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Delayed surgical repair and ECMO improves survival in congenital diaphragmatic hernia
K W West1, K Bengston, F J Rescorla
1Department of Surgery, Indiana University School of Medicine, Indianapolis. Indiana.
Insights
Early stabilization and delayed surgical repair, combined with extracorporeal membrane oxygenation (ECMO), significantly improve survival rates for infants with congenital diaphragmatic hernia (CDH). This approach is particularly beneficial for high-risk neonates requiring prosthetic diaphragmatic patches.
Area of Science:
- Pediatric Surgery
- Neonatology
- Critical Care Medicine
Background:
- Congenital diaphragmatic hernia (CDH) frequently causes life-threatening respiratory distress in newborns.
- Survival rates for infants with CDH have historically been poor, especially those requiring prosthetic patches.
- The pre-extracorporeal membrane oxygenation (ECMO) era showed limited success with immediate surgical repair.
Purpose of the Study:
- To evaluate the impact of early stabilization, delayed surgical repair, and ECMO on survival outcomes in infants with CDH.
- To compare survival rates between the pre-ECMO era and the post-ECMO era for CDH treatment.
- To assess the effectiveness of ECMO in managing severe respiratory failure associated with CDH.
Main Methods:
- Retrospective analysis of 110 infants with CDH.
- Comparison of outcomes between infants treated before 1987 (pre-ECMO) and after 1987 (post-ECMO).
- Inclusion of infants undergoing immediate repair versus delayed repair with or without ECMO support.
- Analysis of specific subgroups, including those requiring prosthetic diaphragmatic patches and those needing ECMO before repair.
Main Results:
- Survival rate improved from 43% in the pre-ECMO era to 67.4% in the post-ECMO era.
- Infants requiring prosthetic patches had a significantly higher survival rate in the ECMO era (81.8% vs. 12.5%).
- Delayed repair combined with ECMO resulted in an overall survival rate of 80% at 3 months.
- ECMO was crucial for stabilizing infants with severe respiratory failure (oxygenation index > 40), with a 77.8% survival rate in this group.
Conclusions:
- Early stabilization, delayed surgical repair, and the use of ECMO are critical for improving survival in high-risk CDH infants.
- ECMO effectively reverses early mortality associated with pulmonary hypertension in CDH.
- The combined strategy offers a significant survival advantage, particularly for neonates requiring prosthetic diaphragmatic repair.
Abstract:
One hundred ten infants with congenital diaphragmatic hernia (CDH) developed life-threatening respiratory distress in the first 6 hours of life. Associated anomalies were present in 33%. Twenty-eight of 65 infants (43%) treated before 1987 (pre-extracorporeal membrane oxygenation [ECMO] era) survived after immediate CDH repair, and mechanical ventilation with or without pharmacologic support. Only two of 16 (12.5%) infants requiring a prosthetic diaphragmatic patch survived. Since 1987, 31 of 46 (67.4%) infants with birth weight, gestational age, and severity of illness similar to the pre-1987 group survived. All patients were immediately intubated and ventilated. Seven (four with lethal chromosomal anomalies) infants died before treatment, and 30 stabilized (partial pressure of carbon dioxide [PCO2] < 50; partial pressure of oxygen [PO2] > 100; pH > 7.3) and underwent delayed CDH repair at 5 to 72 hours. Fifteen did well on conventional support and survived. Fifteen infants deteriorated after operation: 11 were placed on ECMO with eight survivors, and four infants were not considered ECMO candidates. Nine babies failed to stabilize initially and were placed on ECMO before CDH repair (alveolar-arterial gradient > 600 and oxygenation index > 40), and seven survived. The overall survival rate was 80% at 3 months in this ECMO-treated group. Early mortality was due to inability to wean from ECMO (one), intracranial hemorrhage (one), liver injury (one), and pulmonary hypoplasia (one). Nine of 11 babies requiring a prosthetic patch in the post-1987 ECMO group survived (81.8%). There were three late post-ECMO deaths (3 to 18 months) of right heart failure (two) and sepsis (one). Symptomatic gastroesophageal reflux occurred in nine cases, six requiring a fundoplication in the bypass babies. Recurrent diaphragmatic hernia occurred in nine cases (five ECMO). The overall survival rate was significantly improved in the delayed repair/ECMO group (67% versus 43%; p < 0.05) and was most noticeable in infants requiring a prosthetic diaphragm (81.2% versus 12.5%; p < 0.005). These data indicate that early stabilization, delayed repair, and ECMO improve survival in high-risk CDH. Early deaths are related to pulmonary hypertension and can be reversed by ECMO.