Congenital Diaphragmatic Hernia

Etiology:
— Defect at foramen of Bochdalek which is posterolateral in location in diaphragm
— Can involve herniation of intestine or liver or spleen or kidney causing pulmonary hypoplasia bilaterally but greater on the ipsilateral side
— Pathophysiologically hypoplastic lung has fewer airways and alveoli and arteries and veins which leads to decreased surface area for gas exchange and hypoplastic lungs have increased muscularization and thickening of pulmonary vasculature which leads to pulmonary hypertension
Imaging CXR Preop: Multiple fairly uniform cystic structures in the hemithorax causing mediastinal shift
Imaging CXR Postop: Air and then fluid fill the affected hemithorax after congenital diaphragmatic hernia repair so it is 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
DDX:
Complications:
— Hernia recurrence due to graft or suture failure
— Chest wall deformities
— Small bowel obstruction
— Scoliosis
Treatment:
Clinical:
— Usually found at birth
— Left sided 90% of the time because liver not blocking
— Patients with liver herniation have worse prognosis

Radiology 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.

CXR of diaphragmatic hernia — CXR shows opacification of the right hemithorax with several air filled loops of bowel within it and mediastinal shift to the left.

CXR of congenital diaphragmatic hernia — CXR AP shows complete opacification of the left hemithorax with mediastinal shift to the right and a small right pneumothorax. The arterial ECMO canula tip projects over the aortic arch and the venous ECMO canula tip projects over the right atrium. The nasogastric tube tip projects over the mid-esophagus.

CXR of malposition ECMO catheter in patient with congenital diaphragmatic hernia — CXR shows normal position of the arterial catheter tip in the aortic arch and abnormal position of the venous catheter tip (represented by a radio-opaque point) in the superior vena cava. Further advancement of the tip into the right atrium is necessary. Multiple cystic structures are present in the left hemithorax. The lungs are densely opacified.

CXR of evolution of congenital diaphragmatic hernia — CXR AP at 1 hour of life (above) shows the endotracheal tube and mediastinum to be shifted to the right by a large mass in the left hemithorax. The endotracheal tube tip is at the level of the carina. CXR AP at 2 hours of life (below left) shows the large mass in the left hemithorax to now consist of multiple thick walled air bubbles that are causing further mediastinal shift to the right. CXR AP at 3 hours of life (below right) shows even more thick walled air bubbles in the left hemithorax resulting in even more mediastinal shift to the right.

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.

CT of congenital diaphragmatic hernia — CXR (left) shows massive pneumoperitoneum and right pneumothorax which outlines a mass in the lower right chest which is causing mediastinal shift to the left. Coronal CT with contrast of the chest (above right) shows liver herniating through a lateral defect in the diaphragm into the lower right chest and lying above the right diaphragm (below right).

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.

Radiology Cases of Congenital Diaphragmatic Hernia Immediately After Repair

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 of hypoplastic lung bud postoperatively in congenital diaphragmatic hernia — CXR AP (left) shows the hypoplastic lung bud which cannot immediately expand to fill the hemithorax in the apex of the left hemithorax and therefore there is also air in the left pleural space. Note that this is not a pneumothorax and should not be drained via a chest tube. CXR AP obtained 2 days later (right) shows the left pleural space is now filled with fluid rather than air, and again this should not be drained by a chest tube. As the lung bud expands, the pleural effusion will decrease in size.

Radiology Cases of Recurrent Congenital Diaphragmatic Hernia

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.

Surgery Cases of Congenital Diaphragmatic Hernia

Surgery image of congenital diaphragmatic hernia — Surgical image taken after reduction of a diaphragmatic hernia and repair of a right diaphragmatic defect shows the Gore-Tex patch (white) replacing the absent posterolateral portion of diaphragm.

Gross Pathology Cases of Congenital Diaphragmatic Hernia

Gross pathological image of congenital diaphragmatic hernia — Gross pathological image shows portions of the intestine and spleen in the left hemithorax causing the heart to be displaced into the right hemithorax.

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.

Gross pathological image of congenital diaphragmatic hernia — Gross pathological image shows the heart and great vessels flipped superiorly, revealing an extremely hypoplastic left lung. The right lung is also somewhat hypoplastic in size.

Pathological image of congenital diaphragmatic hernia — Post mortem pathological image of the underside of the removed diaphragm shows a Gore-Tex patch in the left posterolateral foramen of Bochdalek.