Abstract
Early reports have suggested survival benefits associated with a hernia sac in congenital diaphragmatic hernia (CDH). However, these studies have included only small subsets of patients. This systematic review aimed to evaluate differences in outcomes of CDH newborns with and without a hernia sac. PubMed and Embase databases were searched using relevant key terms. Papers were independently reviewed by two authors with final selection approved by the senior author. Original search retrieved 537 papers; the final review included 8 studies (n = 837 patients). There were 168 CDH patients (20%) with a hernia sac with an overall survival of 93% vs 73% for CDH newborns without a sac (p < 0.001). Twenty-three percent of patients with a CDH sac required diaphragm patch repair vs 44% patients without a sac (p < 0.001). Pulmonary hypertension was manifested in 44% of CDH babies with a hernia sac vs 64% without a sac (p < 0.001). Three studies compared ECMO requirement: 15% with a hernia sac and 34% without sac, p < 0.001.Conclusion: This study shows significant survival benefits in newborns associated with presence of a CDH sac. This may be likely related to these infants having more favourable physiology with less severe pulmonary hypertension and/or smaller anatomical defects requiring primary closure only.What is Known:• Early reports have suggested survival benefits associated with a hernia sac in CDH.• Previous studies have included only a small number of patients.What is New:• A systematic review of published studies clearly shows that CDH newborns with a hernia sac have better overall survival outcomes and less severe pulmonary hypertension.• ECMO utilization and patch repair were also less often required in newborns with a hernia sac.
Highlights
Congenital diaphragmatic hernia (CDH) is the result of diaphragm maldevelopment during foetal life giving rise to pathological herniation of abdominal viscera into the thorax which impacts on normal lung growth associated with pulmonary hypoplasia and hypertension [1]
This study shows significant survival benefits in newborns associated with presence of a CDH sac
Reports have suggested survival benefits associated with a hernia sac in CDH
Summary
Congenital diaphragmatic hernia (CDH) is the result of diaphragm maldevelopment during foetal life giving rise to pathological herniation of abdominal viscera into the thorax which impacts on normal lung growth associated with pulmonary hypoplasia and hypertension [1]. CDH consists of a direct anatomical defect communicating between the thoracic and abdominal cavities. The presence of a hernia sac with CDH is reported in approximately some 20% of cases [11, 12]. It has been postulated that the presence of a hernia sac may be associated with a better prognosis [11–15]. Early reports have portrayed survival benefit associated with a hernia sac, this has not been consistent in all studies [16]. Due to the rarity of a CDH-associated hernia sac, most studies to date have only described small numbers of patients presenting with a sac anomaly [13, 15, 16]
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