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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

 

WH 

 

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