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Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies
J W Logan1, H E Rice, R N Goldberg
1Division of Neonatology, Department of Pediatrics, Duke University Medical Center, Durham, NC, USA. wellslogan@usa.net
Insights
Strategies like minimizing lung injury and using extracorporeal membrane oxygenation (ECMO) criteria improve survival for infants with congenital diaphragmatic hernia (CDH). Further research is needed for long-term outcomes.
Area of Science:
- Neonatal Medicine
- Pediatric Surgery
- Critical Care
Background:
- Congenital diaphragmatic hernia (CDH) is a serious condition affecting infant survival.
- Recent advancements in care strategies show promise for improving outcomes in CDH infants.
Purpose of the Study:
- To review and detail the care strategies employed by centers reporting high survival rates for infants with congenital diaphragmatic hernia (CDH).
- To identify key interventions contributing to successful management of CDH.
Main Methods:
- A systematic literature search was conducted using MEDLINE and the Cochrane Central Register of Controlled Trials (1995-2006).
- Studies included reports of over 20 infants with symptomatic CDH and a survival rate exceeding 75% for isolated CDH.
Main Results:
- Thirteen reports from 11 centers met the inclusion criteria, with an overall survival rate of 79% (603/763) and 85% for isolated CDH (560/661).
- Extracorporeal membrane oxygenation (ECMO) use varied (11-61%), with a 73% survival rate for isolated CDH infants on ECMO.
- No significant benefit was observed from antenatal glucocorticoids (after 34 weeks) or postnatal surfactant. Minimizing lung injury and adhering to center-specific ECMO criteria were key factors in low mortality.
Conclusions:
- Successful management of CDH involves strategies focused on minimizing lung injury, tolerating acidosis/hypoxemia, and using center-specific ECMO criteria.
- The current literature lacks randomized clinical trials for CDH care strategies.
- Prospective studies are essential to evaluate the safety and long-term outcomes of these interventions in CDH patients.
Objectives:
Recent reports suggest that specific care strategies improve survival of infants with congenital diaphragmatic hernia (CDH). This review presents details of care from centers reporting high rates of survival among CDH infants.
Study Design:
We conducted a MEDLINE search (1995 to 2006) and searched all citations in the Cochrane Central Register of Controlled Trials. Studies were included if they contained reports of >20 infants with symptomatic CDH, and >75% survival of isolated CDH.
Result:
Thirteen reports from 11 centers met inclusion criteria. Overall survival, including infants with multiple anomalies, was 603/763 (79%; range: 69 to 93%). Survival for isolated CDH was 560/661 (85%; range: 78 to 96%). The frequency of extracorporeal membrane oxygenation (ECMO) use for isolated CDH varied widely among reporting centers 251/622 (40%; range: 11 to 61%), as did survival for infants with isolated CDH placed on ECMO: 149/206 (73%; range: 33 to 86%). There was no suggestion of benefit from use of antenatal glucocorticoids given after 34 weeks gestation or use of postnatal surfactant. Low mortality was frequently attributed to minimizing lung injury and adhering to center-specific criteria for ECMO.
Conclusion:
Use of strategies aimed at minimizing lung injury, tolerance of postductal acidosis and hypoxemia, and adhering to center-specific criteria for ECMO were strategies most consistently reported by successful centers. The literature lacks randomized clinical trials of these or other care strategies in this complex patient population; prospective studies of safety and long-term outcome are needed.
