Analysis of an improved survival rate for congenital diaphragmatic hernia

Downard, Cynthia D.; Jaksic, Tom; Garza, Jennifer J.; Dzakovic, Alexander; Nemes, Luanne P.; Jennings, Russell W.; Wilson, Jay M.

doi:10.1016/jpsu.2003.50194

Cited by 251 publications

(191 citation statements)

References 10 publications

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“…While there is no overlap in the reports from the Boston group, there is a small overlap in the outcomes reports from the Columbia group (August 1992 through December 1995). 15,17,25,26 This overlap was not considered in the analysis, as data could not be appropriately extracted from the reports and omission of either or both of the reports seemed inappropriate.…”

Section: Resultsmentioning

confidence: 99%

“…Survival for isolated CDH for the successful centers was 560/661 (85%; range: 78 to 96%). [15][16][17][18][19][20][22][23][24][25]27 Reported survival for isolated CDH using conventional medical management, excluding ECMO but including high-frequency ventilation was 331/367 (90%; range: 83 to 100%). The frequency of ECMO use for isolated CDH was 251/622 (40%; range: 11 to 61%), and overall survival for infants with isolated CDH placed on ECMO was 149/206 (73%; range: 33 to 86%; Table 1).…”

Section: Survivalmentioning

confidence: 99%

“…Most of the successful centers adopted a strategy of 'gentle ventilation' or 'permissive hypercapnia'. [15][16][17][18][19][20][21][22][23][24][25] Mortality was generally attributed to the presence of additional anomalies, iatrogenic lung injury, severe pulmonary hypoplasia and/or PPHN, and bleeding complications (among ECMO-treated infants). One report suggested that after excluding CDH infants with additional anomalies, 'hopeless pulmonary hypoplasia' occurred in no more than 15% of isolated CDH infants.…”

Section: Survivalmentioning

confidence: 99%

“…Among centers reporting antenatal care, 354/630 CDH infants (56%) were prenatally diagnosed, and 247 of 763 infants (33%) were inborn. [15][16][17][18][20][21][22][23]25,27 Reports varied on whether or not prenatal diagnosis or site of delivery was reported or associated with improved survival, but the combined reports suggest that delivery at a tertiary center was beneficial. For most centers, survival increased for both inborns and prenatally diagnosed infants in the most recent epoch of care.…”

Section: Survivalmentioning

confidence: 99%

“…4 Literature review reveals a limited number of controlled clinical trials examining interventions that improve survival of infants with CDH; [5][6][7][8][9][10][11][12][13][14] however, several centers reporting relatively high rates of survival for CDH have described their management strategies in varying detail. [15][16][17][18][19][20][21][22][23][24][25] The objective of this report is to provide clinicians with a summary of specific details of clinical care strategies from these 'benchmark' centers. In the absence of clinical trials, this summary of practice strategies provides a starting point for development of an approach to care for infants with CDH.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2007

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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...

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Section: Resultsmentioning

confidence: 99%