Congenital diaphragmatic hernia (CDH) is associated with high mortality and morbidity. To date, there are no standardized protocols for the treatment of infants with this anomaly. However, protocols based on the literature and expert opinion might improve outcome. This paper is a consensus statement from the CDH EURO Consortium prepared with the aim of achieving standardized postnatal treatment in European countries. During a consensus meeting between high-volume centers with expertise in the treatment of CDH in Europe (CDH EURO Consortium), the most recent literature on CDH was discussed. Thereafter, 5 experts graded the studies according to the Scottish Intercollegiate Guidelines Network (SIGN) Criteria. Differences in opinion were discussed until full consensus was reached. The final consensus statement, therefore, represents the opinion of all consortium members. Multicenter randomized controlled trials on CDH are lacking. Use of a standardized protocol, however, may contribute to more valid comparisons of patient data in multicenter studies and identification of areas for further research.

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

Objective To assess the value of antenatally determined observed to expected fetal lung area to head circumference ratio (LHR) in the prediction of postnatal survival in isolated, congenital diaphragmatic hernia (CDH). Methods Two groups of fetuses were examined. The first group included 650 normal fetuses at 12–32 weeks’ gestation, and the data collected were used to establish a normal range of observed to expected LHR with gestational age. The second group included the data of a retrospective multicenter study of 354 fetuses with isolated CDH in which the LHR was measured on one occasion at 18–38 weeks’ gestation. The patients were divided into those with left-sided CDH with and without intrathoracic herniation of the liver and right-sided CDH. Regression analysis was used to determine the significant predictors of postnatal survival. Results In both the normal fetuses and those with CDH the LHR increased but the observed to expected LHR did not change significantly with gestational age. In normal fetuses the mean observed to expected LHR in the left lung was 100% (95% CI, 61–139%) and in the right lung it was 100% (95% CI, 67–133%). In fetuses with CDH the mean observed to expected LHR was 39% (range 7–79%). Regression analysis demonstrated that significant predictors of survival were the observed to expected LHR (odds ratio (OR) 1.09, 95% CI, 1.06–1.12), side of CDH (left side OR 11.14, 95% CI, 3.41–36.39) and gestational age at delivery (OR 1.18, 95% CI, 1.02–1.36).

https://obgyn.onlinelibrary.wiley.com/doi/pdf/10.1002/uog.4052

Observed to expected lung area to head circumference ratio in the prediction of survival in fetuses with isolated diaphragmatic hernia

Rationale: Endothelin-1 (ET1) is dysregulated in pulmonary hypertension (PH). It may be important in the pathobiology of congenital diaphragmatic hernia (CDH). Objectives: We hypothesized that ET1 levels in the first month would be higher in infants with CDH who subsequently expired or were discharged on oxygen (poor outcome). We further hypothesized that ET1 levels would be associated with concurrent severity of PH.Methods: We sampled plasma at 24 to 48 hours, and 1, 2, and 4 weeks of age in 40 prospectively enrolled newborns with CDH. We performed echocardiograms to estimate pulmonary artery pressure at less than 48 hours of age and weekly to 4 weeks. PH was classified in relationship to systemic blood pressure (SBP): less than 2/3 SBP, 2/3 SBP-systemic is related to pressure, or systemic-to-suprasystemic pressure.Measurements and Main Results: ET1 levels at 1 and 2 weeks were higher in infants with poor outcome compared with infants discharged on room air (median and interquartile range: 27.2 [22.6, 33...

https://www.atsjournals.org/doi/pdf/10.1164/rccm.200204-304CC

Louie L. Sanford

Papers

Congenital diaphragmatic hernia. Epidemiology and outcome