I. Pathophysiology/Presentation
  1. Associated with morbidity and mortality from pulmonary hypoplasia
  2. Usually with respiratory distress and hypoxia at birth.
  3. Px "classically" shows barrel chest; scaphoid abdomen
  4. Diagnosed either prenatally (on ultrasound) or postnatally (CXR; differential dx includes congenital lung cyst)
II. Treatment
  1. If diagnosed postnatally, urgent surgery
  2. Prenatal surgery--Occlusion of the fetal trachea to prevent exit of lung fluid
    1. In a randomized trial of 24 pts at 22-27wks gestation, with congenital diaphragmatic hernia diagnosed by prenatal ultrasound, randomized to prenatal tracheal occlusion surgery vs. expectant treatment and surgery immediately after birth, there was no diff. in 90d postnatal survival (73% with surgery, 77% with expectant management), but prenatal surgery group had sig. higher risk of prenatal complications, e.g. PROM and preterm labor.  Mean GA at delivery was sig. lower with surgery (31 vs. 37wks) (NEJM 349:1916, 2003--JW)