Congenital diaphragmatic hernia. Stabilization and repair on ECMO
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K P Lally1, M S Paranka, J Roden
1Department of Surgery, Wilford Hall USAF Medical Center, San Antonio, Texas.
Insights
Extracorporeal membrane oxygenation (ECMO) can help high-risk infants with congenital diaphragmatic hernia (CDH) survive surgery. However, survivors often need long-term care for complications like recurrent hernias and reflux.
Area of Science:
- Pediatric Surgery
- Neonatal Intensive Care
- Cardiopulmonary Support
Background:
- Congenital diaphragmatic hernia (CDH) is a severe condition requiring advanced respiratory support.
- Delayed surgical repair of CDH, with extracorporeal membrane oxygenation (ECMO) as a bridge, is an emerging strategy.
- Limited data exists on outcomes for infants undergoing CDH repair while on ECMO.
Purpose of the Study:
- To review the outcomes of infants with CDH who underwent surgical repair while supported by ECMO.
- To identify risk factors for mortality and long-term complications in this high-risk population.
Main Methods:
- Retrospective review of 42 infants with symptomatic CDH repaired on ECMO across five centers.
- Analysis of pre-ECMO oxygenation (PaO2) and carbon dioxide (PaCO2) levels, gestational age, need for intraoperative interventions, and long-term follow-up data.
Main Results:
- Overall survival rate was 43% (18 of 42 infants).
- Prematurity (<37 weeks) and significant intraoperative hemorrhage requiring reoperation were associated with poor outcomes.
- Fifty percent of survivors experienced recurrent herniation, and 43% had significant gastroesophageal reflux.
Conclusions:
- Preoperative ECMO stabilization and intra-ECMO repair can enable survival for some extremely high-risk CDH infants.
- Survivors require vigilant long-term follow-up due to high rates of recurrent CDH and gastroesophageal reflux, often necessitating further surgical intervention.
Abstract:
Availability of extracorporeal membrane oxygenation (ECMO) support and the potential advantages of delayed repair of congenital diaphragmatic hernia (CDH) have led several centers to delay CDH repair, using ECMO support if necessary. This study reviews the combined experience of five ECMO centers with infants who underwent stabilization with ECMO and repair of CDH while still on ECMO. All infants were symptomatic at birth, with a mean arterial oxygen pressure (PaO2) of 34 mmHg on institution of bypass despite maximal ventilatory support. A total of 42 infants were repaired on ECMO, with 18 (43%) surviving. Seven infants had total absence of the diaphragm, and 28 required a prosthetic patch to close the defect. Only five infants ever achieved a best postductal PaO2 over 100 mmHg before institution of ECMO. Prematurity was a significant risk factor, with no infants younger than 37 weeks of age surviving. Significant hemorrhage on bypass was also a hallmark of a poor outcome, with 10 of the 24 nonsurvivors requiring five thoracotomies and six laparotomies to control bleeding, whereas only one survivor required a thoracotomy to control bleeding. In follow-up, nine of the 18 survivors (50%) have developed recurrent herniation and seven (43%) have significant gastroesophageal reflux. Importantly, five of the 18 survivors were in the extremely high-risk group who never achieved a PaO2 over 100 mmHg or an arterial carbon dioxide pressure (PaCO2) less than 40 mmHg before the institution of ECMO. In conclusion, preoperative stabilization with ECMO and repair on bypass may allow some high-risk infants to survive. Surviving infants will require long-term follow-up because many will require secondary operations.