Congenital diaphragmatic hernia medical therapy

Medical therapy can be subdivided according to antenatal and postnatal management. Antenatal management focuses on close monitoring. Fetal therapy may be an option for eligible patients with resultant fetal tracheal occlusion performed at 26-30 weeks to allow increased surfactant formation. Postnatal management primarily focuses on preventing birth before 37 weeks of gestation, as these neonates have been shown to have worse outcomes comparatively. Other postnatal options include the use of ECMO, the use of a nasogastric tube and intubation where required as well as maintaining adequate ventilation and oxygenation. Medications that may be helpful in reducing pulmonary hypertension include inhaled nitric oxide, sildenafil, bosentan, PGE1, and PGI2.

• Close monitoring to assess for fetal well-being; fetal therapy may be offered to eligible patients which involves occlusion of the fetal trachea at 26-30 weeks of gestation which allows fluid accumulation in the lung and its stretching. This results in release of surfactant but a potentially increased risk of preterm delivery. Post-natal CDH repair is still required.^[2]

• Ensure delivery is not before 37 weeks, preferably at 39-40 weeks
• Extracorporeal membrane oxygenation (ECMO) therapy has been used as part of the treatment strategy at some hospitals.^[4][5]

• Immediate use of nasogastric tube to decompress the stomach and intestines
• Immediate intubation in case of respiratory distress

• Adequate ventilation and oxygenation
• Vasopressors
• Inhaled nitric oxide (vasodilator)^[6]
• Other drugs: sildenafil, bosentan, PGE1, PGI2