Pulmonary interstitial emphysema (PIE) is a collection of

air An atmosphere () is a layer of gases that envelop an astronomical object, held in place by the gravity of the object. A planet retains an atmosphere when the gravity is great and the temperature of the atmosphere is low. A stellar atmosph ...

outside of the normal air space of the pulmonary alveoli, found instead inside the

connective tissue Connective tissue is one of the four primary types of animal tissue, a group of cells that are similar in structure, along with epithelial tissue, muscle tissue, and nervous tissue. It develops mostly from the mesenchyme, derived from the mesod ...

of the peribronchovascular sheaths, interlobular septa, and

visceral pleura The pleurae (: pleura) are the two flattened closed sacs filled with pleural fluid, each ensheathing each lung and lining their surrounding tissues, locally appearing as two opposing layers of serous membrane separating the lungs from the media ...

. (This supportive tissue is called the pulmonary interstitium.) This collection of air develops as a result of alveolar and terminal bronchiolar rupture. Pulmonary interstitial emphysema is more frequent in premature infants who require

mechanical ventilation Mechanical ventilation or assisted ventilation is the Medicine, medical term for using a ventilator, ventilator machine to fully or partially provide artificial ventilation. Mechanical ventilation helps move air into and out of the lungs, wit ...

for severe lung disease. Infants with pulmonary interstitial emphysema are typically recommended for admission to a

neonatal intensive care unit A neonatal intensive care unit (NICU), also known as an intensive care nursery (ICN), is an intensive care unit (ICU) specializing in the care of ill or premature newborn infants. The NICU is divided into several areas, including a critical c ...

Cause

Pulmonary interstitial emphysema is a concern in any of the following: * Prematurity *

Infant respiratory distress syndrome Infant respiratory distress syndrome (IRDS), also known as surfactant deficiency disorder (SDD), and previously called hyaline membrane disease (HMD), is a syndrome in premature infants caused by developmental insufficiency of pulmonary surfacta ...

(IRDS) * Meconium aspiration syndrome (MAS) *

Amniotic fluid The amniotic fluid is the protective liquid contained by the amniotic sac of a gravid amniote. This fluid serves as a cushion for the growing fetus, but also serves to facilitate the exchange of nutrients, water, and biochemical products betwee ...

aspiration *

Sepsis Sepsis is a potentially life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. This initial stage of sepsis is followed by suppression of the immune system. Common signs and s ...

Infection An infection is the invasion of tissue (biology), tissues by pathogens, their multiplication, and the reaction of host (biology), host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmis ...

s *

Mechanical ventilation Mechanical ventilation or assisted ventilation is the Medicine, medical term for using a ventilator, ventilator machine to fully or partially provide artificial ventilation. Mechanical ventilation helps move air into and out of the lungs, wit ...

Pathophysiology

Pulmonary interstitial emphysema is created when air bursts or ruptures through tissue from the alveoli and

bronchioles The bronchioles ( ) are the smaller branches of the bronchial airways in the lower respiratory tract. They include the terminal bronchioles, and finally the respiratory bronchioles that mark the start of the respiratory zone delivering air to t ...

into the perivascular tissue of the lung.

Diagnosis

A chest X ray may show a "salt and pepper" appearance due to the linear lucencies that are present.

Treatment

Different treatments have been used to manage pulmonary interstitial emphysema with variable success. Admission/transfer to a

(NICU) is common and expected for patients with PIE. Treatments include: *Lateral decubitus position with the affected side down * High-frequency ventilation * Lobectomy *Selective Main Bronchial Intubation and Occlusion

Prognosis

Pulmonary interstitial emphysema often resolves gradually and may take 2–3 weeks. For longer durations of PIE the length of time of mechanical ventilation needed may increase and the incidence of bronchopulmonary dysplasia becomes higher. Some infants may develop chronic lobar emphysema, which may require surgical lobectomies.

Epidemiology

United States

The prevalence of pulmonary interstitial emphysema widely varies with the population studied. In a 1987 study 3% of infants admitted to the

(NICU) developed pulmonary interstitial emphysema.

International

Studies reflecting international frequency demonstrated that 2-3% of all infants in NICUs develop pulmonary interstitial emphysema. When limiting the population studied to premature infants, this frequency increases to 20-30%, with the highest frequencies occurring in infants weighing fewer than 1000 g.

References

External links

{{Medical resources , DiseasesDB = , ICD10 = {{ICD10, P25.0 , ICD9 = {{ICD9, 518.1 , ICDO = , OMIM = , MedlinePlus = , eMedicineSubj = ped , eMedicineTopic = , MeshID = Respiratory therapy Pulmonology