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Objective The purpose of this study was to evaluate the safety of a water-soluble contrast challenge as part of a nonoperative management algorithm in children with an adhesive small bowel obstruction (ASBO). Background Predicting which children will successfully resolve their ASBO with non-operative management at the time of admission remains difficult. Additionally, the safety of a water-soluble contrast challenge for children with ASBO has not been established in the literature. Methods A retrospective review was performed of patients who underwent non-operative management for an ASBO and received a contrast challenge across 5 children's hospitals between 2012 and 2020. Safety was assessed by comparing the complication rate associated with a contrast challenge against a pre-specified maximum acceptable level of 5%. Sensitivity, specificity, negative (NPV) and positive (PPV) predictive values of a contrast challenge to identify successful nonoperative management were calculated. Results Of 82 children who received a contrast challenge, 65% were successfully managed nonoperatively. The most common surgical indications were failure of the contrast challenge or failure to progress after initially passing the contrast challenge. There were no complications related to contrast administration (0%; 95% confidence interval: 0-3.6%, P = 0.03). The contrast challenge was highly reliable in determining which patients would require surgery and which could be successfully managed non-operatively (sensitivity 100%, specificity 86%, NPV 100%, PPV 93%). Conclusion A contrast challenge is safe in children with ASBO and has a high predictive value to assist in clinical decision-making.

OBJECTIVE To assess current clinical outcomes in children with prenatally diagnosed congenital lung malformations (CLMs) and to identify prenatal characteristics associated with adverse outcomes. SUMMARY BACKGROUND DATA Despite a wide spectrum of clinical disease, the identification of fetal CLM subgroups at increased risk for hydrops and respiratory compromise at delivery have not been well defined. METHODS A retrospective cohort study was conducted using an operative database of prenatally diagnosed CLMs managed at eleven children's hospitals from 2009-2016. Statistical analyses were performed using non-parametric bivariate or multivariable logistic regression. RESULTS Three hundred forty-four children were analyzed. Fifteen (5.5%) fetuses were managed with maternal steroids in the setting of hydrops, and prenatal surgical intervention was uncommon (1.7%). Seventy-five (21.8%) had respiratory symptoms at birth, and 34 (10.0%) required neonatal lung resection. Congenital pulmonary airway malformation volume ratio (CVR) measurements were recorded in 169 (49.1%) cases and were significantly associated with perinatal outcome, including hydrops, respiratory distress at birth, need for supplemental oxygen, neonatal ventilator use, and neonatal resection (p < 0.001). An initial CVR ≤ 1.4 was significantly correlated with a reduced risk for hydrops [area under the curve (AUC), 0.93; 95% confidence interval (CI), 0.87-1.00]. A maximum CVR < 0.9 (AUC, 0.72; 95% CI, 0.67-0.85) was associated with a low risk for respiratory symptoms at birth. CONCLUSION In this large, multi-institutional study, an initial CVR ≤ 1.4 identifies fetuses at very low risk for hydrops, and a maximum CVR < 0.9 is associated with asymptomatic disease at birth. These findings represent an opportunity for standardization and quality improvement for prenatal counseling and delivery planning.

OBJECTIVE To determine if risk-adjusted survival of patients with congenital diaphragmatic hernia (CDH) has improved over the last 25 years within centers that are long-term, consistent participants in the CDH Study Group (CDHSG). SUMMARY BACKGROUND DATA The CDHSG is a multicenter collaboration focused on evaluation of infants with CDH. Despite advances in pediatric surgical and intensive care, CDH mortality has appeared to plateau. Herein, we studied CDH mortality rates amongst long-term contributors to the CDHSG. METHODS We divided registry data into five-year intervals, with Era 1 (E1) beginning in 1995, and analyzed multiple variables (operative strategy, defect size, and mortality) to assess evolution of disease characteristics and severity over time. For mortality analyses, patients were risk stratified using a validated prediction score based on 5-minute Apgar (Apgar5) and birth weight. A risk-adjusted, observed to expected (O:E) mortality model was created using E1 as a reference. RESULTS 5,203 patients from 23 centers with ≥22 years of participation were included. Birth weight, Apgar5, diaphragmatic agenesis, and repair rate were unchanged over time (all p > 0.05). In E5 compared to E1, minimally invasive and patch repair were more prevalent, and timing of diaphragmatic repair was later (all p < 0.01). Overall mortality decreased over time: E1 (30.7%), E2 (30.3%), E3 (28.7%), E4 (26.0%), E5 (25.8%) (p = 0.03). Risk-adjusted mortality showed a significant improvement in E5 compared to E1 (OR 0.78, 95% CI 0.62-0.98; p = 0.03). O:E mortality improved over time, with the greatest improvement in E5. CONCLUSIONS Risk-adjusted and observed-to-expected CDH mortality have improved over time.

Objective: The objectives of this study were (i) to evaluate infants with congenital diaphragmatic hernia (CDH) that do not undergo repair, (ii) to identify nonrepair rate by institution, and (iii) to compare institutional outcomes based on nonrepair rate. Background: Approximately 20% of infants with CDH go unrepaired and the threshold to offer surgical repair is variable. Methods: Data were abstracted from a multicenter, prospectively collected database. Standard clinical variables, including repair (or nonrepair), and outcome were analyzed. Institutions were grouped based on volume and rate of nonrepair. Preoperative mortality predictors were identified using logistic regression, expected mortality for each center was calculated, and observed /expected (O/E) ratios were computed for center groups and compared by Kruskal-Wallis ANOVA. Results: A total of 3965 infants with CDH were identified and 691 infants (17.5%) were not repaired. Nonrepaired patients had lower Apgar scores (P < 0.05) and increased incidence of anomalies (P < 0.0001). Low-volume centers ("Lo", n=44 total, < 10 CDH pts/yr) and high-volume centers ("Hi", n = 21) had median nonrepair rates of 19.8% (range 0%-66.7%) and 16.7% (5.1%-38.5%), respectively. High-volume centers were further dichotomized by rate of nonrepair (HiLo = 5.1-16.7% and HiHi = 17.6-38.5%), leaving 3 groups: HiLo, HiHi, and Lo. Predictors of mortality were lower birth weight, lower Apgar scores, prenatal diagnosis, and presence of congenital anomalies. O/E ratios for mortality in the HiLo, HiHi, and Lo groups were 0.81, 0.94, and 1.21, respectively (P < 0.0001). For every 100 CDH patients, HiLo centers have 2.73 (2.4-3.1, 95% confidence interval) survivors beyond expectation. Conclusions: There are significant differences between repaired and nonrepaired CDH infants and significant center variation in rate of nonrepair exists. Aggressive surgical management, leading to a low rate of nonrepair, is associated with improved risk-adjusted mortality.

OBJECTIVE: To evaluate the safety and efficacy of partial liquid ventilation (PLV) in adult patients with the acute respiratory distress syndrome (ARDS). SUMMARY BACKGROUND DATA: Previous studies have evaluated gas exchange and the safety of PLV in adult patients with severe respiratory failure whose gas exchange was partially provided by extracorporeal life support (ECLS). This is the first experience with adult patients who were not on ECLS. METHODS: Intratracheal perflubron in a total dose of 30.1 +/- 7.1 ml/kg was administered over a period of 45 +/- 9 hours to nine adult patients with mean age = 49 +/- 4 years and mean PaO2/FiO2 ratio = 128 +/- 7 as part of a prospective, multicenter, phase I-II noncontrolled trial. RESULTS: Significant decreases in mean (A-a)DO2 (baseline = 430 +/- 47, 48 hour = 229 +/- 17, p = 0.0127 by ANOVA) and FiO2 (baseline = 0.82 +/- 0.08, 48 hour = 0.54 +/- 0.06, p = 0.025), along with an increase in mean SvO2 (baseline = 75 +/- 3, 48 hour = 85 +/- 2, p = 0.018 by ANOVA) were observed. No significant changes in pulmonary compliance or hemodynamic variables were noted. Seven of the nine patients in this study survived beyond 28 days after initiation of partial liquid ventilation whereas 5 patients survived to discharge. Three adverse events [hypoxia (2) and hyperbilirubinemia (1)] were determined to be severe in nature. CONCLUSIONS: These data suggest that PLV may be performed safely with few related severe adverse effects. Improvement in gas exchange was observed in this series of adult patients over the 48 hours after initiation of PLV.

OBJECTIVE: The authors retrospectively reviewed their experience with extracorporeal life support (ECLS) in 100 adult patients with severe respiratory failure (ARF) to define techniques, characterize its efficacy and utilization, and determine predictors of outcome. SUMMARY BACKGROUND DATA: Extracorporeal life support maintains gas exchange during ARF, providing diseased lungs an optimal environment in which to heal. Extracorporeal life support has been successful in the treatment of respiratory failure in infants and children. In 1990, the authors instituted a standardized protocol for treatment of severe ARF in adults, which included ECLS when less invasive methods failed. METHODS: From January 1990 to July 1996, the authors used ECLS for 100 adults with severe acute hypoxemic respiratory failure (n = 94): paO2/FiO2 ratio of 55.7+/-15.9, transpulmonary shunt (Qs/Qt) of 52+/-22%, or acute hypercarbic respiratory failure (n = 6): paCO2 84.0+/-31.5 mmHg, despite and after maximal conventional ventilation. The technique included venovenous percutaneous access, lung "rest," transport on ECLS, minimal anticoagulation, hemofiltration, and optimal systemic oxygen delivery. RESULTS: Overall hospital survival was 54%. The duration of ECLS was 271.9+/-248.6 hours. Primary diagnoses included pneumonia (49 cases, 53% survived), adult respiratory distress syndrome (45 cases, 51 % survived), and airway support (6 cases, 83% survived). Multivariate logistic regression modeling identified the following pre-ECLS variables significant independent predictors of outcome: 1) pre-ECLS days of mechanical ventilation (p = 0.0003), 2) pre-ECLS paO2/FiO2 ratio (p = 0.002), and 3) age (years) (p = 0.005). Modeling of variables during ECLS showed that no mechanical complications were independent predictors of outcome, and the only patient-related complications associated with outcome were the presence of renal failure (p < 0.0001) and significant surgical site bleeding (p = 0.0005). CONCLUSIONS: Extracorporeal life support provides life support for ARF in adults, allowing time for injured lungs to recover. In 100 patients selected for high mortality risk despite and after optimal conventional treatment, 54% survived. Extracorporeal life support is extraordinary but reasonable treatment in severe adult respiratory failure. Predictors of survival exist that may be useful for patient prognostication and design of future prospective studies.

OBJECTIVE: The authors evaluated gas exchange, pulmonary function, and lung histology during perfluorocarbon liquid ventilation (LV) when compared with gas ventilation (GV) in the setting of severe respiratory failure. BACKGROUND: The efficacy of LV in the setting of respiratory failure has been evaluated in premature animals with surfactant deficiency. However, very little work has been performed in evaluating the efficacy of LV in older animal models of the adult respiratory distress syndrome (ARDS). METHODS: A stable model of lung injury was induced in 12 young sheep weighing 16.4 +/- 3.0 kg using right atrial injection of 0.07 mL/kg of oleic acid followed by saline pulmonary lavage and bijugular venovenous extracorporeal life support (ECLS). For the first 30 minutes on ECLS, all animals were ventilated with gas. Animals were then ventilated with either 15 mL/kg gas (GV, n = 6) or perflubron ([PFC], LV, n = 6) over the ensuing 2.5 hours. Subsequently, ECLS was discontinued in five of the GV animals and five of the LV animals, and GV or LV continued for 1 hour or until death. MAIN FINDINGS: Physiologic shunt (Qps/Qt) was significantly reduced in the LV animals when compared with the GV animals (LV = 31 +/- 10%; GV = 93 +/- 4%; p < 0.001) after 3 hours of ECLS. At the same time point, pulmonary compliance (CT) was significantly increased in the LV group when compared with the GV group (LV = 1.04 +/- 0.19 mL/cm H2O/kg; GV = 0.41 +/- 0.02 mL/cm H2O/kg; p < 0.001). In addition, the ECLS flow rate required to maintain the PaO2 in the 50- to 80-mm Hg range was substantially and significantly lower in the LV group when compared with that of the GV group (LV = 14 +/- 5 mL/kg/min; GV = 87 +/- 15 mL/kg/min; p < 0.001). All of the GV animals died after discontinuation of ECLS, whereas all the LV animals demonstrated effective gas exchange without extracorporeal support for 1 hour (p < 0.01). Lung biopsy light microscopy demonstrated a marked reduction in alveolar hemorrhage, lung fluid accumulation, and inflammatory infiltration in the LV group when compared with the GV animals. CONCLUSION: In a model of severe respiratory failure, LV improves pulmonary gas exchange and compliance with an associated reduction in alveolar hemorrhage, edema, and inflammatory infiltrate.

Severe acute respiratory distress syndrome (ARDS) is associated with a high level of mortality. Extracorporeal life support (ECLS) during severe ARDS maintains oxygen and carbon dioxide gas exchange while providing an optimal environment for recovery of pulmonary function. Since 1989, we have used a protocol-driven algorithm for treatment of severe ARDS, which includes the use of ECLS when standard therapy fails. The objective of this study was to evaluate our experience with ECLS in adult patients with severe ARDS with respect to mortality and morbidity.We reviewed our complete experience with ELCS in adults from January 1, 1989, through December 31, 2003. Severe ARDS was defined as acute onset pulmonary failure, with bilateral infiltrates on chest x-ray, and PaO2/fraction of inspired oxygen (FiO2) ratioor =100 or A-aDO2600 mm Hg despite maximal ventilator settings. The indication for ECLS was acute severe ARDS unresponsive to optimal conventional treatment. The technique of ECLS included veno-venous or veno-arterial vascular access, lung "rest" at low FiO2 and inspiratory pressure, minimal anticoagulation, and optimization of systemic oxygen delivery.During the study period, ECLS was used for 405 adult patients age 17 or older. Of these 405 patients, 255 were placed on ECLS for severe ARDS refractory to all other treatment. Sixty-seven percent were weaned off ECLS, and 52% survived to hospital discharge. Multivariate logistic regression analysis identified the following pre-ELCS variables as significant independent predictors of survival: (1) age (P = 0.01); (2) gender (P = 0.048); (3) pHor =7.10 (P = 0.01); (4) PaO2/FiO2 ratio (P = 0.03); and (5) days of mechanical ventilation (P0.001). None of the patients who survived required permanent mechanical ventilation or supplemental oxygen therapy.Extracorporeal life support for severe ARDS in adults is a successful therapeutic option in those patients who do not respond to conventional mechanical ventilator strategies.

OBJECTIVE: To evaluate the safety and efficacy of partial liquid ventilation (PLV) in adult patients with the acute respiratory distress syndrome (ARDS). SUMMARY BACKGROUND DATA: Previous studies have evaluated gas exchange and the safety of PLV in adult patients with severe respiratory failure whose gas exchange was partially provided by extracorporeal life support (ECLS). This is the first experience with adult patients who were not on ECLS. METHODS: Intratracheal perflubron in a total dose of 30.1 +/- 7.1 ml/kg was administered over a period of 45 +/- 9 hours to nine adult patients with mean age = 49 +/- 4 years and mean PaO2/FiO2 ratio = 128 +/- 7 as part of a prospective, multicenter, phase I-II noncontrolled trial. RESULTS: Significant decreases in mean (A-a)DO2 (baseline = 430 +/- 47, 48 hour = 229 +/- 17, p = 0.0127 by ANOVA) and FiO2 (baseline = 0.82 +/- 0.08, 48 hour = 0.54 +/- 0.06, p = 0.025), along with an increase in mean SvO2 (baseline = 75 +/- 3, 48 hour = 85 +/- 2, p = 0.018 by ANOVA) were observed. No significant changes in pulmonary compliance or hemodynamic variables were noted. Seven of the nine patients in this study survived beyond 28 days after initiation of partial liquid ventilation whereas 5 patients survived to discharge. Three adverse events [hypoxia (2) and hyperbilirubinemia (1)] were determined to be severe in nature. CONCLUSIONS: These data suggest that PLV may be performed safely with few related severe adverse effects. Improvement in gas exchange was observed in this series of adult patients over the 48 hours after initiation of PLV.

Objective The authors reviewed their experience with extracorporeal life support (ECLS) in neonatal respiratory failure; they define changes in patient population, technique, and outcomes. Summary background data Extracorporeal life support has progressed from laboratory research to initial clinical trials in 1972. Following a decade of clinical research, ECLS is now standard treatment for neonatal respiratory failure refractory to conventional pulmonary support techniques. Our group has the longest and largest experience with this technique. Methods Between 1973 and 1993, 460 neonates with severe respiratory failure were treated using ECLS. The records of all patients were reviewed. Results Overall survival was 87%. Primary diagnoses were meconium aspiration syndrome (MAS; 169 cases [96% survival]), respiratory distress syndrome/hyaline membrane disease (91 cases [88% survival]), persistent pulmonary hypertension of the newborn (37 cases [92%]), pneumonia/sepsis (75 cases [84% survival]), congenital diaphragmatic hernia (CDH; 67 cases [67% survival]), and other diagnoses (21 cases [71% survival]). Common mechanical complications included clots in the circuit (136; 85% survival); air in the circuit (67; 82% survival); cannula problems (65; 83% survival) and oxygenator failure (34; 65% survival). Patient-related complications included intracranial infarct or bleed (54 cases; 61% survival), major bleeding (48 cases; 81% survival), seizures (88 cases; 76% survival), metabolic abnormalities (158 cases; 71% survival) and infection (21 cases; 48% survival). Since 1989, treatment groups have been expanded to include premature infants (13 cases; 62% survival), infants with grade I intracranial hemorrhage (28 cases; 54% survival) and "non-honeymoon" CDH patients (15 cases; 27% survival). Since 1990, single-catheter venovenous access has been used in 131 patients (97% survival) and currently is the preferred mode of access. Follow-up ranges from 1 to 19 years; 80% of patients are growing and developing normally. Conclusions Extracorporeal life support has become standard treatment for severe neonatal respiratory failure in our center (460 cases; 87% survival), and worldwide (8913 cases; 81% survival). The availability of ECLS makes the evaluation of other innovative methods of treatment, such as late elective repair of diaphragmatic hernia and new pulmonary vasodilators, possible. The application of ECLS is now being extended to premature and low-birth weight infants as well as older children and adults.

Objective To review retrospectively a 4-year experience with pediatric surgical networking at a major academic medical center in the Midwest. Background The growth of managed care in the United States during the past decade has had a major impact on the practice of medicine in general, but especially on academic medicine. In some academic medical centers, the loss of market share has not only affected clinical activity but has also compromised the educational and research missions of these institutions. Methods At the authors' institution, a networking strategy in pediatric surgery was established in 1993 and implemented on July 1, 1994. In 1994, one new satellite practice was established; over the next 4 years, four additional practices were added, including one in another state. To assess the impact on financial status, clinical activity, education, and academic productivity, the following parameters were analyzed: gross and net revenue, surgical cases, clinic visits, ranking of the pediatric surgery residency, publications, grant support, and development and endowment funds. Results Gross and net revenue increased from $3,273,000 and $302,000 in 1993 to $10,087,000 and $2,826,000, respectively, in 1998. Surgical cases and clinic visits increased from 1240 and 3751 in 1993 to 5872 and 11,604, respectively, in 1998. At the medical center's children's hospital, surgical cases and clinic visits increased from 1240 and 3751 to 2592 and 4729 during the same time period. During this 4-year period, the faculty increased from 4 to 11. Since 1997, the National Resident Matching Program has provided data on how pediatric surgery residency candidates ranked a training program. In 1997, this program received the second-most one to five rankings; in 1998, it tied for first. This exceeds the faculty's perception of previous years' rankings. Publications increased from 26 in 1993 to a peak number of 62 in 1996; in 1997 and 1998 the publications were 48 and 37, respectively. Extemal grant support increased from $139,882 in 1993 to a total of $6,109,971 in 1998. Development and endowment funds increased from $103,559 in 1993 to $2,702,2777 in 1998. Conclusions Pediatric surgical networking at the authors' institution has had a markedly positive impact on finances, clinical activity, education, and academic productivity during a 4-year period. The residency training program appears to have improved in popularity among candidates, probably because of the increased referral of complex cases to the medical center from the various networking satellites. External grant support and basic laboratory research significantly increased, most likely because of the greater number of faculty with protected time for research recruited. Development and endowment funds dramatically grew because of the excellent fiscal health of the pediatric surgical program. This experience may serve as a model for other academic surgical specialties.