We compared early neurodevelopmental morbidity in young children with severe CDH who underwent FETO to those without fetal therapy.
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.
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.
These initial data suggest that FETO is associated with favorable neurodevelopmental outcomes at 24-months compared to severe CDH under expectant management.
III.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
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).
Infant lung surgery is a relatively uncommon procedure performed by pediatric surgeons nationwide. The relationship between surgical volume and postoperative outcomes remains unknown.
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.
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).
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.
To compare disease severity and mortality differences between female and male patients with congenital diaphragmatic hernia (CDH).
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).
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).
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.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
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.
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.
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).
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.
This study aimed to evaluate the contemporary surgical management of long-gap esophageal atresia, a rare and challenging problem managed by pediatric general surgeons.
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.
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).
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.
Display omitted
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
The purpose of this study was to assess diagnostic accuracy and neonatal outcomes in fetuses with a suspected proximal gastrointestinal obstruction (GIO).
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.
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).
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.
Diagnostic Study, Level III.
•Congenital gastrointestinal obstruction (GIO) is rare with incidence of 1 in 2000 live births.•No previous reports on the diagnostic accuracy of double bubble with or without polyhydramnios have used a prenatal ultrasound database as well as a surgical registry to assess the true false negative rates.•Prenatal double bubble has high specificity and positive predictive value for GIO, which are increased when associated with polyhydramnios.•Concomitant cardiac anomalies carry an increased risk of associated complications.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
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.
Full text
Available for:
BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
•What is currently known about this topic?•The use of FETO in severe CDH is associated with improved inpatient survival compared to expectant prenatal management.•What new information is contained in ...this article? The survival rate after FETO was 67% at a median age of 5.8 years.•Pulmonary antihypertensive use declined between 12% and 29%, depending on the medication.•Bronchodilator and inhaled corticosteroids decreased by 21% and 28%, respectively.
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.
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).
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%).
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 III
Display omitted
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
There is some data to suggest that racial and ethnic minority infants with congenital diaphragmatic hernia (CDH) have poorer clinical outcomes.
To determine what patient- and institutional-level ...factors are associated with racial and ethnic differences in CDH mortality.
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.
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%.
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.
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.
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.
