Treatment of Congenital Diaphragmatic Hernia in a One-Month-Old Child
The treatment of congenital diaphragmatic hernia (CDH) in a one-month-old child requires surgical repair with careful respiratory management, including lung-protective ventilation strategies and pulmonary hypertension management, with ECMO reserved for cases refractory to medical therapy. 1
Initial Management Approach
Respiratory Management
- Ventilation strategy:
- Minimize peak inspiratory pressure and avoid large tidal volumes to reduce ventilator-associated lung injury 1
- Target delivered tidal volumes of approximately 3.5 to 5 mL/kg 1
- Consider high-frequency oscillatory ventilation (HFOV) when poor lung compliance, low volumes, and poor gas exchange complicate the clinical course 1, 2
Pulmonary Hypertension (PH) Management
- PH is a critical determinant of survival in infants with CDH, with high prevalence (63%) and significant mortality (45%) 1
- Treatment options include:
- Inhaled nitric oxide (iNO): Use cautiously in patients with suspected left ventricular dysfunction 1
- Reserve iNO for patients with suprasystemic pulmonary vascular resistance with right-to-left shunting causing critical preductal hypoxemia 1
- Prostaglandin E1: Consider to maintain patency of the ductus arteriosus in infants with suprasystemic PH or right ventricular failure 1
- Sildenafil: May augment pulmonary vasodilator effects and has shown resolution of PH during prolonged therapy 1
Surgical Approach
- Delayed surgical repair after a period of stabilization is preferred over immediate surgery 3, 4
- Median time of surgical repair reported at approximately 10 days of life in preterm infants 5
- Surgery can be performed in the NICU if the patient remains unstable on conventional ventilation 4
Advanced Management Options
ECMO (Extracorporeal Membrane Oxygenation)
- Indicated for CDH patients with severe PH who do not respond to medical therapy 1
- Can improve survival rates from 67% with conventional mechanical ventilation to 94% with HFOV and ECMO when needed 2
- Consider ECMO when severe hypoxemia is refractory to iNO and optimization of respiratory and cardiac function 1
Post-Surgical Management
- Monitor for persistent PH, which may require continued pulmonary vasodilator therapy even after mechanical ventilation is no longer needed 1
- Evaluate for long-term PAH-specific therapy, which should include cardiac catheterization 1
- Longitudinal care in an interdisciplinary pediatric PH program is essential for infants with CDH who have PH or are at risk of developing late PH 1
Prognostic Indicators and Follow-up
- 93% of infants achieving pulmonary arterial pressure less than two-thirds systemic pressure by 2 weeks of age were discharged alive on room air 1
- 100% survival among infants with pulmonary/systemic arterial pressure ratio <0.5 by 3 weeks of age 1
- Poor prognosis for infants with persistence of systemic levels of pulmonary arterial pressure at 6 weeks of age 1
- Long-term follow-up should include:
- Annual echocardiography
- Assessment of overall respiratory course
- Monitoring for late or sustained PH 1
Common Pitfalls and Caveats
- Avoid routine use of iNO in CDH as it may worsen pulmonary venous hypertension and cause pulmonary edema in patients with left ventricular dysfunction 1
- Beware of infection risk in CDH newborns requiring NICU surgery 4
- Monitor for persistent PH which can contribute to late morbidity and mortality even after successful surgical repair 1
- Recognize that pulmonary arterial and venous structural abnormalities can be difficult to diagnose by echocardiography alone, suggesting a role for cardiac catheterization 1