State-of-the-Art
Journal of Perinatology (2007) 27, 535–549; doi:10.1038/sj.jp.7211794; published online 19 July 2007
Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies
J W Logan1, H E Rice2, R N Goldberg1 and C M Cotten1
- 1Division of Neonatology, Department of Pediatrics, Duke University Medical Center, Durham, NC, USA
- 2Department of Surgery, Duke University Medical Center, Durham, NC, USA
Correspondence: Dr JW Logan, Brody School of Medicine, Division of Neonatal-Perinatal Medicine, East Carolina University, 600 Moye Blvd, Greenville, NC 27858, USA. E-mail: wellslogan@usa.net
Received 18 March 2007; Revised 18 June 2007; Accepted 19 June 2007; Published online 19 July 2007.
Abstract
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.
Keywords:
congenital diaphragmatic hernia, pulmonary hypertension, pulmonary hypoplasia, inhaled nitric oxide, extracorporeal membrane oxygenation, systematic review
