Congenital Diaphragmatic Hernia

  • Etiology: defect at foramen of Bochdalek which is posterolateral in location in diaphragm, can involve herniation of intestine / liver / spleen / kidney causing pulmonary hypoplasia bilaterally but greater on the ipsilateral side, pathophysiologically hypoplastic lung has fewer airways + alveoli / arteries / veins -> decreased surface area for gas exchange and hypoplastic lungs have increased muscularization / thickening of pulmonary vasculature -> pulmonary hypertension
  • CXR: multiple fairly uniform cystic structures in the hemithorax causing mediastinal shift, OK to still have pneumothorax on immediate post-op CXR because hypoplastic lung bud cannot reexpand to fill pleural space immediately and the pleural space will fill with fluid over time and should never be drained with a chest tube
  • Complications: hernia recurrence, chest wall deformities, small bowel obstruction, scoliosis
  • Clinical: usually found at birth, left sided 90% of the time because liver not blocking

Cases of Congenital Diaphragmatic Hernia

CXR of congenital diaphragmatic hernia
AP and lateral CXR shows multiple fairly uniform cystic structures in the left hemithorax causing mediastinal shift to the right. The stomach is in the abdomen.
CXR of ride sided congenital diaphragmatic hernia / Bochdalek hernia
CXR AP shows findings of near-complete opacification of the right hemithorax without loops of bowel in the right hemithorax. The mediastinum is shifted to the left and there is hypoplasia of the left lung as well as of the right lung.
Upper GI exam of congenital diaphragmatic hernia / Bochdalek hernia
AXR AP (above) is remarkable for a paucity of bowel gas in the abdomen. CXR PA (below left) obtained at the same time to rule out right lower lobe pneumonia as a cause of referred right lower quadrant pain shows dense opacity throughout the left hemithorax. CXR PA (below right) obtained during an upper GI and small bowel follow through exam shows a large amount of small bowel in the left hemithorax.
CXR and CT of intrathoracic kidney
CXR AP (upper left) shows a rounded mass in the left lower lobe which appears posterior in location on the CXR lateral (upper right). Coronal and axial CT with contrast of the chest (below) shows the left kidney to be in the lower left hemithorax. In the operating room, a defect at the foramen of Bochdalek was noted and repaired after the kidney had been reduced into the abdomen.
CXR after congenital diaphragmatic hernia repair showing no need for a chest tube
CXR AP (above) taken immediately postop shows that the hypoplastic left lung cannot expand to completely fill the pleural space particularly in the basilar region and subsequently there is a moderate amount of air in the pleural space. This should not be called a pneumothorax. CXR AP taken 1 day later (below) shows the left pleural space now filling with fluid.
CXR and upper GI of recurrent congenital diaphragmatic hernia / Bochdalek hernia
CXR PA and lateral (above) shows loops of bowel in the left lower chest posteriorly. This finding had been present and unchanged post-operatively for over 10 years. AP and lateral images from a small bowel follow through (below) shows colon in lower left chest.
Pathologic image of congenital diaphragmatic hernia
Pathological image of the lungs shows the markedly hypoplastic left lung. Functionally and histologically the right lung was also hypoplastic.