Congenital diaphragmatic hernia has a better prognosis when associated with a hernia sac

Bouchghoul, Hanane; Marty, O.; Fouquet, Virginie; Cordier, Anne-Gaël; Sénat, Marie-Victoire; Saada, Julien; Mokhtari, Mostafa; Saché, Nolwenn Le; Martinovic, Jéléna; Benachi, Alexandra

doi:10.1002/pd.5326

Cited by 16 publications

(17 citation statements)

References 21 publications

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“…A particular strength of our work and its findings reported here is the comparatively longer duration of patient follow-up compared with other published studies. [12][13][14][15][16][17][18] The effect size or survival difference(s) in CDH neonates identified to have a sac at operation compared with no sac at our United Kingdom center is not significant in contrast to previous studies examining this issue with shorter patient follow-up. This is, despite our series, being the largest reported and thus having the greatest statistical power to detect a potential difference.…”

Section: Discussionmentioning

confidence: 99%

“…Since then, several other publications have reported the association of hernia sac in CDH with either improved survival or other surrogate measures associated with better outcomes. [13][14][15][16][17][18] It has been proposed that the distinction between CDH with sac and diaphragm eventration is not clear and there does appear to be some confusion in the literature likely originating from an incomplete understanding of the embryology and the urge to classify and discretize a continuum. The etiology of a Bochdalek diaphragmatic hernia is widely believed to be due, in part, to the embryological failure of fusion of the pleuroperitoneal membranes or when a sac is present, consisting solely of a pleuroperitoneal membrane with complete failure of muscularization.…”

Section: Discussionmentioning

confidence: 99%

“…These findings may lend further support to the conjecture that the two entities ("CDH sac vs. no CDH sac") may have distinct embryological origins and subsequent pathophysiology. 12,17,21 Ultrasound remains the main screening modality during the fetal period with selective use of MRI, which is expensive and time consuming. The utility of ultrasound in identifying neonates with a CDH sac based on three criteria has been recently published showing limited positive predictive values.…”

Section: Discussionmentioning

confidence: 99%

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Does the Presence of a Hernia Sac Improve Survival in Newborns with Congenital Diaphragmatic Hernia? A United Kingdom Single-Center Experience

2020

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Introduction A developing body of literature suggests that the presence of a hernia sac in fetuses with congenital diaphragmatic hernia (CDH) may indicate improved prognosis. By examining a large cohort of CDH newborns admitted to a single United Kingdom specialist center, we aimed to establish if presence of hernia sac is a robust predictor of improved survival. Materials and Methods All CDH patients admitted to a single center were recruited. Postneonatal presentations and Morgagni hernias were excluded. Demographics, defect type, laterality, survival, and hernia recurrence were recorded. Results In this study, 192 CDH newborns were managed from 1997 to 2017; 39 were excluded (10 Morgagni and 29 postneonatal); 22 (14%) neonates had a hernia sac. Survival in patients with a hernia sac was 21/22 (95%) versus 107/124 (86%) in cases without hernia sac (p = 0.2). There was no difference in hernia sac proportion by gender (male:female 15 vs. 13.2%, p = 0.8). Conclusion In contrast to studies showing a survival advantage, albeit with smaller patient numbers, we report a statistical nonsignificant benefit of hernia sac. Better survival outcomes at this specialist center with CDH patients without a hernia sac than reported in other published studies are likely responsible for the lack of statistical significance observed, despite a larger cohort. National and international CDH registries yielding “big data” may provide further answers on the utility of a CDH hernia sac as a new prognostic scoring tool.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Does the Presence of a Hernia Sac Improve Survival in Newborns with Congenital Diaphragmatic Hernia? A United Kingdom Single-Center Experience

2020

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“…Numerous studies have shown that low birthweight, prematurity, large defect size, right sided defects, liver herniation, absence of a hernia sac, and severe pulmonary hypertension have a negative impact on neonatal outcomes. 2,3,13,[20][21][22][23][24][25][26][27][28][29] For example, the presence of a hernia sac in CDH has been associated with decreased time spent on ventilation, 30 and the presence of severe pulmonary hypertension has been associated with mortality. 22,31 Despite the previously reported associations with poor neonatal outcomes, we found that only lower birthweights and lower gestational ages were associated with worse respiratory outcomes between 0 and 5 years of age.…”

Section: Discussionmentioning

confidence: 99%

Asthma Medication Use in Congenital Diaphragmatic Hernia Survivors: A Retrospective Population Level Data Analysis

et al. 2019

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Introduction The purpose of this study was to determine if congenital diaphragmatic hernia (CDH) survivors had worse long-term respiratory outcomes compared with age-matched controls, as measured by inhaled bronchodilator use, inhaled steroid use, and asthma-related physician visits. Materials and Methods We performed a retrospective case-control study of infants with isolated CDH from 1991 to 2013. The primary outcome measures were inhaled bronchodilator prescriptions, inhaled steroid prescriptions, and asthma-related physician visits between 0 and 5 years of age and between 5 and 10 years of age. Subgroup analysis compared the same outcomes for CDH patients grouped by: birth weight, gestational age, side of defect, defect size, liver herniation, hernia sac, and pulmonary hypertension. Results Fifty-six patients with CDH and 753 age-matched controls met the inclusion criteria for the 0 to 5 years of age analysis. Between 0 and 5 years of age, more CDH survivors were prescribed an inhaled bronchodilator (odds ratio [OR] = 2.47[1.38–4.48], p = 0.001) and inhaled steroid (OR = 2.03[1.07–3.74], p = 0.03), and had an asthma-related physician visit (OR = 1.92[1.00–3.56], p = 0.04). Thirty-eight cases and 491 controls met the inclusion criteria for the 5 to 10 years of age analysis. Between 5 and 10 years of age, CDH survivors were not more likely to be prescribed inhaled bronchodilators, inhaled steroids, or have an asthma-related physician visit. Among the CDH patients, we did not find a clinical characteristic associated with increased inhaled bronchodilator or steroid prescriptions at any age. Conclusion A history of CDH is associated with higher rates of inhaled bronchodilator prescriptions, inhaled steroid prescriptions, and asthma-related physician visits from 0 to 5 years of age compared with age-matched controls. However, this difference resolves by 5 to 10 years of age.

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“…The presence of a hernia sac predicts better survival [71,72]. The location of fetal stomach, such as intraabdominal, anterior left chest, posterior-mid left chest or retro cardiac, has been shown to strongly predict neonatal outcomes in isolated left CDH [73][74][75].…”

Section: Assessment Of Lung Size Indirect Methodsmentioning

confidence: 99%

Congenital Diaphragmatic Hernia: A Major Challenge for Neonatologists

Prasad¹

2021

Congenital Anomalies in Newborn Infants - Clinical and Etiopathological Perspectives

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Congenital diaphragmatic hernia (CDH) is a major congenital anomaly of the neonates, characterized by the herniation of abdominal contents into the thoracic cavity during fetal life. This results in significant pulmonary hypertension and hypoxemia after birth, which responds poorly to therapeutic interventions. CDH is associated with high morbidity and mortality. The exact pathogenesis is not well understood, and genetic factors have been proposed. The management starts in utero, with antenatal diagnosis and identification of prenatal predictors for the outcomes, which help in the selection of cases suitable for fetal therapy. The postnatal management is complicated by the need for variable cardio-respiratory support and even extra corporeal membrane oxygenation (ECMO), before corrective surgery is undertaken. Improvement in the understanding of the pathophysiology of the underdeveloped lungs and pulmonary vessels has contributed to substantial progress in the management of CDH, which has translated into improved outcomes and survival. Still, many questions regarding CDH remain unanswered and the management is largely based on weak evidence.

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Congenital diaphragmatic hernia has a better prognosis when associated with a hernia sac

Cited by 16 publications

References 21 publications

Does the Presence of a Hernia Sac Improve Survival in Newborns with Congenital Diaphragmatic Hernia? A United Kingdom Single-Center Experience

Does the Presence of a Hernia Sac Improve Survival in Newborns with Congenital Diaphragmatic Hernia? A United Kingdom Single-Center Experience

Asthma Medication Use in Congenital Diaphragmatic Hernia Survivors: A Retrospective Population Level Data Analysis

Congenital Diaphragmatic Hernia: A Major Challenge for Neonatologists

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