Management advances for congenital diaphragmatic hernia: integrating prenatal and postnatal perspectives.

In congenital diaphragmatic hernia (CDH), abdominal organs are displaced into the chest, compress the lungs, and cause mediastinal shift. This contributes to development of pulmonary hypoplasia and hypertension, which is the primary determinant of morbidity and mortality for affected newborns. The severity is determined using prenatal imaging as early as the first trimester and is related to the laterality of the defect, extent of lung compression, and degree of liver herniation. Comprehensive evaluation of fetal CDH includes imaging-based severity assessment, severity assessment, and evaluation for structural or genetic abnormalities to differentiate isolated from complex cases. Prenatal management involves multispecialty counseling, consideration for fetal therapy with fetoscopic endoluminal tracheal occlusion (FETO) for severe cases, monitoring and intervention for associated polyhydramnios or signs of preterm labor if indicated, administration of antenatal corticosteroids in the appropriate setting, and planned delivery to optimize the fetal condition at birth. Integrated programs that provide a smooth transition from prenatal to postnatal care produce better outcomes. Neonatal care involves gentle ventilation to avoid hyperinflation and must account for transitional physiology to avoid exacerbating cardiac dysfunction and decompensation. Infants who have undergone and responded to FETO have greater pulmonary capacity than expected, but cardiac dysfunction seems unaffected. In about 25-30% of CDH neonates extracorporeal life support is utilized, and this provides a survival benefit for patients with the highest predicted mortality, including those who underwent FETO. Surgical repair after initial medical management for the first 24-48 hours of life is preferred since later repair is associated with delayed oral feeding, increased need for tube feeds, and increased post-repair ventilation requirement and supplemental oxygen at discharge. With overall survival rates >70%, contemporary care involves management of chronic morbidities in the context of a multidisciplinary clinic setting.

Neurodevelopmental Outcomes in Children After Fetoscopic Endoluminal Tracheal Occlusion for Severe Congenital Diaphragmatic Hernia: Results From a Multidisciplinary Clinic.

BACKGROUND: We compared early neurodevelopmental morbidity in young children with severe CDH who underwent FETO to those without fetal therapy. METHODS: We conducted a prospective study of severe CDH patients undergoing FETO (n = 18) at a single North American center from 2015 to 2021 (NCT02710968). Outpatient survivors (n = 12) were evaluated by a multidisciplinary team and compared to expectantly managed CDH patients. Neurodevelopmental outcomes were assessed using the Capute Scales [Clinical Linguistic and Auditory Milestone Scales (CLAMS) and Cognitive Adaptive Test (CAT)], with a developmental quotient (DQ) < 85 indicative of at-risk for delay. RESULTS: At one year, 58% (n = 7) of FETO patients underwent evaluation, with notable concern for language delay (CLAMS median DQ, 80.1 [interquartile range, 67.6-86.7]). FETO scores improved by 24-months, whereas high severity/non-FETO scores declined [CLAMS median DQ (Difference in DQ), 92.3 (+12.2) vs. 77.1 (-13.4), respectively; p = 0.049]. On the initial CAT, FETO patients had concern for visual motor and problem-solving delays, with a median DQ of 81.3 (62.1-89.4). At 24-months, FETO patients had improving scores [Median CAT DQ, 90.8 (+9.5)], whereas high severity/non-FETO [87.5 (-3.0), p = 0.28] had declining scores. CONCLUSION: These initial data suggest that FETO is associated with favorable neurodevelopmental outcomes at 24-months compared to severe CDH under expectant management. LEVEL OF EVIDENCE: III.

Patient Characteristics, Treatment, and Survival in Pleuropulmonary Blastoma: An Analysis from the National Cancer Database.

Pleuropulmonary blastoma (PPB) is a rare childhood tumor originating from the lung or pleura, typically treated with surgery, chemotherapy (CTx), and/or radiation therapy (RTx). This study aimed to assess patient and tumor features, treatment methods, and survival rates in PPB. We retrospectively analyzed PPB patients under 18 from 2004 to 2019, using the National Cancer Database (NCDB). We assessed 5-year overall survival (OS) rates based on disease presentation and treatment regimens, using Kaplan-Meier curves and Cox proportional regression. Among 144 cases identified, 45.9% were female, with a median age of 2 years (interquartile range 1-3). In all, 62.5% of tumors originated from the lung, and 10.4% from the pleura. Moreover, 6.9% were bilateral, and the median tumor size was 8.9 cm, with 4.2% presenting with metastases. The 5-year OS rate was 79.6%, with no significant change over time (2004-2009, 77.6%; 2010-2014, 90.8%; 2015-2019, OS 75.3%; p = 0.08). In all, 5.6% received CTx alone, 31.3% surgery alone, 45.8% surgery/CTx, and 17.4% surgery/CTx/RTx. The OS rates were comparable between the surgery/CTx/RTx (80.0%) and surgery/CTx (76.5%) groups (adjusted Hazard Ratio, 1.72; 95% CI, 0.57-5.19; p = 0.34). Therefore, due to the unchanged survival rates over time, further prospective multicenter studies are needed to determine the optimal multimodal treatment regimens for this rare pediatric tumor.

Operative Volumes and Postoperative Outcomes After Elective Resection of Congenital Lung Malformations in Infants.

OBJECTIVE: To determine whether hospital- and surgeon-level operative volumes are associated with differences in postoperative outcomes among infants undergoing elective lung surgery for a congenital lung malformation (CLM). SUMMARY BACKGROUND DATA: Infant lung surgery is a relatively uncommon procedure performed by pediatric surgeons nationwide. The relationship between surgical volume and postoperative outcomes remains unknown. METHODS: A retrospective cohort study of asymptomatic infants who underwent elective lung resection of a CLM was conducted using the Pediatric Health Information System database (2016-2020). Multivariable linear and poisson regressions were performed based on annual lung resection tertiles. RESULTS: There were 1420 infants managed by 48 hospitals and 309 primary surgeons. Institutions that performed seven or fewer CLM resections per year (56%) were associated with significantly higher postoperative complication rates compared to medium- and high-volume hospitals (low: 134 [34%], medium: 110 [21%], high: 144 [29%]; P<0.001). Surgeons who performed one or fewer CLM resections per year (82%) were associated with significantly higher complication rates compared to medium- and high-volume surgeons (low: 171 [31%], medium: 75 [26%], high: 119 [24%]; P=0.02). Multivariable analyses confirmed that low-volume hospitals were associated with higher complications (OR 1.81, CI 1.38-2.37; P<0.001), and low-volume surgeons had an increased risk of complications (overall: OR 1.37, CI 1.01-1.84; P=0.04). CONCLUSIONS: In this cohort study of infants undergoing elective lung resection for a CLM, lower volume providers were associated with higher postoperative patient morbidity. These findings represent an opportunity to inform quality improvement initiatives on pediatric lung resection and the debate on surgical subspecialization for this unique patient population.

Management of long-gap esophageal atresia.

A uniquely challenging subset of infants diagnosed with esophageal atresia (EA) are those born with long-gap EA (LGEA). The common unifying feature in infants with LGEA is that the proximal and distal segments of the esophagus are too far apart to enable primary anastomosis via a single operation in the newborn period. Although any type of EA can technically result in a long gap, the Gross type A variant occurs in 8% of all EA cases and is most commonly associated with LGEA. In this review, we provide an evidence-based approach to the current challenges and management strategies employed in LGEA. There are fortunately a range of available surgical techniques for LGEA repair, including delayed primary repair, staged repair based on longitudinal traction strategies to lengthen both ends (e.g., Foker procedure, internal traction), and esophageal replacement using other portions of the gastrointestinal tract. The literature on the management of LGEA has long been dominated by single-center retrospective reviews, but the field has recently witnessed increased multi-center collaboration that has helped to increase our understanding on how to best manage this challenging patient population. Delayed primary repair is strongly preferred as the initial approach in management of LGEA in the United States as well as several European countries and is supported by the American Pediatric Surgery Association recommendations. Should esophageal replacement be required in cases where salvaging the native thoracic esophagus is not possible, gastric conduits are the preferred approach, based on the relative simplicity of the operation, low postoperative morbidity, and long-term durability. Long-term followup for monitoring of swallowing function, nutritional status, aspiration/respiratory illnesses, gastroesophageal reflux, and associated comorbidities is essential in the comprehensive care of these complex patients.

The impact of undergoing elective pediatric lung resection during respiratory syncytial virus peak season on patient outcomes: A nationwide retrospective analysis.

OBJECTIVES: Observational data to support delaying elective pediatric thoracic surgery during peak respiratory viral illness season is lacking. This study evaluated whether lung surgery during peak viral season is associated with differences in postoperative outcomes and resource utilization. METHODS: A retrospective observational cohort study was performed using the Pediatric Health Information System (PHIS). Patients with a congenital lung malformation (CLM) who underwent elective lung resection between 1 January 2016 and 29 February 2020 were included. Respiratory syncytial virus (RSV) incidence was used as a proxy for respiratory viral illness circulation. Monthly hospital-specific RSV incidence was calculated from PHIS data, and peak RSV season was defined by Centers for Disease Control data. Multivariable regression models were built to identify predictors of postoperative mechanical ventilation, which was the main outcome measure, as well as secondary outcomes including 30-day readmission after lung resection, postoperative length of stay (LOS) and hospital billing charges. RESULTS: Of 1542 CLM patients identified, 344 (22.3%) underwent lung resection during peak RSV season. 38% fewer operations were performed per month during peak RSV season than during off-peak months (p < .001). Children who underwent surgery during peak RSV season did not differ from the off-peak group in terms of age at operation, race, or comorbid conditions (i.e., congenital heart disease, newborn respiratory distress, and preoperative pneumonia). There was no association between hospital-specific RSV incidence at the time of surgery and postoperative mechanical ventilation, postoperative LOS, 30-day readmission rate or hospital billing charges. DISCUSSION: Performing elective lung surgery in children with CLMs during peak viral season is not associated with adverse surgical outcomes or increased utilization of healthcare resources.

Postoperative Antibiotics, Outcomes, and Resource Use in Children With Gangrenous Appendicitis.

Importance: Gangrenous, suppurative, and exudative (GSE) findings have been associated with increased surgical site infection (SSI) risk and resource use in children with nonperforated appendicitis. Establishing the role for postoperative antibiotics may have important implications for infection prevention and antimicrobial stewardship. Objective: To compare SSI rates in children with nonperforated appendicitis with GSE findings who did and did not receive postoperative antibiotics. Design, Setting, and Participants: This was a retrospective cohort study using American College of Surgeons' National Surgical Quality Improvement Program (NSQIP)-Pediatric Appendectomy Targeted data from 16 hospitals participating in a regional research consortium. NSQIP data were augmented with operative report and antibiotic use data obtained through supplemental medical record review. Children with nonperforated appendicitis with GSE findings who underwent appendectomy between July 1, 2015, and June 30, 2020, were identified using previously validated intraoperative criteria. Data were analyzed from October 2022 to July 2023. Exposure: Continuation of antibiotics after appendectomy. Main Outcomes and Measures: Rate of 30-day postoperative SSI including both incisional and organ space infections. Complementary hospital and patient-level analyses were conducted to explore the association between postoperative antibiotic use and severity-adjusted outcomes. The hospital-level analysis explored the correlation between postoperative antibiotic use and observed to expected (O/E) SSI rate ratios after adjusting for differences in disease severity (presence of gangrene and postoperative length of stay) among hospital populations. In the patient-level analysis, propensity score matching was used to balance groups on disease severity, and outcomes were compared using mixed-effects logistic regression to adjust for hospital-level clustering. Results: A total of 958 children (mean [SD] age, 10.7 [3.7] years; 567 male [59.2%]) were included in the hospital-level analysis, of which 573 (59.8%) received postoperative antibiotics. No correlation was found between hospital-level SSI O/E ratios and postoperative antibiotic use when analyzed by either overall rate of use (hospital median, 53.6%; range, 31.6%-100%; Spearman ρ = -0.10; P = .71) or by postoperative antibiotic duration (hospital median, 1 day; range, 0-7 days; Spearman ρ = -0.07; P = .79). In the propensity-matched patient-level analysis including 404 patients, children who received postoperative antibiotics had similar rates of SSI compared with children who did not receive postoperative antibiotics (3 of 202 [1.5%] vs 4 of 202 [2.0%]; odds ratio, 0.75; 95% CI, 0.16-3.39; P = .70). Conclusions and Relevance: Use of postoperative antibiotics did not improve outcomes in children with nonperforated appendicitis with gangrenous, suppurative, or exudative findings.

Utility of a Benchmarking Report for Balancing Infection Prevention and Antimicrobial Stewardship in Children with Complicated Appendicitis.

OBJECTIVE: To develop a severity-adjusted, hospital-level benchmarking comparative performance report for postoperative organ space infection and antibiotic utilization in children with complicated appendicitis. BACKGROUND: No benchmarking data exist to aid hospitals in identifying and prioritizing opportunities for infection prevention or antimicrobial stewardship in children with complicated appendicitis. METHODS: This was a multicenter cohort study using NSQIP-Pediatric data from 16 hospitals participating in a regional research consortium, augmented with antibiotic utilization data obtained through supplemental chart review. Children with complicated appendicitis who underwent appendectomy from 07/01/2015 to 06/30/2020 were included. Thirty-day postoperative OSI rates and cumulative antibiotic utilization were compared between hospitals using observed-to-expected (O/E) ratios after adjusting for disease severity using mixed effects models. Hospitals were considered outliers if the 95% confidence interval for O/E ratios did not include 1.0. RESULTS: 1790 patients were included. Overall, the OSI rate was 15.6% (hospital range: 2.6-39.4%) and median cumulative antibiotic utilization was 9.0 days (range: 3.0-13.0). Across hospitals, adjusted O/E ratios ranged 5.7-fold for OSI (0.49-2.80, P=0.03) and 2.4-fold for antibiotic utilization (0.59-1.45, P<0.01). Three (19%) hospitals were outliers for OSI (1 high and 2 low performers), and eight (50%) were outliers for antibiotic utilization (5 high and 3 low utilizers). Ten (63%) hospitals were identified as outliers in one or both measures. CONCLUSIONS: A comparative performance benchmarking report may help hospitals identify and prioritize quality improvement opportunities for infection prevention and antimicrobial stewardship, as well as identify exemplar performers for dissemination of best practices.

Use of Antipseudomonal Antibiotics is not Associated with Lower Rates of Postoperative Drainage Procedures or More Favorable Culture Profiles in Children with Complicated Appendicitis: Results from a Multicenter Regional Research Consortium.

OBJECTIVE: To compare rates of postoperative drainage and culture profiles in children with complicated appendicitis treated with the two most common antibiotic regimens with and without antipseudomonal activity (piperacillin-tazobactam [PT] and ceftriaxone with metronidazole [CM]). SUMMARY OF BACKGROUND DATA: Variation in use of antipseudomonal antibiotics has been driven by a paucity of multicenter data reporting clinically relevant, culture-based outcomes. METHODS: Retrospective cohort study of patients with complicated appendicitis (7/2015-6/2020) using NSQIP-Pediatric data from 15 hospitals participating in a regional research consortium. Operative report details, antibiotic utilization, and culture data were obtained through supplemental chart review. Rates of 30-day postoperative drainage and organism-specific culture positivity were compared between groups using mixed effects regression to adjust for clustering after propensity matching on measures of disease severity. RESULTS: 1002 children met criteria for matching (58.9% received CM and 41.1% received PT). In the matched sample of 778 patients, children treated with PT had similar rates of drainage overall (PT: 11.8%, CM: 12.1%; OR 1.44 [OR:0.71-2.94]) and higher rates of drainage associated with growth of any organism (PT: 7.7%, CM: 4.6%; OR 2.41 [95%CI:1.08-5.39]) and Escherichia coli (PT: 4.6%, CM: 1.8%; OR 3.42 [95%CI:1.07-10.92]) compared to treatment with CM. Rates were similar between groups for drainage associated with multiple organisms (PT: 2.6%, CM: 1.5%; OR 3.81 [95%CI:0.96-15.08]) and Pseudomonas (PT: 1.0%, CM: 1.3%; OR 3.42 [95%CI:0.55-21.28]). CONCLUSIONS AND RELEVANCE: Use of antipseudomonal antibiotics is not associated with lower rates of postoperative drainage procedures or more favorable culture profiles in children with complicated appendicitis.

Serial lung mass volume ratios as prognostic indicators of neonatal respiratory morbidity in fetal pulmonary malformations.

BACKGROUND: Several studies have shown that the congenital pulmonary airway malformation volume ratio is a useful prognosticator of neonatal outcome in prenatally diagnosed lung lesions. However, there remains a lack of consensus on which congenital pulmonary airway malformation volume ratio values have the best predictive value because of operator dependence, inherent changes in lung lesion size throughout gestation, and the widespread use of maternal steroids. OBJECTIVE: This study sought to determine the association between serial congenital pulmonary airway malformation volume ratio measurements and neonatal outcomes among fetuses with lung malformations. STUDY DESIGN: This was a retrospective cohort study of fetuses with a prenatally diagnosed lung malformation managed at 2 major fetal centers from January 2010 to December 2021. Prenatal variables, including prospectively measured congenital pulmonary airway malformation volume ratio measurements (initial, maximum, and final), were analyzed. The results were correlated with 3 outcome measures, namely surgical resection before 30 days of life, a need for supplemental O2 at birth, and endotracheal intubation at birth. Statistical analyses were performed using receiver operating characteristic curve analyses, Welch 2 sample t tests, and multivariable logistic regressions (P<.05). RESULTS: There were 123 fetuses with isolated lung lesions identified. Eight (6.5%) had hydrops. The mean initial congenital pulmonary airway malformation volume ratio was 0.67±0.61 cm2 at 22.9±3.9 weeks' gestation. The mean maximum congenital pulmonary airway malformation volume ratio was 1.08 ± 0.94 cm2 at 27.0 ± 4.0 weeks' gestation. The mean final congenital pulmonary airway malformation volume ratio was 0.58±0.60 cm2 at 33.2±4.1 weeks' gestation. At a mean gestational age at delivery of 38.3±2.6 weeks, 15 (12.2%) underwent neonatal lung resection for symptomatic disease. In a multivariable regression, all 3 congenital pulmonary airway malformation volume ratio measurements showed a significant correlation with neonatal lung resection (P<.001). Optimal congenital pulmonary airway malformation volume ratio cutoffs were established based on an initial congenital pulmonary airway malformation volume ratio of ≥0.8 cm2, maximum congenital pulmonary airway malformation volume ratio of ≥1.5 cm2, and a final congenital pulmonary airway malformation volume ratio of ≥1.3 cm2 with associated areas under the curve of 0.89, 0.97, and 0.93, respectively. The final congenital pulmonary airway malformation volume ratio had the highest specificity for predicting surgical lung resection in the early postnatal period. CONCLUSION: Measuring congenital pulmonary airway malformation volume ratios throughout pregnancy in fetuses with pulmonary malformations has clinical value for prenatal counseling and planning care transition after delivery. Fetuses with a final congenital pulmonary airway malformation volume ratio of more than 1.3 cm2 are likely to require neonatal surgery and therefore should be delivered at tertiary care centers with a neonatal intensive care unit and pediatric surgical expertise.

Sex-Specific Differences in Congenital Diaphragmatic Hernia Mortality.

OBJECTIVE: To compare disease severity and mortality differences between female and male patients with congenital diaphragmatic hernia (CDH). STUDY DESIGN: We queried the CDH Study Group (CDHSG) database for CDH neonates managed between 2007 and 2018. Female and males were compared in statistical analyses using t tests, χ² tests, and Cox regression, as appropriate (P ≤ .05). RESULTS: There were 7288 CDH patients, of which 3048 (41.8%) were female. Females weighed less on average at birth than males (2.84 kg vs 2.97 kg, P < .001) despite comparable gestational age. Females had similar rates of extracorporeal life support (ECLS) utilization (27.8% vs 27.3%, P = .65). Although both cohorts had equivalent defect size and rates of patch repair, female patients had increased rates of intrathoracic liver herniation (49.2% vs 45.9%, P = .01) and pulmonary hypertension (PH) (86.6% vs 81.1%, P < .001). Females had lower survival rates at 30-days (77.3% vs 80.1%, P = .003) and overall lower survival to discharge (70.2% vs 74.2%, P < .001). Subgroup analysis revealed that increased mortality was significant among those who underwent repair but were never supported on ECLS (P = .005). On Cox regression analysis, female sex was independently associated with mortality (adjusted hazard ratio 1.32, P = .02). CONCLUSION: After controlling for the established prenatal and postnatal predictors of mortality, female sex remains independently associated with a higher risk of mortality in CDH. Further study into the underlying causes for sex-specific disparities in CDH outcomes is warranted.

Outcomes and Resource Utilization Associated with Use of Routine Pre-Discharge White Blood Cell Count for Clinical Decision-Making in Children with Complicated Appendicitis: A Multicenter Hospital-Level Analysis.

BACKGROUND: The objective was to explore the hospital-level relationship between routine pre-discharge WBC utilization (RPD-WBC) and outcomes in children with complicated appendicitis. METHODS: Multicenter analysis of NSQIP-Pediatric data from 14 consortium hospitals augmented with RPD-WBC data. WBC were considered routine if obtained within one day of discharge in children who did not develop an organ space infection (OSI) or fever during the index admission. Hospital-level observed-to-expected ratios (O/E) for 30-day outcomes (antibiotic days, imaging utilization, healthcare days, and OSI) were calculated after adjusting for appendicitis severity and patient characteristics. Spearman correlation was used to explore the relationship between hospital-level RPD-WBC utilization and O/E's for each outcome. RESULTS: 1528 children were included. Significant variation was found across hospitals in RPD-WBC use (range: 0.7-100%; p < 0.01) and all outcomes (mean antibiotic days: 9.9 [O/E range: 0.56-1.44, p < 0.01]; imaging: 21.9% [O/E range: 0.40-2.75, p < 0.01]; mean healthcare visit days: 5.7 [O/E 0.74-1.27, p < 0.01]); OSI: 14.1% [O/E range: 0.43-3.64, p < 0.01]). No correlation was found between RPD-WBC use and antibiotic days (r = +0.14, p = 0.64), imaging (r = -0.07, p = 0.82), healthcare days (r = +0.35, p = 0.23) or OSI (r = -0.13, p = 0.65). CONCLUSIONS: Increased RPD-WBC utilization in pediatric complicated appendicitis did not correlate with improved outcomes or resource utilization at the hospital level. LEVEL OF EVIDENCE: III. TYPE OF STUDY: Clinical Research.

Racial and Ethnic Disparities in Outcomes Among Newborns with Congenital Diaphragmatic Hernia.

Importance: There is some data to suggest that racial and ethnic minority infants with congenital diaphragmatic hernia (CDH) have poorer clinical outcomes. Objective: To determine what patient- and institutional-level factors are associated with racial and ethnic differences in CDH mortality. Design, Setting, and Participants: Multicenter cohort study of 49 US children's hospitals using the Pediatric Health Information System database from January 1, 2015, to December 31, 2020. Participants were patients with CDH admitted on day of life 0 who underwent surgical repair. Patient race and ethnicity were guardian-reported vs hospital assigned as Black, Hispanic (White or Black), or White. Data were analyzed from August 2021 to March 2022. Exposures: Patient race and ethnicity: (1) White vs Black and (2) White vs Hispanic; and institutional-level diversity (as defined by the percentage of Black and Hispanic patients with CDH at each hospital): (1) 30% or less, (2) 31% to 40%, and (3) more than 40%. Main Outcomes and Measures: The primary outcomes were in-hospital and 60-day mortality. The study hypothesized that hospitals managing a more racially and ethnically diverse population of patients with CDH would be associated with lower mortality among Black and Hispanic infants. Results: Among 1565 infants, 188 (12%), 306 (20%), and 1071 (68%) were Black, Hispanic, and White, respectively. Compared with White infants, Black infants had significantly lower gestational ages (mean [SD], White: 37.6 [2] weeks vs Black: 36.6 [3] weeks; difference, 1 week; 95% CI for difference, 0.6-1.4; P < .001), lower birthweights (White: 3.0 [1.0] kg vs Black: 2.7 [1.0] kg; difference, 0.3 kg; 95% CI for difference, 0.2-0.4; P < .001), and higher extracorporeal life support use (White: 316 patients [30%] vs Black: 69 patients [37%]; χ21 = 3.9; P = .05). Black infants had higher 60-day (White: 99 patients [9%] vs Black: 29 patients [15%]; χ21 = 6.7; P = .01) and in-hospital (White: 133 patients [12%] vs Black: 40 patients [21%]; χ21 = 10.6; P = .001) mortality . There were no mortality differences in Hispanic patients compared with White patients. On regression analyses, institutional diversity of 31% to 40% in Black patients (hazard ratio [HR], 0.17; 95% CI, 0.04-0.78; P = .02) and diversity greater than 40% in Hispanic patients (HR, 0.37; 95% CI, 0.15-0.89; P = .03) were associated with lower mortality without altering outcomes in White patients. Conclusions and Relevance: In this cohort study of 1565 who underwent surgical repair patients with CDH, Black infants had higher 60-day and in-hospital mortality after adjusting for disease severity. Hospitals treating a more racially and ethnically diverse patient population were associated with lower mortality in Black and Hispanic patients.

Delayed primary repair in 100 infants with isolated long-gap esophageal atresia: A nationwide analysis of children's hospitals.

BACKGROUND: This study aimed to evaluate the contemporary surgical management of long-gap esophageal atresia, a rare and challenging problem managed by pediatric general surgeons. METHODS: A retrospective review of the Pediatric Health Information System database for infants who underwent neonatal gastrostomy, followed by surgical reconstruction for long-gap esophageal atresia (2014-2021). Patients with birthweight less than 1.5 kg and those who received neonatal cardiac surgery were excluded. Outcomes were analyzed, including the need for further procedures, length of stay, and mortality. RESULTS: Of 1,346 infants who underwent repair across 47 major children's hospitals, 100 (7%) met the inclusion criteria for long-gap esophageal atresia. Cardiac anomalies were identified in 43% of patients. The median age at repair was 87 days (interquartile range, 62-133). Ten percent of patients had a planned or unplanned reoperation ≤30 days after index surgery, and 4% underwent reoperation at >30 days. The median time to reoperation was 9 days (interquartile range, 7-60). Mortality during index admission was 5%, and the median hospital length of stay was 143 days (interquartile range, 101-192). Length of stay was significantly longer in patients with cardiac anomalies (cardiac: 179 days, non-cardiac: 125 days; P < .001), and 52% of patients required at least 1 postoperative dilation. The median time to the first dilation was 70 days (interquartile range, 42-173). CONCLUSION: This large multicenter study highlights the challenges of infants with long-gap esophageal atresia but suggests a high rate of successful delayed primary reconstruction. Hospitalizations are prolonged, and anastomotic stricture rates remain high. These data are useful for pediatric surgeons in counseling families on surgical repair strategy, timing, and postoperative outcomes.

Prenatal Sonography in Suspected Proximal Gastrointestinal Obstructions: Diagnostic Accuracy and Neonatal Outcomes.

BACKGROUND: The purpose of this study was to assess diagnostic accuracy and neonatal outcomes in fetuses with a suspected proximal gastrointestinal obstruction (GIO). METHODS: After IRB approval, a retrospective chart review was conducted on prenatally suspected and/or postnatally confirmed cases of proximal GIO at a tertiary care facility (2012-2022). Maternal-fetal records were queried for presence of a double bubble ± polyhydramnios, and neonatal outcomes were assessed to calculate the diagnostic accuracy of fetal sonography. RESULTS: Among 56 confirmed cases, the median birthweight and gestational age at birth were 2550 g [interquartile range (IQR) 2028-3012] and 37 weeks (IQR 34-38), respectively. There was one (2%) false-positive and three (6%) false-negatives by ultrasound. Double bubble had a sensitivity, specificity, positive predictive value, and negative predictive value for proximal GIO of 85%, 98%, 98%, and 83%, respectively. Pathologies included 49 (88%) with duodenal obstruction/annular pancreas, three (5%) with malrotation, and three (5%) with jejunal atresia. The median postoperative length of stay was 27 days (IQR 19-42). Cardiac anomalies were associated with significantly higher complications (45% vs 17%, p = 0.030). CONCLUSIONS: In this contemporary series, fetal sonography has high diagnostic accuracy for detecting proximal gastrointestinal obstruction. These data are informative for pediatric surgeons in prenatal counseling and preoperative discussions with families. LEVEL OF EVIDENCE: Diagnostic Study, Level III.

Esophageal Surveillance Practices in Esophageal Atresia Patients: A Survey by the Eastern Pediatric Surgery Network.

INTRODUCTION: Endoscopic surveillance guidelines for patients with repaired esophageal atresia (EA) rely primarily on expert opinion. Prior to embarking on a prospective EA surveillance registry, we sought to understand EA surveillance practices within the Eastern Pediatric Surgery Network (EPSN). METHODS: An anonymous, 23-question Qualtrics survey was emailed to 181 physicians (surgeons and gastroenterologists) at 19 member institutions. Likert scale questions gauged agreement with international EA surveillance guideline-derived statements. Multiple-choice questions assessed individual and institutional practices. RESULTS: The response rate was 77%. Most respondents (80%) strongly agree or agree that EA surveillance endoscopy should follow a set schedule, while only 36% claimed to perform routine upper GI endoscopy regardless of symptoms. Many institutions (77%) have an aerodigestive clinic, even if some lack a multi-disciplinary EA team. Most physicians (72%) expressed strong interest in helping develop evidence-based guidelines. CONCLUSIONS: Our survey reveals physician agreement with current guidelines but weak adherence. Surveillance methods vary greatly, underscoring the lack of evidence-based data to guide EA care. Aerodigestive clinics may help implement surveillance schedules. Respondents support evidence-based protocols, which bodes well for care standardization. Results will inform the first multi-institutional EA databases in the United States (US), which will be essential for evidence-based care. LEVEL OF EVIDENCE: This is a prognosis study with level 4 evidence.

Morbidity and Mortality in Neonates with Symptomatic Congenital Lung Malformation.

BACKGROUND: Neonatal resection is the mainstay treatment of children presenting with symptomatic congenital lung malformation (CLM) at birth. The objective of this study was to evaluate risk factors for increased morbidity and mortality after neonatal CLM resection using a large multicenter database. STUDY DESIGN: Retrospective review of the Pediatric Health Information System database was performed. Children with a symptomatic CLM managed by lung resection before 30 days of age were included (2016 to 2021). Primary outcomes measures were postoperative respiratory complication and any complication, including death. RESULTS: Of 1,791 CLM patients identified, 256 (14%) underwent neonatal resection for symptomatic disease. Pathology included 123 (48%) congenital pulmonary airway malformation, 24 (10%) bronchopulmonary sequestration, 5 (2%) congenital lobar emphysema, 16 (6%) hybrid, and 88 (34%) unclassified lesion. Preoperative mechanical ventilation and extracorporeal membrane oxygenation (ECMO) were employed in 149 (58.2%) and 17 (6.7%) of cases, respectively. The median age at resection was 6.5 days (interquartile range 2 to 23). Postoperatively, 25 (10%) required mechanical ventilation for 48 hours or more, 3 (1%) continued ECMO, and 3 (1%) required ECMO rescue. The overall respiratory complication rate was 34% (87), rate of any complication was 51% (130), median postoperative length of stay was 20 days (interquartile range 9 to 52), and mortality rate was 14.5% (37). Birthweight was inversely correlated with complication risk (incidence rate ratio 0.55, 95% CI 0.36 to 0.83, p = 0.006). Cardiac structural anomaly was associated with a 21-day longer postoperative length of stay (95% CI 6 to 35, p = 0.006) and 2.2 times increased risk of any complication (95% CI 1.18 to 4.02, p = 0.014). CONCLUSIONS: In this large multicenter study, ECMO use and mortality are relatively uncommon among neonates undergoing lung resection for a symptomatic CLM. However, postoperative morbidity remains high, particularly in those with cardiac structural disease.

Predictive Value of Routine WBC Count Obtained Before Discharge for Organ Space Infection in Children with Complicated Appendicitis: Results from the Eastern Pediatric Surgery Network.

BACKGROUND: The objective of this study was to evaluate the clinical utility of a routine predischarge WBC count (RPD-WBC) for predicting postdischarge organ space infection (OSI) in children with complicated appendicitis. STUDY DESIGN: This was a multicenter study using NSQIP-Pediatric data from 14 hospitals augmented with RPD-WBC data obtained through supplemental chart review. Children with fever or surgical site infection diagnosed during the index admission were excluded. The positive predictive value (PPV) for postdischarge OSI was calculated for RPD-WBC values of persistent leukocytosis (≥9.0 × 10 3 cells/µL), increasing leukocytosis (RPD-WBC > preoperative WBC), quartiles of absolute RPD-WBC, and quartiles of relative proportional change from preoperative WBC. Logistic regression was used to calculate predictive values adjusted for patient age, appendicitis severity, and use of postdischarge antibiotics. RESULTS: A total of 1,264 children were included, of which 348 (27.5%) had a RPD-WBC obtained (hospital range: 0.8 to 100%, p < 0.01). The median RPD-WBC was similar between children who did and did not develop a postdischarge OSI (9.0 vs 8.9; p = 0.57), and leukocytosis was absent in 50% of children who developed a postdischarge OSI. The PPV of RPD-WBC was poor for both persistent and increasing leukocytosis (3.9% and 9.8%, respectively) and for thresholds based on the quartiles of highest RPD-WBC values (>11.1, PPV: 6.4%) and greatest proportional change (<32% decrease from preoperative WBC; PPV: 7.8%). CONCLUSIONS: Routine predischarge WBC data have poor predictive value for identifying children at risk for postdischarge OSI after appendectomy for complicated appendicitis.

Association Between Antibiotic Redosing Before Incision and Risk of Incisional Site Infection in Children With Appendicitis.

OBJECTIVE: To evaluate whether redosing antibiotics within an hour of incision is associated with a reduction in incisional surgical site infection (iSSI) in children with appendicitis. BACKGROUND: Existing data remain conflicting as to whether children with appendicitis receiving antibiotics at diagnosis benefit from antibiotic redosing before incision. METHODS: This was a multicenter retrospective cohort study using data from the Pediatric National Surgical Quality Improvement Program augmented with antibiotic utilization and operative report data obtained though supplemental chart review. Children undergoing appendectomy at 14 hospitals participating in the Eastern Pediatric Surgery Network from July 2016 to June 2020 who received antibiotics upon diagnosis of appendicitis between 1 and 6 hours before incision were included. Multivariable logistic regression was used to compare odds of iSSI in those who were and were not redosed with antibiotics within 1 hour of incision, adjusting for patient demographics, disease severity, antibiotic agents, and hospital-level clustering of events. RESULTS: A total of 3533 children from 14 hospitals were included. Overall, 46.5% were redosed (hospital range: 1.8%-94.4%, P <0.001) and iSSI rates were similar between groups [redosed: 1.2% vs non-redosed: 1.3%; odds ratio (OR) 0.84, (95%,CI, 0.39-1.83)]. In subgroup analyses, redosing was associated with lower iSSI rates when cefoxitin was used as the initial antibiotic (redosed: 1.0% vs nonredosed: 2.5%; OR: 0.38, (95% CI, 0.17-0.84)], but no benefit was found with other antibiotic regimens, longer periods between initial antibiotic administration and incision, or with increased disease severity. CONCLUSIONS: Redosing of antibiotics within 1 hour of incision in children who received their initial dose within 6 hours of incision was not associated with reduction in risk of incisional site infection unless cefoxitin was used as the initial antibiotic.

Morbidity in children after fetoscopic endoluminal tracheal occlusion for severe congenital diaphragmatic hernia: Results from a multidisciplinary clinic.

BACKGROUND: Although fetoscopic endoluminal tracheal occlusion (FETO) was recently shown to improve survival in a multicenter, randomized trial of severe congenital diaphragmatic hernia (CDH), morbidity outcomes remain essentially unknown. The purpose of this study was to assess long-term outcomes in children with severe CDH who underwent FETO. METHODS: We conducted a prospective study of severe CDH patients undergoing FETO at an experienced North American center from 2015-2021 (NCT02710968). This group was compared to a cohort of non-FETO CDH patients with severe disease as defined by liver herniation, large defect size, and/or ECMO use. Clinical data were collected through a multidisciplinary CDH clinic. Statistics were performed with t-tests and Chi-squared analyses (p≤0.05). RESULTS: There were 18 FETO and 17 non-FETO patients. ECMO utilization was 56% in the FETO cohort. Despite significantly lower median observed/expected lung-to-head ratio (O/E LHR) in the FETO group, [FETO: 23% (IQR:18-25) vs. non-FETO: 36% (IQR: 28-41), p<0.001], there were comparable survival rates at discharge (FETO: 78% vs. non-FETO: 59%, p = 0.23) and at 5-years (FETO: 67% vs. non-FETO: 59%, p = 0.53) between the two cohorts. At a median follow up of 5.8 years, metrics of pulmonary hypertension, pulmonary morbidity, and gastroesophageal reflux disease improved among patients after FETO. However, most FETO patients remained on bronchodilators/inhaled corticosteroids (58%) and were feeding tube dependent (67%). CONCLUSIONS: These North American data show that prenatal tracheal occlusion, in conjunction with a long-term multidisciplinary CDH clinic, is associated with acceptable long-term survival and morbidity in children after FETO. LEVEL OF EVIDENCE: Level III.