Prenatal Care for Fetus with Congenital Diaphragmatic Hernia
When CDH is diagnosed prenatally, the newborn must be delivered at a tertiary center with immediate plans for intubation in the delivery room, and prenatal diagnosis itself is a crucial prognostic factor that improves survival by enabling specialized neonatal care. 1
Immediate Prenatal Actions After CDH Diagnosis
Comprehensive Fetal Evaluation
- Perform amniocentesis with karyotype analysis to rule out chromosomal abnormalities, which occur in 3% of CDH cases 2
- Conduct detailed anatomic ultrasound to identify associated malformations, present in 10-50% of cases, including CNS, cardiac, digestive, and urogenital anomalies 2
- Obtain fetal echocardiography to evaluate for congenital heart disease and assess for pulmonary hypertension, which occurs in 63% of CDH cases and carries 45% mortality 1, 3
Severity Stratification and Prognostic Assessment
- Use CT imaging as the gold standard to evaluate defect size, identify herniated organs, and assess the degree of pulmonary hypoplasia 1
- Determine liver position (up vs. down) and calculate lung-to-head ratio, as these are the most accurate predictors of postnatal prognosis 4
- Monitor for intrauterine growth restriction (IUGR), which is commonly associated with CDH 2
Delivery Planning and Coordination
Tertiary Center Delivery
- Arrange delivery at a tertiary perinatal center with neonatal intensive care unit, pediatric surgery, and ECMO capabilities 1, 2, 5
- Plan elective cesarean section at 37 weeks to optimize timing and preparation for immediate neonatal intervention 5
- Administer prenatal corticosteroids for lung maturation prior to delivery 5
Multidisciplinary Team Assembly
- Establish care coordination between maternal-fetal medicine, neonatology, pediatric surgery, and anesthesiology before delivery 1
- Prepare for immediate intubation in the delivery room rather than allowing spontaneous breathing, which can worsen herniation 1, 5
Consideration for Fetal Intervention
FETO (Fetal Endoscopic Tracheal Occlusion)
- Consider FETO for severe CDH cases with poor prognostic indicators (liver herniation, low lung-to-head ratio) as it promotes lung growth through reversible tracheal occlusion 6, 4
- Select candidates carefully based on severity stratification, as this intervention is reserved for fetuses predicted to have the worst outcomes 6
- Understand that FETO remains investigational and should only be performed at specialized fetal surgery centers with appropriate expertise 4
Prenatal Counseling Requirements
Parental Education
- Discuss the 45% overall mortality rate and explain that survival is critically dependent on the severity of pulmonary hypoplasia and presence of pulmonary hypertension 3, 2
- Explain long-term morbidities including gastroesophageal reflux (up to 62%), chronic lung disease, neurodevelopmental deficits, and need for lifelong multidisciplinary follow-up 7
- Address option for pregnancy termination when severe associated anomalies or chromosomal abnormalities are identified 2
Postnatal Management Preview
- Inform parents about planned immediate intubation with gentle ventilation strategies (tidal volumes 3.5-5 mL/kg) to prevent barotrauma 7
- Explain potential need for ECMO in cases with severe pulmonary hypertension unresponsive to medical therapy 3, 7
- Clarify that surgical repair is not emergent and typically occurs 34 hours after birth once stabilization is achieved 5
Critical Pitfalls to Avoid
- Do not deliver at a non-tertiary center, as prenatal diagnosis loses its survival benefit without immediate access to specialized neonatal intensive care 2, 5
- Do not plan for surfactant administration, as it has been associated with increased ECMO need, chronic lung disease, and mortality in CDH 3
- Do not assume isolated CDH without thorough evaluation for associated anomalies and chromosomal abnormalities, which significantly alter prognosis 2
- Do not delay karyotype analysis, as 3% of CDH cases have chromosomal abnormalities that may influence management decisions 2