Congenital diaphragmatic hernia

Abstract

Infants with congenital diaphragmatic hernia (CDH) have significant mortality and long-term morbidity. Only 60-70% survive and usually those in high-volume centres. The current Task Force, therefore, has convened experts to evaluate the current literature and make recommendations on both the antenatal and post-natal management of CDH. The incidence of CDH varies from 1.7 to 5.7 per 10,000 live-born infants depending on the study population. Antenatal ultrasound scanning is routine and increasingly complemented by the use of magnetic resonance imaging. For isolated CDH, antenatal interventions should be considered, but the techniques need vigorous evaluation. After birth, management protocols are often used and have improved outcome in nonrandomised studies, but immediate intubation at birth and gentle ventilation are important. Pulmonary hypertension is common and its optimal management is crucial as its severity predicts the outcome. Usually, surgery is delayed to allow optimal medical stabilisation. The role of minimal invasive post-natal surgery remains to be further defined. There are differences in opinion about whether extracorporeal membrane oxygenation improves outcome. Survivors of CDH can have a high incidence of comorbidities; thus, multidisciplinary follow-up is recommended. Multicentre international trials are necessary to optimise the antenatal and post-natal management of CDH patients.

Current Concepts in the Management of Congenital Diaphragmatic Hernia in Infants

“…Whatever the cause results in a defect in the diaphragm, commonly on the left side (85-90 %), but can occur on the right or bilaterally and may be associated with other anomalies. The commonest (90 %) defect involves the posterolateral region of the diaphragm (Bochdalek hernia) but the anterior (Morgagni hernia) or central regions can also be affected [1].…”

A review of congenital diaphragmatic hernia

“…Amniocentesis to diagnose associated genetic anomalies is recommended 5 . Three to 30% of cases will have an associated chromosomal anomaly, with a resultant poorer prognosis 13 …”

Congenital Diaphragmatic Hernia with Delayed Diagnosis: Report of Two Cases

“…According to the Extracorporeal Life Support Organization Registry, the median survival estimate of children with CDH is around 60% [4], being one of the main determinants of mortality in CDH, which include coexistence of the major anomalies (complex CDH), the diaphragm defect size, the degree of pulmonary hypoplasia, the severity of pulmonary hypertension in the perinatal period and the side of the hernia defect [5] [6].…”

“…The CDH affects approximately 1 in every 2000 -5000 live births [2] [3] and it is a major cause of neonatal mortality and morbidity, and despite the advances in therapeutic, such as high frequency ventilation, nitric oxide and extracorporeal membrane oxygenation (ECMO), and improvement in surgical management of CDH, the rate of mortality remains high [4].…”