Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies

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 Introduction Congenital diaphragmatic hernia (CDH) is a rare malformation observed in approximately 1 in 3000 live births, with estimates of postnatal survival ranging from 50 to 70% despite advances in neonatal care. 1 The combination of pathophysiologic derangements associated with CDH, including lung hypoplasia, lung dysmaturity and pulmonary hypertension (PPHN), requires a multidisciplinary approach to care. 2,3 The complex nature of clinical management and the relatively infrequent presentation of infants with this rare malformation to single centers has lead to difficulty identifying consistent strategies for CDH management. A recent report from the Canadian Neonatal Research Network indicates better than predicted survival in Network centers, with significant improvement in the higher mortality odds strata, and 90% survival in the three centers that cared for at least 12 infants over a 22-month period. Details of management strategies are not provided, but the report suggests that survival is best achieved by caring for CDH infants at centers that have developed a standardized approach to care, with adequate numbers of CDH infants to develop expertise. 4 Literature review reveals a limited number of controlled clinical trials examining inter...

Mortality in infants with congenital diaphragmatic hernia: a study of the United States National Database

“…8,16 Our data show no statistical difference in mortality, when comparing a group of babies operated before 3 days of life or between the ages of 3 to 7 days of life. However, when babies were operated on after 7 days of life, their mortality and use of ECMO increased significantly.…”

A Clinical Prediction Rule for the Severity of Congenital Diaphragmatic Hernias in Newborns

“…Increasingly, there is a recognition that centers with larger volumes may have improved outcomes in CDH. 2,[22][23][24] The burden on the family and health care system that would be involved in the transfer of all patients with CDH to high-volume centers could be diminished through identification of a population with a lower risk of mortality that can be cared for in smaller volume institutions; those at higher risk could then be considered for transport to those centers with greater expertise. This practice is already accepted in many European countries.…”

Inhaled Nitric Oxide Use in Neonates With Congenital Diaphragmatic Hernia

“…Initial treatment of hypoxemic respiratory failure in newborns with CDH is generally consistent with management of persistent pulmonary hypertension of the newborn, and includes sedation, permissive hypercapnia, highfrequency oscillatory ventilation, and the avoidance of acidosis and barotrauma. 2,3 When these modalities fail, neonates with CDH may be supported with extracorporeal membrane oxygenation (ECMO), which has been shown to improve survival. 4,5 Nitric oxide, an important endogenous mediator of vascular tone, causes vascular smooth muscle relaxation and subsequent vasodilatation.…”