Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus

Reiss, Ikm; Schaible, Thomas; Hout, L. van den; Capolupo, Irma; Allegaert, Karel; Heijst, Arno van; Silva, M. Gorett; Greenough, Anne; Tibboel, Dick

doi:10.1159/000320622

Cited by 279 publications

(254 citation statements)

References 145 publications

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“…1 The overall survival of infants with this condition is highly variable, [2][3][4][5][6] as is their care, although the standardization of postnatal care has evolved over the years. 7 This is illustrated by the dramatic differences in survival between infants undergoing patch repair compared with those amenable to primary repair. 8,9 A method to identify these highand low-risk infants in the postnatal period but early in the course of their disease would offer the opportunity to tailor therapies based on severity.…”

mentioning

confidence: 99%

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

et al. 2014

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BACKGROUND: Congenital diaphragmatic hernia (CDH) is a condition with a highly variable outcome. Some infants have a relatively mild disease process, whereas others have significant pulmonary hypoplasia and hypertension. Identifying high-risk infants postnatally may allow for targeted therapy. METHODS: Data were obtained on 2202 infants from the Congenital Diaphragmatic Hernia Study Group database from January 2007 to October 2011. Using binary baseline predictors generated from birth weight, 5-minute Apgar score, congenital heart anomalies, and chromosome anomalies, as well as echocardiographic evidence of pulmonary hypertension, a clinical prediction rule was developed on a randomly selected subset of the data by using a backward selection algorithm. An integer-based clinical prediction rule was created. The performance of the model was validated by using the remaining data in terms of calibration and discrimination. RESULTS: The final model included the following predictors: very low birth weight, absent or low 5-minute Apgar score, presence of chromosomal or major cardiac anomaly, and suprasystemic pulmonary hypertension. This model discriminated between a population at high risk of death (∼50%) intermediate risk (∼20%), or low risk (<10%). The model performed well, with a C statistic of 0.806 in the derivation set and 0.769 in the validation set and good calibration (Hosmer-Lemeshow test, P = .2). CONCLUSIONS: A simple, generalizable scoring system was developed for CDH that can be calculated rapidly at the bedside. Using this model, intermediate- and high-risk infants could be selected for transfer to high-volume centers while infants at highest risk could be considered for advanced medical therapies.

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confidence: 99%

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

et al. 2014

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“…When neonatal ECMO therapy was required, informed parental consent was obtained. ECMO therapy was administered according to previously published guidelines (12,13). In all patients, veno-arterial ECMO therapy was performed via cannulation of the rCCA and the right internal jugular vein.…”

Section: Methodsmentioning

confidence: 99%

Cerebral Perfusion After Repair of Congenital Diaphragmatic Hernia with Common Carotid Artery Occlusion After ECMO Therapy

Henzler

Zöllner

Weis

et al. 2017

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Abstract. Aim: To prospectively evaluate cerebral perfusion after repair of congenital diaphragmatic hernia (CDH) and right-common-carotid-artery (rCCA) Congenital diaphragmatic hernia (CDH) occurs in approximately one in 3,000-4,000 live births (1). A defect of the diaphragm leads to herniation of abdominal organs into the thoracic cavity. Lung hypoplasia and secondary pulmonary hypertension are the major causes of death (2). The implementation of new therapeutic strategies has improved the survival rate even in those patients with severe CDH (3). If conservative treatment in newborns with CDH fails, extracorporeal membrane oxygenation (ECMO) therapy is an important alternative for accomplishing sufficient oxygenation of the newborns. The Congenital Diaphragmatic Hernia Study Group showed an improved survival rate in patients with CDH after ECMO therapy, in whom, without ECMO, mortality was predicted to be higher than 80% (4). As a consequence, morbidity plays an increasing role in patients with CDH. Not only pulmonary problems but also neurological disorders have been identified as significant morbidities among CDH survivors (5, 6). In a study by McGahren et al., 8/12 (67%) ECMO survivors showed delayed neurological development compared to only 5/21(24%) of the non-ECMO-treated survivors.In CDH, veno-arterial ECMO is usually performed. Vascular access is usually achieved by insertion of the ECMO cannulas through the right common carotid artery (rCCA) and the right internal jugular vein. At the time of decannulation, ligation or surgical reconstruction of the rCCA can be performed but secondary occlusion of the surgical reconstructed rCCA can occur (7). Büsing et al. investigated 2-year-old children with history of CDH and reconstruction of the rCCA after ECMO therapy. Their study showed that in 72% of the patients, the rCCA was found to be occluded or highly stenotic using contrast-enhanced magnetic resonance angiography (7). Using Doppler ultrasound in 5-year-old patients, Desai et al. reported that most of the reconstructed rCCAs after ECMO therapy were patent and in only 24% of the patients there was significant 557

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“…In preparing the current guideline, the panel reviewed existing guidelines published by the CDH EURO Consortium (CDH EURO) 8,9 and the American Heart Association and American Thoracic Society. 10 We used pre-established consensus criteria to accept, modify or reject these baseline recommendations in the guideline-finalization process described below.…”

Section: Guideline Developmentmentioning

confidence: 99%

Diagnosis and management of congenital diaphragmatic hernia: a clinical practice guideline

Puligandla¹,

Skarsgard²,

Offringa³

et al. 2018

CMAJ

186

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Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus

Cited by 279 publications

References 145 publications

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

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

Cerebral Perfusion After Repair of Congenital Diaphragmatic Hernia with Common Carotid Artery Occlusion After ECMO Therapy

Diagnosis and management of congenital diaphragmatic hernia: a clinical practice guideline

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