Pulmonary Hypoplasia

  • Etiology:
    — Oligohydramnios due to bladder outlet obstruction
    — Congenital diaphragmatic hernia
  • Imaging: Airways and alveoli and pulmonary vessels are decreased in size and number leading to small lung volumes
  • DDX:
  • Complications:
  • Treatment:
  • Clinical: Can present with bilateral pneumothoraces at birth due to poor lung development

Radiology Cases of Pulmonary Hypoplasia

CXR of pulmonary hypoplasia due to oligohydramnios
CXR AP shows bilateral poorly developed and poorly expanded lungs.

Radiology Cases of Pulmonary Hypoplasia due to Oligohydramnios due to Autosomal Recessive Polycystic Kidney Disease

CXR and US of autosomal recessive polycystic kidney disease causing oligohydramnios and pulmonary hypoplasia
CXR AP (above) shows a small bell-shaped thorax with poorly expanded lungs despite being on a ventilator. Transverse US of the left kidney (below left) and right kidney (below right) shows the kidneys bilaterally to be enlarged and to be composed of innumerable microcysts giving the kidneys overall an echogenic appearance.

Radiology Cases of Pulmonary Hypoplasia due to Scoliosis

CXR of congenital scoliosis due to rib fusion
CXR AP shows multiple left-sided anterior and multiple right-sided posterior rib fusions and a mild curvature of the spine convex left resulting in a small thorax.

Radiology Cases of Pulmonary Hypoplasia due to Congenital Diaphragmatic Hernia

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.

Clinical Cases of Pulmonary Hypoplasia

Clinical image of Potter syndrome due to pulmonary hypoplasia
Clinical image shows Potter’s syndrome – a flattened nose, recessed chin, prominent epicanthial folds and low set of abnormal ears.

Clinical Cases of Pulmonary Hypoplasia Due to Urethral Atresia

Clinical image of Potter's facies due to urethral atresia
Post-mortem gross pathological image shows Potter’s facies – a flattened face and low set ears – and a small thorax with pulmonary hypoplasia and large abdomen.