A risk-stratified analysis of delayed congenital diaphragmatic hernia repair: Does timing of operation matter?

Hollinger, Laura E.; Lally, Pamela A.; Tsao, KuoJen; Wray, Curtis J.; Lally, Kevin P.

doi:10.1016/j.surg.2014.04.015

Cited by 46 publications

(24 citation statements)

References 24 publications

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“…We believe the central necessary components of CDH care to achieve maximal survival are strict adherence to lung protective ventilation[5,18,19] [20], repair of CDH [12,21], inclusive use of ECMO for more severe CDH as needed until lung recovery[10], and belief that the CDH infant can survive. Not all centers will achieve these results, but we hope these data will serve as a cautionary note for decisions to withhold treatments such as ECMO or CDH repair, as only by treating for survival will maximal survival be achieved.…”

Section: Discussionmentioning

confidence: 99%

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

Kays¹,

Islam²,

Perkins³

et al. 2015

Journal of Pediatric Surgery

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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 model 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 re-evaluated where practiced.

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Section: Discussionmentioning

confidence: 99%

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

Kays¹,

Islam²,

Perkins³

et al. 2015

Journal of Pediatric Surgery

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“…However, the individual heterogeneity in these effects makes it difficult to accurately predict outcomes [3,4]. Patient stratification according to CDH severity is not always reported [5,6,7]. The overall prenatal detection rate of CDH with ultrasound is approximately 60%.…”

Section: Introductionmentioning

confidence: 99%

Differences in Outcomes in Prenatally Diagnosed Congenital Diaphragmatic Hernia Compared to Postnatal Detection: A Single-Center Experience

Burgos¹,

Hammarqvist-Vejde

Frenckner³

et al. 2015

Fetal Diagn Ther

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Objectives: To compare outcomes in pregnancies with a prenatal detection of congenital diaphragmatic hernia (CDH) with children diagnosed after birth, treated at the same institution, and to determine the ability to predict prognosis through measurements of the observed to expected lung-to-head ratio (O/E LHR). Methods: This is a retrospective review of all children with CDH treated at our institution during 2006-2014. We compared outcomes of infants referred for surgery after postnatal diagnosis with outcomes of infants with prenatally diagnosed CDH. Results: In the prenatal group, O/E LHR was significantly different between survivors and deceased patients, with a cutoff at 35% O/E LHR. Survival to discharge and 1-year survival were significantly higher in the postnatal group that required intubation within 24 h; i.e., 92 and 89% versus 85 and 73% in the prenatal group (p < 0.05). There was less need for extracorporeal membrane oxygenation (ECMO), 41 versus 60%, and patch, 41 versus 75% (p < 0.001), in the postnatal group with early diagnosis compared with the prenatal group, respectively. Conclusion: Children with prenatally diagnosed CDH represent a population with a more severe condition compared to infants diagnosed after birth. They have poorer outcomes with higher needs for ECMO or use of patch, and lower survival rates were observed at an O/E LHR below 35%.

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“…Clinical manifestations are diverse, varying from asymptomatic to life-threatening respiratory distress. Overall, it is thought to have a relatively good outcome, but exact numbers are not described [7], because most studies do not specify this subtype as a specific entity [2,[8][9][10][11][12].…”

Section: Introductionmentioning

confidence: 99%

Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study

et al. 2020

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Congenital diaphragmatic eventration (CDE) and congenital diaphragmatic hernia (CDH) with or without hernia sac are three different types of congenital diaphragmatic malformations, which this study evaluates. All surgically treated patients with CDE or Bochdalek type CDH between 2000 and 2016 were included in this retrospective analysis. Demographics, CDH-characteristics, treatment, and clinical outcome were evaluated. In total, 200 patients were included. Patients with an eventration or hernia sac had no significant differences and were compared as patients without a true defect to patients with a true defect. The 1-year survival of patients with a true defect was significantly lower than patients with no true defect (76% versus 97%, p = 0.001). CDH with no true defect had significantly better short-term outcomes than CDH with true defect requiring patch repair. However, at 30 days, they more often required oxygen supplementation (46% versus 26%, p = 0.03) and had a higher recurrence rate (8% versus 0%, p = 0.006) (three eventration and two hernia sac patients). Conclusion: Patients without a true defect seem to have a more similar clinical outcome than CDH patients with a true defect, with a better survival. However, the recurrence rate and duration of oxygen supplementation at 30 days are higher than CDH patients with a true defect. What is Known:• Congenital diaphragmatic hernia with or without hernia sac and congenital diaphragmatic eventration (incomplete muscularization) are often treated similarly. • Patients with hernia sac and eventration are thought to have a relatively good outcome, but exact numbers are not described. What is New: • Congenital diaphragmatic eventration and patients with hernia sac seem to have a more similar clinical outcome than Bochdalek type CDH with a true defect. • Patients without a true defect (eventration or hernia sac) have a high recurrence rate.

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A risk-stratified analysis of delayed congenital diaphragmatic hernia repair: Does timing of operation matter?

Cited by 46 publications

References 24 publications

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

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

Differences in Outcomes in Prenatally Diagnosed Congenital Diaphragmatic Hernia Compared to Postnatal Detection: A Single-Center Experience

Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study

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