Survival rate in congenital diaphragmatic hernia: the experience of the canadian neonatal network

Javid, Patrick J.; Jaksic, Tom; Skarsgard, Erik D.; Lee, Shoo

doi:10.1016/j.jpedsurg.2004.01.022

Cited by 160 publications

(99 citation statements)

References 11 publications

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“…Centralized management, with increased case load and using consistent neonatal protocols leads to increased survival rates. This was demonstrated by the Canadian Neonatal Network that showed that high volume centers (>12 CDH admissions over the 22 months period) had 13% higher survival rate than low volume centers (Javid et al, 2004).…”

Section: Neonatal Management and The Difficulty Of Assessing Exact Sumentioning

confidence: 95%

Prenatal diagnosis, prediction of outcome and in utero therapy of isolated congenital diaphragmatic hernia

et al. 2008

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Congenital diaphragmatic hernia (CDH) can be associated with genetic or structural anomalies with poor prognosis. In isolated cases, survival is dependent on the degree of lung hypoplasia and liver position. Cases should be referred in utero to tertiary care centers familiar with this condition both for prediction of outcome as well as timed delivery. The best validated prognostic indicator is the lung area to head circumference ratio. Ultrasound is used to measure the lung area of the index case, which is then expressed as a proportion of what is expected normally (observed/expected LHR). When O/E LHR is <25% survival chances are <15%. Prenatal intervention, aiming to stimulate lung growth, can be achieved by temporary fetal endoscopic tracheal occlusion (FETO). A balloon is percutaneously inserted into the trachea at 26-28 weeks, and reversal of occlusion is planned at 34 weeks. Growing experience has demonstrated the feasibility and safety of the technique with a survival rate of about 50%. The lung response to, and outcome after FETO, is dependent on pre-existing lung size as well gestational age at birth. Early data show that FETO does not increase morbidity in survivors, when compared to historical controls. Several trials are currently under design.

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Section: Neonatal Management and The Difficulty Of Assessing Exact Sumentioning

confidence: 95%

Prenatal diagnosis, prediction of outcome and in utero therapy of isolated congenital diaphragmatic hernia

et al. 2008

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“…Survivors were operated on at a median age of 2.0 (range, [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] days; 77% of them required a patch augmented repair. Full enteral feeding was possible at 26 (range, 5-150) days of age.…”

Section: Postnatal Morbidity Indices and Their Predictors In The Fetomentioning

confidence: 99%

Predictors of neonatal morbidity in fetuses with severe isolated congenital diaphragmatic hernia undergoing fetoscopic tracheal occlusion

Doné

Gratacós

Nicolaides

et al. 2013

Ultrasound in Obstet & Gyne

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“…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 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.…”

Section: Introductionmentioning

confidence: 99%

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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Survival rate in congenital diaphragmatic hernia: the experience of the canadian neonatal network

Cited by 160 publications

References 11 publications

Prenatal diagnosis, prediction of outcome and in utero therapy of isolated congenital diaphragmatic hernia

Prenatal diagnosis, prediction of outcome and in utero therapy of isolated congenital diaphragmatic hernia

Predictors of neonatal morbidity in fetuses with severe isolated congenital diaphragmatic hernia undergoing fetoscopic tracheal occlusion

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

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