What are the antenatal interventions for Congenital Diaphragmatic Hernia (CDH)?

Antenatal Interventions for Congenital Diaphragmatic Hernia (CDH)

Fetal tracheal occlusion (FETO) is the primary antenatal intervention for severe CDH cases with poor prognosis, though it is not routinely recommended for all CDH cases due to limited evidence on long-term outcomes. 1

Understanding CDH and Its Prenatal Management

• CDH is a relatively rare disorder occurring in 1 of every 2500 births, characterized by marked lung hypoplasia with pulmonary hypertension (PH) and impaired cardiac performance 2
• Severe PH is a critical determinant of survival in infants with CDH, with high prevalence (63%) and mortality (45%) 2
• Prenatal diagnosis allows for identification of high-risk cases that may benefit from antenatal interventions 3

Antenatal Interventions

Fetal Tracheal Occlusion (FETO)

• FETO is performed via minimally invasive fetoscopy and works by promoting lung growth through fluid accumulation in the lungs 1
• Evidence suggests FETO may increase long-term infant survival (3-6 months) and reduce pulmonary hypertension in severe CDH cases 1
• FETO is associated with a small reduction in gestational age at birth but may increase the risk of preterm birth 1
• Potential maternal complications include preterm ruptured membranes and infectious morbidity 1

Antenatal Corticosteroids

• Limited evidence exists regarding the efficacy of antenatal corticosteroids specifically for CDH 1
• One small trial showed no clear difference in perinatal mortality between antenatal corticosteroids and placebo 1
• No significant differences were observed in days of mechanical ventilation or hospital admission 1
• Further research is needed to determine the benefit of antenatal steroids specifically for CDH 1, 4

Prenatal Evaluation and Planning

• Prenatal diagnosis and multidisciplinary perinatal management may improve outcomes despite higher risk factors in prenatally diagnosed cases 3
• Comprehensive prenatal evaluation should include assessment for liver herniation, which is associated with higher risk 3
• Planned delivery at centers with appropriate neonatal intensive care capabilities is recommended for optimal management 4, 3

Post-Delivery Management Considerations

• Minimizing peak inspiratory pressure and avoiding large tidal volumes is recommended to reduce ventilator-associated acute lung injury 2
• High-frequency oscillatory ventilation is a reasonable alternative when poor lung compliance and gas exchange complicate management 2
• Inhaled nitric oxide (iNO) can be used to improve oxygenation but should be used cautiously in patients with suspected left ventricular dysfunction 2, 5
• Extracorporeal membrane oxygenation (ECMO) is recommended for patients with severe PH who do not respond to medical therapy 2
• Prostaglandin E1 may be considered to maintain patency of the ductus arteriosus and improve cardiac output in infants with suprasystemic PH 2

Important Caveats and Pitfalls

• Surfactant treatment for CDH is not recommended as it has been associated with increased need for ECMO, higher incidence of chronic lung disease, and increased mortality 2
• iNO therapy should not be used routinely in CDH; its use should be limited to patients with suprasystemic pulmonary vascular resistance causing critical preductal hypoxemia 2, 5
• Failure of iNO to cause sustained improvement in CDH may be due to left ventricular dysfunction, which can worsen pulmonary edema when pulmonary vascular resistance is lowered 2, 5
• Preterm CDH (especially with FETO) confers high morbidity and mortality, requiring specialized management 6

Future Directions

• More studies are needed to further examine the effect of FETO on important neonatal outcomes and long-term infant survival 1
• Standardization of procedures, inclusion criteria, and long-term childhood follow-up should be included in ongoing research 1
• Multicenter studies and standardized guidelines are needed to improve outcomes in challenging patient populations, particularly preterm infants with CDH 6