Improved Survival in Left Liver-Up Congenital Diaphragmatic Hernia by Early Repair Before Extracorporeal Membrane Oxygenation: Optimization of Patient Selection by Multivariate Risk Modeling.

BACKGROUND: Delayed repair of congenital diaphragmatic hernia (CDH) for days or longer has become standard, allowing improved stabilization for many, but potentially complicating treatment in severely affected infants who require extracorporeal membrane oxygenation (ECMO) and arrive unrepaired. Survival in left liver-up CDH, the most severe anatomic subset, averages 45% in published studies, with deaths often occurring in patients who failed to improve on ECMO and are repaired late, or not at all. Reliable early prediction of ECMO risk in these patients could identify the best candidates for repair before ECMO. We sought to predict ECMO risk in left liver-up CDH, and to further evaluate survival stratified by surgical timing in these patients. STUDY DESIGN: We reviewed 298 single-center, consecutive CDH patients, focusing on 87 inborn left liver-up patients without associated lethal anomalies. Multivariate analysis using anatomic and physiologic markers of severity was performed to define associations with need for ECMO. RESULTS: Sixty of 87 ECMO-eligible inborn left liver-up CDH patients required ECMO (69%). Of these, 20 of 21 (95%) repaired in the first 60 hours and before ECMO survived; whereas 13 of 20 (65%) who had repair delayed and arrived to ECMO unrepaired survived (p = 0.018). Lung-to-head ratio, Apgar scores, Congenital Diaphragmatic Hernia Study Group-predicted survival, pH, PCO2, and PO2 at 1 hour of life all correlated strongly with risk for ECMO. Accurate multivariate models to predict ECMO (area under the receiver operating characteristic curve [AUC] 0.91 and 0.91) were successfully developed. CONCLUSIONS: Early repair of left liver-up CDH before ECMO results in improved survival. Multivariate models can accurately assess risk for ECMO at 1 hour of life, permitting stratification of CDH surgical timing to maximize survival potential while minimizing risk.

Outcomes in the physiologically most severe congenital diaphragmatic hernia (CDH) patients: Whom should we treat?

PURPOSE: Centers that care for newborns with congenital diaphragmatic hernia (CDH) may impose selection criteria for offering or limiting aggressive support in those patients most severely affected. The purpose of this study was to analyze outcomes in newborns with highly severe CDH uniformly treated for survival. METHODS: We reviewed 172 consecutive inborn patients without associated lethal anomalies treated at a single institution with a dedicated CDH program. Survival, respiratory outcome, and time to discharge in the most severe 10% (or fewer) of patients based on the physiologic measures of 5-minute Apgar, CDH Study Group (CDHSG) predicted survival, need for ECMO in the first 6 hours, and need for ECMO in the first 3 hours of life were studied. We also identified patients with best PaCO2 greater than 100 and best pH less than 7.0. A multivariate model (AUC-0.92) predicting mortality was also used to define the most severe 10%. RESULTS: Of 172 consecutive inborn patients, 18 had a 5-minute Apgar of 3 or less, and 11 survived (61%), 10 had a 5-minute Apgar of 2 or less, and 6 survived (60%), and 6 had a 5-minute Apgar of 1 or less, and 4 survived (67%). Seventeen had a CDHSG predicted survival less than 25%, and 9 survived (53%). Thirteen of 172 required ECMO for rescue in the first 6 hours of life, and 9 survived (69%), including 7 in the first 3 hours, and 5 survived (71%). Despite focused resuscitation in the delivery room and high levels of ventilatory support, 22 patients had a best PCO2 greater than 100 and best pH less than 7.0 for 1 hour or longer. Twelve of these 22 survived to discharge (55%). Of 17 defined by multivariate predictive modeling as the most severe, 8 survived (47%) with zero of the 3 ECMO ineligible prematures surviving. Of the 16 (10%) most severe ECMO-eligible patients, 10 of 16 survived (63%). All survivors were discharged home on no ventilatory support greater than nasal cannula oxygen. CONCLUSION: In newborn CDH patients without lethal associated anomalies, accepted measures of physiologic severity failed to predict mortality. Survival met or exceeded 50% even in the most severe 10% as defined by these measures. These data support the practice of treating each patient for survival regardless of the physiologic severity in the first hours of life, and selection criteria for not offering ECMO should be reevaluated where practiced.

Extracorporeal life support in patients with congenital diaphragmatic hernia: how long should we treat?

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a frequently lethal birth defect and, despite advances, extracorporeal life support (ie, extracorporeal membrane oxygenation [ECMO]) is commonly required for severely affected patients. Published data suggest that CDH survival after 2 weeks on ECMO is poor. Many centers limit duration of ECMO support. STUDY DESIGN: We conducted a single-institution retrospective review of 19 years of CDH patients treated with ECMO, designed to evaluate which factors affect survival and duration of ECMO and define how long patients should be supported. RESULTS: Of two hundred and forty consecutive CDH patients without lethal associated anomalies, 96 were treated with ECMO and 72 (75%) survived. Eighty required a single run of ECMO and 65 survived (81%), 16 required a second ECMO run and 7 survived (44%). Of patients still on ECMO at 2 weeks, 56% survived, at 3 weeks 46% survived, and at 4 weeks, 43% of patients still on ECMO survived to discharge. After 5 weeks of ECMO, survival had dropped to 15%, and after 40 days of ECMO support there were no survivors. Apgar score at 1 minute, Apgar score at 5 minutes, and Congenital Diaphragmatic Hernia Study Group predicted survival all correlated with survival on ECMO, need for second ECMO, and duration of ECMO. Lung-to-head ratio also correlated with duration of ECMO. All survivors were discharged breathing spontaneously with no support other than nasal cannula oxygen if needed. CONCLUSIONS: In patients with severe CDH, improvement in pulmonary function sufficient to wean from ECMO can take 4 weeks or longer, and might require a second ECMO run. Pulmonary outcomes in these CDH patients can still be excellent, and the assignment of arbitrary ECMO treatment durations <4 weeks should be avoided.

Long-term maturation of congenital diaphragmatic hernia treatment results: toward development of a severity-specific treatment algorithm.

OBJECTIVES: To assess the impact of varying approaches to congenital diaphragmatic hernia (CDH) repair timing on survival and need for ECMO when controlled for anatomic and physiologic disease severity in a large consecutive series of patients with CDH. BACKGROUND: Our publication of 60 consecutive patients with CDH in 1999 showed that survival was significantly improved by limiting lung inflation pressures and eliminating hyperventilation. METHODS: We retrospectively reviewed 268 consecutive patients with CDH, combining 208 new patients with the 60 previously reported. Management and ventilator strategy were highly consistent throughout. Varying approaches to surgical timing were applied as the series matured. RESULTS: Patients with anatomically less severe left liver-down CDH had significantly increased need for ECMO if repaired in the first 48 hours, whereas patients with more severe left liver-up CDH survived at a higher rate when repair was performed before ECMO. Overall survival of 268 patients was 78%. Survival was 88% for those without lethal associated anomalies. Of these, 99% of left liver-down CDH survived, 91% of right CDH survived, and 76% of left liver-up CDH survived. CONCLUSIONS: This study shows that patients with anatomically less severe CDH benefit from delayed surgery whereas patients with anatomically more severe CDH may benefit from a more aggressive surgical approach. These findings show that patients respond differently across the CDH anatomic severity spectrum and lay the foundation for the development of risk-specific treatment protocols for patients with CDH.

Twenty years of progress in congenital diaphragmatic hernia at the University of Florida.

Over the past 20 years the clinical paradigms underlying the care of children with congenital diaphragmatic hernia (CDH) have undergone profound changes. The purpose of this work is to provide an historic review of research and clinical studies related to CDH at the University of Florida (UF) and Shands Children's Hospital during the chairmanship of Edward M. Copeland, III, M.D. and to present our current clinical results. During Dr Copeland's tenure survival for newborns symptomatic with CDH treated at UF/Shands Children's Hospital has improved from less than 20 per cent to 85 per cent. Clinical observations have suggested and research studies at UF using a fetal lamb model have confirmed that fetal distress can occur late in gestation, which may predispose infants with CDH to pulmonary hypertension. However, our patient experience has confirmed that the most significant cause of mortality in human infants is not pulmonary hypertension but iatrogenic injury to their hypoplastic lungs. Strict avoidance of barotrauma in these babies has been the most important clinical advance during these two decades. Significant clinical and research problems remain including defining optimal prenatal care, management of complications during the first few weeks of life, and development of strategies to accelerate lung growth. Dramatic improvements in survival have resulted in children who manifest a number of clinical problems that were not evident when most of these patients died early in infancy. Our experience at Shand's Children's Hospital/UF indicates that feeding problems, respiratory infections, and management of subtle or overt neurologic complications may become major issues for some of these survivors and their families.

Small watercraft injuries in children.

The speed and use of small watercraft have increased dramatically in recent years. We report our experience with pediatric trauma resulting from small watercraft accidents. We conducted a retrospective chart review including all children admitted with injuries sustained in small watercraft accidents. Sixteen children were included; nine were injured in jet ski accidents and seven in accidents involving other craft. Jet ski accidents tended to result in more serious injuries (closed-head injuries, hollow and solid viscus injuries, chest trauma, spinal injuries leading to paralysis, and death) than those sustained in accidents with small boats. Skin and soft-tissue injuries and long-bone fractures were the most frequent injuries following accidents with other small boats. Six of eight children (75%) injured on jet skis required operative interventions. Only three of seven (43%) children in other watercraft accidents required surgery (P < 0.05 jet ski vs other watercraft). When compared with children injured in accidents involving small boats those involved in jet ski accidents tended to have more serious injuries and require operative intervention more frequently. A high index of suspicion for serious injuries must be maintained when evaluating children with this mechanism of injury.