Congenital Diaphragmatic Hernia 

 

 

Background 

 

  • Diaphragmatic hernia with intestinal contents in chest 

  • 90% left sided

  • Lung will be underdeveloped & newborn could have persistent pulmonary hypertension

  • Delayed surgery is preferred to stabilize prior to closure 

  • Conventional ventilation with permissive hypercapnia is now favoured 

  • Other therapies: 

    • Surfactant 

    • High-frequency oscillatory ventilation, in addition to nitric oxide

 

 

Considerations

 

  • Emergency situation 

  • Critically ill neonate

  • Hypoplastic lungs:

    • Respiratory insufficiency (hypoxemia, hypercarbia, acidosis)

    • Permissive hypercarbia may be required

    • Consider HVO or ECMO

  • Pulmonary hypertension:

    • Potential for RV failure/↓ cardiac output 

    • Consider inhaled nitric oxide

  • Transitional circulation:

    • Potential for R→L & L→R shunting

    • PDA

  • Delayed surgical repair, resuscitation is first priority 

  • NICU required 

 

 

Resuscitation

 

  • Call NICU

  • Indication for immediate intubation (no bag mask ventilation)

  • NG to decompress stomach

  • ABG, chest x-ray, echocardiogram 

  • Umblical artery/vein lines

  • Lung protective ventilation strategy:

    • Target SaO2 > 85% & permissive hypercapnia (PaCO2 <65 mmHg, pH >7.25)

    • PCV or PSV PIP < 25 cmH2O

    • Inspiratory time 0.35 s

    • PEEP 3-5 mmHg

    • RR < 65

    • Consider HVO, iNO or ECMO

  • Pulmonary hypertension:

    • Consider inhaled nitric oxide

    • Inotropes

  • Fluid: target MAP 45-50 mmHg

  • Sedation: opioids & benzodiazepines, thoracic epidural

  • Avoid NMB