Delivery room management
Do not use routine bag mask ventilation.
Immediately:
- Intubate the trachea
- Insert a large orogastric tube
- Place gastric tube on continuous suction
- Establish vascular access
- Monitor preductal and postductal oxygen saturation
The aim is gentle ventilation while avoiding barotrauma to the hypoplastic lungs.
Ventilation
Use conventional mechanical ventilation initially.
Limit peak inspiratory pressure to approximately 25 cm H₂O or less where feasible. Accept permissive hypercapnia rather than using injurious pressures simply to normalise PaCO₂. ([pmc.ncbi.nlm.nih.gov](https://pmc.ncbi.nlm.nih.gov/articles/PMC5790558/))
A practical target is:
- Preductal oxygen saturation approximately 85% to 95%
- Accept PaCO₂ approximately 45 to 60 mmHg when systemic pH remains acceptable
Avoid excessive oxygen exposure once adequate preductal saturation is achieved.
Use high frequency ventilation as rescue therapy when adequate gas exchange cannot be achieved without excessive conventional ventilator pressures. ([pmc.ncbi.nlm.nih.gov](https://pmc.ncbi.nlm.nih.gov/articles/PMC5790558/))
Pulmonary hypertension
Optimise:
- Oxygenation
- Ventilation
- Acid base status
- Temperature
- Haemoglobin
- Systemic blood pressure
Avoid systemic hypotension because right to left shunting worsens when pulmonary vascular resistance exceeds systemic resistance.
Inhaled nitric oxide may benefit selected infants with pulmonary hypertension when left ventricular function is adequate, but response is variable in CDH.
Severe left ventricular dysfunction can worsen pulmonary oedema if pulmonary vasodilation increases flow into a poorly functioning left heart.
Use echocardiography to guide vasoactive and pulmonary vascular therapy.
ECMO
Consider extracorporeal support for severe reversible cardiopulmonary failure despite optimal conventional management.
Potential triggers include persistent:
- Severe hypoxaemia
- Acidosis
- Hypercapnia despite safe ventilatory pressures
- Refractory hypotension
- Severe pulmonary hypertension with inadequate systemic oxygen delivery
Timing of repair
Do not rush to theatre immediately after birth.
Repair after physiological stabilisation.
Useful evidence of readiness includes:
- Stable blood pressure
- Improving lactate
- Acceptable urine output
- Reduced ventilator requirements
- Controlled pulmonary hypertension
- Adequate oxygenation on tolerable support
The infant may require several days of intensive stabilisation before surgery.
Operative repair
Reduce abdominal organs gently from the thorax.
Do not injure:
Assess the diaphragmatic defect.
Small defect
Primary closure with nonabsorbable sutures.
Large defect
Use prosthetic or biological patch reconstruction when primary closure would be under excessive tension.
Avoid excessive tension because it contributes to recurrence.
Open abdominal repair is common in neonates because it allows assessment of abdominal viscera and abdominal domain.
Thoracoscopic repair can be considered in selected stable infants with smaller defects, but severe neonatal disease usually requires open surgery.
Abdominal compartment considerations
After reduction of longstanding thoracic viscera, the abdominal cavity may be relatively small.
Do not force primary abdominal fascial closure when this produces:
- Severe ventilatory deterioration
- Reduced venous return
- Oliguria
- Lower limb perfusion compromise
A temporary abdominal closure can be used when necessary.
Long term follow up
Survivors require surveillance for:
- Chronic lung disease
- Persistent pulmonary hypertension
- Gastroesophageal reflux
- Feeding problems
- Failure to thrive
- Scoliosis
- Chest wall deformity
- Sensorineural hearing loss
- Neurodevelopmental impairment
- Hernia recurrence
Recurrence risk is greatest after large patch repaired defects.
