Pulmonary interstitial emphysema

Pulmonary interstitial emphysema (PIE) refers to the abnormal location of air within the pulmonary interstitium and lymphatics. It typically results from rupture of overdistended alveoli following barotrauma in infants who have hyaline membrane disease. Interstitial emphysema can also occasionally be incidentally detected in adults.  

PIE is almost always associated with mechanical ventilation or continuous positive airway pressure in the first weeks of life. 

Reduced lung compliance, prematurity, low birth weight, meconium aspiration syndrome and pneumonia are other risk factors. 

The reported incidence of PIE amongst infants admitted to the neonatal ICU is 2-3% 6. The incidence rises to 20-30% amongst premature infants. The highest frequencies are seen in low-birth-weight infants 7.

PIE is typically an incidental finding on routine chest radiographs in the NICU before the onset of symptoms. If it is more extensive, it may result in compression of adjacent lung and vasculature. This can impede both ventilation and pulmonary blood flow, which can affect oxygenation, ventilation, and blood pressure. PIE may also present with air-block complications such as pneumomediastinum, pneumothorax, pneumopericardium, pneumoperitoneum, or subcutaneous emphysema.

PIE almost always occurs during the first weeks of life in newborns on ventilatory support. Alveolar pressure increases in the setting of mechanical ventilation or continuous positive airway pressure. A combination of increased pressure and poor compliance can result in alveolar rupture with the escape of air into the adjacent interstitium and lymphatics. This interstitial air may resolve spontaneously, persist chronically or progress with further air-leak into adjacent spaces, causing pneumomediastinum, pneumothorax, pneumopericardium, pneumoperitoneum, or subcutaneous emphysema.

Overall lung volumes are increased; however, the lungs are less compliant because they are splinted at a large volume by the air within the interstitium. This may further compromise the already critically ill child. Air-block complications of PIE such as pneumomediastinum, pneumothorax and pneumopericardium can be fatal.

Distribution

PIE may be focal affecting one lobe or diffuse and bilateral. It has no predilection for any particular lobe.

Plain radiograph

Chest radiographs will demonstrate some or all of the following features:

  • cystic or linear radiolucencies in the interstitium radiating from the hilum
  • affected segment is often hyperexpanded and static in volume on multiple radiographs
  • pneumothoraxpneumomediastinum, or pneumopericardium
  • the heart tends to get smaller as intrathoracic pressure increases and results in diminished venous return into the chest
CT 
  • not usually part of the routine investigation (unnecessary risk of radiation exposure)
  • shows cystic radiolucencies in affected segment
  • may characteristically show a line and dot pattern with pulmonary arterial branches surrounded by radiolucent air 4
  • may help differentiate persistent PIE from a hyperlucent mass such as congenital lobar emphysema, congenital pulmonary airway malformation (CPAM)
  • allows better visualisation of a pneumothorax or pneumomediastinum 
  • if incidentally detected in adults, it may appear as perivascular lucent or low-attenuating halos and small cysts 5

Switching from conventional to high-frequency ventilation is commonly performed in infants with PIE. Many cases resolve spontaneously. Frequent radiographs may be performed to assess for complications such as pneumothorax or pneumomediastinum. 

Selective bronchial intubation, lobectomy and pneumonectomy are rarely used to treat progressive PIE 8.

General imaging differential considerations include:

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Article information

rID: 1939
Systems: Chest, Paediatrics
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Pulmonary interstitial emphysema (PIE)
  • PIE
  • Interstitial emphysema of the lung
  • Interstitial emphysema of lung

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