Pulmonary interstitial emphysema (PIE) is a collection of

air The atmosphere of Earth is the layer of gases, known collectively as air, retained by Earth's gravity that surrounds the planet and forms its planetary atmosphere. The atmosphere of Earth protects life on Earth by creating pressure allowing f ...

outside of the normal air space of the

pulmonary alveoli A pulmonary alveolus (plural: alveoli, from Latin ''alveolus'', "little cavity"), also known as an air sac or air space, is one of millions of hollow, distensible cup-shaped cavities in the lungs where oxygen is exchanged for carbon dioxide. Al ...

, found instead inside the

connective tissue Connective tissue is one of the four primary types of animal tissue, along with epithelial tissue, muscle tissue, and nervous tissue. It develops from the mesenchyme derived from the mesoderm the middle embryonic germ layer. Connective tiss ...

of the peribronchovascular sheaths, interlobular septa, and visceral pleura. (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, assisted ventilation or intermittent mandatory ventilation (IMV), is the medical term for using a machine called a ventilator to fully or partially provide artificial ventilation. Mechanical ventilation helps move a ...

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. Neonatal refers to the first 28 days of life. Neonatal care, as k ...

Cause

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

Infant respiratory distress syndrome Infantile respiratory distress syndrome (IRDS), also called respiratory distress syndrome of newborn, or increasingly surfactant deficiency disorder (SDD), and previously called hyaline membrane disease (HMD), is a syndrome in premature infants ...

(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 between ...

aspiration *

Sepsis Sepsis, formerly known as septicemia (septicaemia in British English) or blood poisoning, is a life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. This initial stage is follo ...

Infection An infection is the invasion of tissues by pathogens, their multiplication, and the reaction of host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmissible disease or communicable di ...

s *

Mechanical ventilation Mechanical ventilation, assisted ventilation or intermittent mandatory ventilation (IMV), is the medical term for using a machine called a ventilator to fully or partially provide artificial ventilation. Mechanical ventilation helps move a ...

Pathophysiology

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

bronchioles The bronchioles or bronchioli (pronounced ''bron-kee-oh-lee'') 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 ...

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 High-frequency ventilation is a type of mechanical ventilation which utilizes a respiratory rate greater than four times the normal value. (>150 (Vf) breaths per minute) and very small tidal volumes. High frequency ventilation is thought to reduce ...

Lobectomy Lobectomy means ''surgical excision of a lobe''. This may refer to a lobe of the lung (also simply called a lobectomy), a lobe of the thyroid ( hemithyroidectomy), a lobe of the brain (as in anterior temporal lobectomy), or a lobe of the liver ...

*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 Bronchopulmonary dysplasia (BPD; part of the spectrum of chronic lung disease of infancy) is a chronic lung disease in which premature infants, usually those who were treated with supplemental oxygen, require long-term oxygen. The alveoli that are ...

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