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Management advances for congenital diaphragmatic hernia: integrating prenatal and postnatal perspectives
Ahmet A Baschat1, Suneetha Desiraju2, Meghan L Bernier3
1Johns Hopkins Center for Fetal Therapy, Department of Gynecology and Obstetrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Insights
Congenital diaphragmatic hernia (CDH) requires early prenatal diagnosis and management. Advanced fetal therapies like FETO and integrated care programs improve survival rates for newborns with this complex condition.
Area of Science:
- Pediatric Surgery
- Neonatology
- Fetal Medicine
Background:
- Congenital diaphragmatic hernia (CDH) involves displacement of abdominal organs into the chest, leading to pulmonary hypoplasia and hypertension.
- Severity is assessed prenatally via imaging, considering defect laterality, lung compression, and liver herniation.
Purpose of the Study:
- To outline comprehensive prenatal and postnatal management strategies for fetal CDH.
- To highlight the role of advanced interventions and integrated care in improving outcomes.
Main Methods:
- Prenatal diagnosis and severity assessment using imaging.
- Multispecialty counseling and consideration for fetal therapy (FETO).
- Neonatal management including gentle ventilation and extracorporeal life support when indicated.
Main Results:
- Fetoscopic endoluminal tracheal occlusion (FETO) can improve pulmonary capacity.
- Extracorporeal life support benefits neonates with high predicted mortality.
- Early surgical repair (24-48 hours) is associated with better post-operative outcomes.
Conclusions:
- Integrated prenatal and postnatal care programs enhance survival rates for CDH.
- Contemporary management focuses on multidisciplinary care for chronic morbidities.
- Overall survival rates for CDH exceed 70% with current treatment protocols.
Abstract:
In congenital diaphragmatic hernia (CDH), abdominal organs are displaced into the chest, compress the lungs, and cause mediastinal shift. This contributes to development of pulmonary hypoplasia and hypertension, which is the primary determinant of morbidity and mortality for affected newborns. The severity is determined using prenatal imaging as early as the first trimester and is related to the laterality of the defect, extent of lung compression, and degree of liver herniation. Comprehensive evaluation of fetal CDH includes imaging-based severity assessment, severity assessment, and evaluation for structural or genetic abnormalities to differentiate isolated from complex cases. Prenatal management involves multispecialty counseling, consideration for fetal therapy with fetoscopic endoluminal tracheal occlusion (FETO) for severe cases, monitoring and intervention for associated polyhydramnios or signs of preterm labor if indicated, administration of antenatal corticosteroids in the appropriate setting, and planned delivery to optimize the fetal condition at birth. Integrated programs that provide a smooth transition from prenatal to postnatal care produce better outcomes. Neonatal care involves gentle ventilation to avoid hyperinflation and must account for transitional physiology to avoid exacerbating cardiac dysfunction and decompensation. Infants who have undergone and responded to FETO have greater pulmonary capacity than expected, but cardiac dysfunction seems unaffected. In about 25-30% of CDH neonates extracorporeal life support is utilized, and this provides a survival benefit for patients with the highest predicted mortality, including those who underwent FETO. Surgical repair after initial medical management for the first 24-48 hours of life is preferred since later repair is associated with delayed oral feeding, increased need for tube feeds, and increased post-repair ventilation requirement and supplemental oxygen at discharge. With overall survival rates >70%, contemporary care involves management of chronic morbidities in the context of a multidisciplinary clinic setting.
