Bochdalek hernia - MC hernia, located posteriorly on LEFT – Usually do not contain a hernia sac

Morgagni – More common in adults. Located anteriorly on the RIGHT, usually does contain hernia SAC.

!!50% survival!!!

Most die from congenital defects (neural tube and cardiac defects)

Usually on Left

Associated with vitamin A deficiency and exposure to drugs

Both lungs are dysfunctional

Ipsilateral lung (with hernia) → severe pulmonary hypoplasia

Contralateral lung – severe pulmonary HTN, and some hypoplasia

Pulmonary HTN is the most important physiological affect causing hypoxia

50% will have life-long chronic pulmonary disease

80% have, malrotation, cardiac and neural tube defects (Most of them die due to the congenital defects not CDH)

Dx:

• Prenatal dx can be made with fetal MRI or US
• Work up includes Echo, genetic evaluation
• CXR for newborns to confirm will show bowel is in chest

Planned induction at 39 weeks

• Maternal corticosteroid treatment accelerates lung development in pre-term infants. Routine use in CDH is not currently recommended

Tx: medical optimization first

• Urgent surgical intervention IS NOT INDICATED HERE
• High frequency ventilation, NO, may need ECMO
  • Most devastating ECMO complication → intracerebral bleeding
• ETT, NG, vent support → PaO2 >60 and PaCO2 <60
• Surgery once stable
  • Intra-abdominal approach – reduce bowel and repair with mesh!
  • Need to look for visceral anomalies (malrotation)