95.6 vs. 68.8%, p = 0.003), with average age at repair 2 days earlier (p = 0.004). Need for extracorporeal membrane oxygenation (p = 0.490), inhaled nitric oxide (p = 0.585), supplemental oxygen (p = 0.341), and pulmonary hypertension medications (p = 0.381) were similar. CONCLUSION: In CDH patients who fail respiratory support with conventional ventilation, HFPPV may be used as an intermediary mode of rescue ventilation prior to HFOV without adverse effects. KEY POINTS: · HFPPV may be used as an intermediary mode of rescue ventilation prior to HFOV without adverse effect.. · HFPPV is more widely available and can mitigate the limitations faced when using HFOV.. · HFPPV allows for intra- or interhospital transfer of neonates with CDH..

Rare and de novo variants in 827 congenital diaphragmatic hernia probands implicate LONP1 as candidate risk gene.

Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly that is often accompanied by other anomalies. Although the role of genetics in the pathogenesis of CDH has been established, only a small number of disease-associated genes have been identified. To further investigate the genetics of CDH, we analyzed de novo coding variants in 827 proband-parent trios and confirmed an overall significant enrichment of damaging de novo variants, especially in constrained genes. We identified LONP1 (lon peptidase 1, mitochondrial) and ALYREF (Aly/REF export factor) as candidate CDH-associated genes on the basis of de novo variants at a false discovery rate below 0.05. We also performed ultra-rare variant association analyses in 748 affected individuals and 11,220 ancestry-matched population control individuals and identified LONP1 as a risk gene contributing to CDH through both de novo and ultra-rare inherited largely heterozygous variants clustered in the core of the domains and segregating with CDH in affected familial individuals. Approximately 3% of our CDH cohort who are heterozygous with ultra-rare predicted damaging variants in LONP1 have a range of clinical phenotypes, including other anomalies in some individuals and higher mortality and requirement for extracorporeal membrane oxygenation. Mice with lung epithelium-specific deletion of Lonp1 die immediately after birth, most likely because of the observed severe reduction of lung growth, a known contributor to the high mortality in humans. Our findings of both de novo and inherited rare variants in the same gene may have implications in the design and analysis for other genetic studies of congenital anomalies.

Primary gastrojejunostomy tube placement using laparoscopy with endoscopic assistance: A novel technique.

BACKGROUND: Gastrojejunostomy (GJ) tubes are commonly used to provide postpyloric enteral nutrition in pediatric patients who cannot tolerate gastric feeds. Most techniques depend on a preexisting gastrostomy tube (GT) site to convert to a gastrojejunostomy. Several minimally invasive techniques have been described; however, their risk profile varies widely. DESCRIPTION OF THE OPERATIVE TECHNIQUE: We present a technique for primary laparoscopic GJ tube placement that minimizes the risk of hollow viscus injury and the use of fluoroscopy through endoscopic assistance. RESULTS: Eleven GJ tubes were placed using this technique in patients ranging from 5 months to 17 years of age and weighing 6.3 to 46.0 kg. Endoscopy through the gastrostomy site allowed direct visualization of wire and tube placement. There were no intraoperative or postoperative complications within 30 days of operation. Use of fluoroscopy was limited with minimal total radiation exposure. CONCLUSION: The described technique of laparoscopic primary gastrojejunostomy tube placement with endoscopic assistance was associated with a low complication rate and minimal use of fluoroscopy. LEVEL OF EVIDENCE: IV.

Delayed presentation and sub-optimal outcomes of pediatric patients with acute appendicitis during the COVID-19 pandemic.

OBJECTIVE: Early presentation and prompt diagnosis of acute appendicitis are necessary to prevent progression of disease leading to complicated appendicitis. We hypothesize that patients had a delayed presentation of acute appendicitis during the COVID-19 pandemic, which affected severity of disease on presentation and outcomes. PATIENTS AND METHODS: We conducted a retrospective review of all patients who were treated for acute appendicitis at Morgan Stanley Children's Hospital (MSCH) between March 1, 2020 and May 31, 2020 when the COVID-19 pandemic was at its peak in New York City (NYC). For comparison, we reviewed patients treated from March 1, 2019 to May 31, 2019, prior to the pandemic. Demographics and baseline patient characteristics were analyzed for potential confounding variables. Outcomes were collected and grouped into those quantifying severity of illness on presentation to our ED, type of treatment, and associated post-treatment outcomes. Fisher's Exact Test and Kruskal-Wallis Test were used for univariate analysis while cox regression with calculation of hazard ratios was used for multivariate analysis. RESULTS: A total of 89 patients were included in this study, 41 patients were treated for appendicitis from March 1 to May 31 of 2019 (non-pandemic) and 48 were treated during the same time period in 2020 (pandemic). Duration of symptoms prior to presentation to the ED was significantly longer in patients treated in 2020, with a median of 2 days compared to 1 day (p = 0.003). Additionally, these patients were more likely to present with reported fever (52.1% vs 24.4%, p = 0.009) and had a higher heart rate on presentation with a median of 101 beats per minute (bpm) compared to 91 bpm (p = 0.040). Findings of complicated appendicitis on radiographic imaging including suspicion of perforation (41.7% vs 9.8%, p < 0.001) and intra-abdominal abscess (27.1% vs 7.3%, p = 0.025) were higher in patients presenting in 2020. Patients treated during the pandemic had higher rates of non-operative treatment (25.0% vs 7.3%, p = 0.044) requiring increased antibiotic use and image-guided percutaneous drain placement. They also had longer hospital length of stay by a median of 1 day (p = 0.001) and longer duration until symptom resolution by a median of 1 day (p = 0.004). Type of treatment was not a predictor of LOS (HR = 0.565, 95% CI = 0.357-0.894, p = 0.015) or duration until symptom resolution (HR = 0.630, 95% CI = 0.405-0.979, p = 0.040). CONCLUSION: Patients treated for acute appendicitis at our children's hospital during the peak of the COVID-19 pandemic presented with more severe disease and experienced suboptimal outcomes compared to those who presented during the same time period in 2019. LEVEL OF EVIDENCE: III.

Neonates With Complex Cardiac Malformation and Congenital Diaphragmatic Hernia Born to SARS-CoV-2 Positive Women-A Single Center Experience.

BACKGROUND: Our understanding of the impact of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) on pregnancies and perinatal outcomes is limited. The clinical course of neonates born to women who acquired coronavirus disease 2019 (COVID-19) during their pregnancy has been previously described. However, the course of neonates born with complex congenital malformations during the COVID-19 pandemic is not known. METHODS: We report a case series of seven neonates with congenital heart and lung malformations born to women who tested positive for SARS-CoV-2 during their pregnancy at a single academic medical center in New York City. RESULTS: Six infants had congenital heart disease and one was diagnosed with congenital diaphragmatic hernia. In all seven infants, the clinical course was as expected for the congenital lesion. None of the seven exhibited symptoms generally associated with COVID-19. None of the infants in our case series tested positive by nasopharyngeal test for SARS-CoV-2 at 24 hours of life and at multiple points during their hospital course. CONCLUSIONS: In this case series, maternal infection with SARS-CoV-2 during pregnancy did not result in adverse outcomes in neonates with complex heart or lung malformations. Neither vertical nor horizontal transmission of SARS-CoV-2 was noted.

Strategies in liver Trauma.

The management of pediatric liver trauma has evolved significantly over the last few decades. While surgical intervention was frequently and mostly unsuccessfully practiced during the first half of the last century, the 1960s were witness to the birth and gradual acceptance of non-operative management of these injuries. In 2000, the American Pediatric Surgical Association (APSA) Trauma Committee disseminated evidenced-based guidelines to help guide the non-operative management of pediatric blunt solid organ injury. The guidelines significantly contributed to conformity in the management of these patients. Since then, a number of well-designed studies have questioned the strict categorization of these injuries and have led to a renewed reliance on clinical signs of the patient's hemodynamic status. In 2019, APSA introduced an updated set of guidelines emphasizing the use of physiologic status rather than radiologic grade as a driver of clinical decision making for these injuries. This review will focus on liver injuries, in particular blunt injury, as this mechanism is by far the most commonly seen in children. Procedures required when non-operative management fails will be detailed, including surgery, angioembolization, and less commonly employed interventions. Finally, the updated inpatient and post-discharge aspects of care will be reviewed, including hemoglobin monitoring, bedrest, length of hospital stay, and activity restriction.

Likely damaging de novo variants in congenital diaphragmatic hernia patients are associated with worse clinical outcomes.

PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with significant mortality and long-term morbidity in some but not all individuals. We hypothesize monogenic factors that cause CDH are likely to have pleiotropic effects and be associated with worse clinical outcomes. METHODS: We enrolled and prospectively followed 647 newborns with CDH and performed genomic sequencing on 462 trios to identify de novo variants. We grouped cases into those with and without likely damaging (LD) variants and systematically assessed CDH clinical outcomes between the genetic groups. RESULTS: Complex cases with additional congenital anomalies had higher mortality than isolated cases (P = 8 × 10-6). Isolated cases with LD variants had similar mortality to complex cases and much higher mortality than isolated cases without LD (P = 3 × 10-3). The trend was similar with pulmonary hypertension at 1 month. Cases with LD variants had an estimated 12-17 points lower scores on neurodevelopmental assessments at 2 years compared with cases without LD variants, and this difference is similar in isolated and complex cases. CONCLUSION: We found that the LD genetic variants are associated with higher mortality, worse pulmonary hypertension, and worse neurodevelopment outcomes compared with non-LD variants. Our results have important implications for prognosis, potential intervention and long-term follow up for children with CDH.