Congenital Diaphragmatic Hernia 

 

• If defective development, diaphragmatic hernia develops
  • Anterior – Morgagni
    • Anteromedial parasternal defect (Space of Larrey)
    • Maldevelopment of septum transversum
    • Tends to occur in overweight, middle-aged, women
    • Right > left (heart protects)
    • Usually not large
    • Usually unilateral
    • Associated with
      • Pericardial defects
      • Omental fat in pericardial space
  • Posterior – Bochdalek
    • Most common
      • Occurs through old pleuroperitoneal canals
        • Just lateral to the spine on either side
    • More frequent on left side
      • Possibly due to “protection” of right-side by liver
      • Hernia may contain intestine, stomach, spleen, liver or omentum
    • If hernia occurs on right
      • Intestine and liver or only liver may herniate
    • If the defect is large, newborns usually present with
      • Severe respiratory distress
      • Cyanosis
      • Scaphoid abdomen
    • Entire diaphragm is almost never absent
      • Some part of diaphragm is usually found at surgery, even if defect is very large
    • Hypoplasia of ipsilateral lung occurs from mass effect of bowel
    • Most often these are isolated congenital abnormalities
      • But they can have
        • Congenital Heart Disease
        • 13 ribs
        • Malrotation of GI tract frequently present
• Imaging findings
  • Initially, hemithorax may appear opaque because loops are fluid-filled
  • Paucity of bowel loops beneath the diaphragm
  • Once air swallowing begins, multiple lucencies contained within bowel are seen in chest
    • Respiratory distress may increase as intestine occupies more of thorax
  • Some loops may remain fluid-filled
  • Mediastinal shift to the opposite side
  • Relative paucity of gas in abdomen
    • If stomach remains in abdomen, it is more centrally located than normal

Multiple air-containing loops of bowel occupy the left hemithorax
and there is a shift of the heart and mediastinal structures to the right side

• Contrast through an NGT is diagnostic but often not needed

• Differential diagnosis of lucent cysts in infant lung
  • Cystic adenomatoid malformation
  • Staphylococcal pneumonia
    • In both, abdomen contains normal amount of air-filled loops
• Delayed herniation of bowel may occur in older infants following streptococcal pneumonia
• Mortality around 50%
  • Pulmonary hypoplasia
  • Persistent Fetal Circulation Syndrome
• Treatment
  • Surgical repair
  • Many demonstrate ipsilateral pulmonary hypoplasia for years after repair
  • Obstructive emphysema can occur in either lower lobe

 

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