Standardized reporting for congenital diaphragmatic hernia – An international consensus

Lally, Kevin P.; Lasky, Robert E.; Lally, Pamela A.; Bagolan, Pietro; Davis, Carl; Frenckner, Björn; Hirschl, Ronald; Langham, Max R.; Buchmiller, Terry L.; Usui, Noriaki; Tibboel, Dick; Wilson, Jay M.

doi:10.1016/j.jpedsurg.2013.08.014

Cited by 260 publications

(184 citation statements)

References 28 publications

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“…A 2D echocardiography performed within the first 24 h after birth remains the best modality to (1) rule out the presence of cardiac anomalies; (2) assess the right heart function, and (3) determine the amount of pulmonary hypertension classified accordingly (less or more than 2/3 systemic blood pressure) [37,38]. Especially in severe cases of pulmonary hypertension, a cardiac ultrasound may help to evaluate right ventricular dysfunction and/or right ventricular overload, which condition can also lead to left ventricular dysfunction [39].…”

Section: Resultsmentioning

confidence: 99%

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Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus - 2015 Update

et al. 2016

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In 2010, the congenital diaphragmatic hernia (CDH) EURO Consortium published a standardized neonatal treatment protocol. Five years later, the number of participating centers has been raised from 13 to 22. In this article the relevant literature is updated, and consensus has been reached between the members of the CDH EURO Consortium. Key updated recommendations are: (1) planned delivery after a gestational age of 39 weeks in a high-volume tertiary center; (2) neuromuscular blocking agents to be avoided during initial treatment in the delivery room; (3) adapt treatment to reach a preductal saturation of between 80 and 95% and postductal saturation >70%; (4) target PaCO₂ to be between 50 and 70 mm Hg; (5) conventional mechanical ventilation to be the optimal initial ventilation strategy, and (6) intravenous sildenafil to be considered in CDH patients with severe pulmonary hypertension. This article represents the current opinion of all consortium members in Europe for the optimal neonatal treatment of CDH.

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Section: Resultsmentioning

confidence: 99%

“…Recently, Costerus et al [60] concluded that thoracoscopic primary closure seems a safe and effective procedure, but efficacy of thoracoscopic patch repair has not been established. To allow for better comparison of patient groups between studies it is recommended to record the diaphragmatic defect size in all surgeries [37]. …”

Section: Resultsmentioning

confidence: 99%

Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus - 2015 Update

et al. 2016

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“…Surgical parameters included defect size (grades A-D), patch repair, and the need for re-do surgery [11]. …”

Section: Methodsmentioning

confidence: 99%

Clinical Outcome for Congenital Diaphragmatic Hernia at the Age of 1 Year in the Era of Fetal Intervention

Ginderdeuren

Allegaert²,

Decaluwé³

et al. 2017

Neonatology

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Background: Congenital diaphragmatic hernia (CDH) is an abnormal development of the diaphragm leading to high neonatal mortality and morbidity. Beyond the neonatal period, prospective data on overall long-term outcome in CDH survivors is scarce. In particular, for those treated with fetoscopic endoluminal tracheal occlusion (FETO), a promising new technique to increase survival chances for severe cases, the outcomes are even less documented. Aim: To prospectively document the clinical outcome of CDH at 1 year including FETO-treated infants in relation to ante- and postnatal variables. Methods: A single-centre prospective clinical follow-up at 1 year of age included a standardised respiratory questionnaire. Results: Thirty-four CDH survivors were included, 10 of which were FETO-treated infants. At 1 year, the median weight z score was -1.50 (interquartile range [IQR]: -2.45 to -0.88) and 10/34 were “failure to thrive” (FTT) cases. Gastro-intestinal (GI) problems at 1 year were mainly related to gastro-oesophageal reflux (16/34) with a high need for tube feeding in the first year (9/34). Tachypnoea was present in 10/29 (34%) and 8/34 (24%) were on chronic inhaled medication. The median total respiratory symptom score was 20 (IQR: 13-32) and correlated with the number of neonatal ventilation days (p = 0.048). Hospital re-admission occurred in 19/34 infants (56%), and was for respiratory problems in almost half of them (8/19). Conclusion: In this CDH cohort, morbidity at 1 year was determined more by GI problems and FTT than respiratory morbidity. The respiratory questionnaire may, however, have underestimated the respiratory morbidity since respondents had a 1-in-4 chance to have persistent tachypnoea and be on chronic inhalation therapy.

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“…Congenital diaphragmatic hernia (CDH) is a common congenital anomaly that occurs in 1 in 3,500 to 5,000 live births (1,2). Despite improvements in the care of infants with CDH, the mortality rate associated with the disease continues to be 20% in isolated cases and 50% in complex cases (3)(4)(5). The primary cause of the high mortality rate is a lethal combination of pulmonary hypoplasia and pulmonary hypertension (PH) (6,7).…”

Section: Introductionmentioning

confidence: 99%

PBX transcription factors drive pulmonary vascular adaptation to birth

McCulley¹,

Wienhold²,

Hines³

et al. 2017

Journal of Clinical Investigation

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Standardized reporting for congenital diaphragmatic hernia – An international consensus

Cited by 260 publications

References 28 publications

Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus - 2015 Update

Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus - 2015 Update

Clinical Outcome for Congenital Diaphragmatic Hernia at the Age of 1 Year in the Era of Fetal Intervention

PBX transcription factors drive pulmonary vascular adaptation to birth

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