Pulmonary interstitial emphysema

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Pulmonary interstitial emphysema
CXR-Pulmonary interstitial emphysema.jpg
Chest radiography showing severe pulmonary interstitial emphysema
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Pulmonary interstitial emphysema (PIE) is a collection of air outside of the normal air space of the pulmonary alveoli, found instead inside the connective tissue 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 for severe lung disease. Infants with pulmonary interstitial emphysema are typically recommended for admission to a neonatal intensive care unit.

Contents

Cause

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

Pathophysiology

Pulmonary interstitial emphysema is created when air bursts or ruptures through tissue from the alveoli and bronchioles 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 neonatal intensive care unit (NICU) is common and expected for patients with PIE. Treatments include:

Prognosis

Pulmonary interstitial emphysema often resolves gradually and may take 2–3 weeks. [2] 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. [3]

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 neonatal intensive care unit (NICU) developed pulmonary interstitial emphysema. [2]

International

Studies reflecting international frequency demonstrated that 2-3% of all infants in NICUs develop pulmonary interstitial emphysema. [4] [5] 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. [6]

See also

Related Research Articles

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<span class="mw-page-title-main">Mechanical ventilation</span> Method to mechanically assist or replace spontaneous breathing

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 air into and out of the lungs, with the main goal of helping the delivery of oxygen and removal of carbon dioxide. Mechanical ventilation is used for many reasons, including to protect the airway due to mechanical or neurologic cause, to ensure adequate oxygenation, or to remove excess carbon dioxide from the lungs. Various healthcare providers are involved with the use of mechanical ventilation and people who require ventilators are typically monitored in an intensive care unit.

<span class="mw-page-title-main">Oxygen toxicity</span> Toxic effects of breathing oxygen at high concentrations

Oxygen toxicity is a condition resulting from the harmful effects of breathing molecular oxygen at increased partial pressures. Severe cases can result in cell damage and death, with effects most often seen in the central nervous system, lungs, and eyes. Historically, the central nervous system condition was called the Paul Bert effect, and the pulmonary condition the Lorrain Smith effect, after the researchers who pioneered the discoveries and descriptions in the late 19th century. Oxygen toxicity is a concern for underwater divers, those on high concentrations of supplemental oxygen, and those undergoing hyperbaric oxygen therapy.

<span class="mw-page-title-main">Infant respiratory distress syndrome</span> Human disease affecting newborns

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<span class="mw-page-title-main">Liquid breathing</span> Respiration of oxygen-rich liquid by a normally air-breathing organism

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2
) in the respiratory gases. Its main development has been as a monitoring tool for use during anesthesia and intensive care. It is usually presented as a graph of CO
2
(measured in kilopascals, "kPa" or millimeters of mercury, "mmHg") plotted against time, or, less commonly, but more usefully, expired volume (known as volumetric capnography). The plot may also show the inspired CO
2
, which is of interest when rebreathing systems are being used. When the measurement is taken at the end of a breath (exhaling), it is called "end tidal" CO
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(PETCO2).

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<span class="mw-page-title-main">Bronchopulmonary dysplasia</span> Medical condition

Bronchopulmonary dysplasia 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 present tend to not be mature enough to function normally. It is more common in infants with low birth weight (LBW) and those who receive prolonged mechanical ventilation to treat respiratory distress syndrome (RDS). It results in significant morbidity and mortality. The definition of BPD has continued to evolve primarily due to changes in the population, such as more survivors at earlier gestational ages, and improved neonatal management including surfactant, antenatal glucocorticoid therapy, and less aggressive mechanical ventilation.

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Apnea of prematurity is defined as cessation of breathing by a premature infant that lasts for more than 20 seconds and/or is accompanied by hypoxia or bradycardia. Apnea is traditionally classified as either obstructive, central, or mixed. Obstructive apnea may occur when the infant's neck is hyperflexed or conversely, hyperextended. It may also occur due to low pharyngeal muscle tone or to inflammation of the soft tissues, which can block the flow of air though the pharynx and vocal cords. Central apnea occurs when there is a lack of respiratory effort. This may result from central nervous system immaturity, or from the effects of medications or illness. Many episodes of apnea of prematurity may start as either obstructive or central, but then involve elements of both, becoming mixed in nature.

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<span class="mw-page-title-main">Pneumopericardium</span> Medical condition

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<span class="mw-page-title-main">Subcutaneous emphysema</span> Medical condition

Subcutaneous emphysema occurs when gas or air accumulates and seeps under the skin, where normally no gas should be present. Subcutaneous refers to the subcutaneous tissue, and emphysema refers to trapped air pockets. Since the air generally comes from the chest cavity, subcutaneous emphysema usually occurs around the upper torso, such as on the chest, neck, face, axillae and arms, where it is able to travel with little resistance along the loose connective tissue within the superficial fascia. Subcutaneous emphysema has a characteristic crackling-feel to the touch, a sensation that has been described as similar to touching warm Rice Krispies. This sensation of air under the skin is known as subcutaneous crepitation, a form of crepitus.

Alveolar capillary dysplasia (ACD) is a rare, congenital diffuse lung disease characterized by abnormal blood vessels in the lungs that cause highly elevated pulmonary blood pressure and an inability to effectively oxygenate and remove carbon dioxide from the blood. ACD typically presents in newborn babies within hours of birth as rapid and labored breathing, blue-colored lips or skin, quickly leading to respiratory failure and death. Atypical forms of ACD have been reported with initially milder symptoms and survival of many months before the onset of respiratory failure or lung transplantation.

<span class="mw-page-title-main">Emphysema</span> Medical condition

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<span class="mw-page-title-main">Smoking-related interstitial fibrosis (SRIF)</span> Abnormal amount of collagen in the lung (fibrosis) caused by cigarette smoking

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References

  1. Chalak LF, Kaiser JR, Arrington RW (2007). "Resolution of pulmonary interstitial emphysema following selective left main stem intubation in a premature newborn: an old procedure revisited". Paediatr Anaesth. 17 (2): 183–6. doi:10.1111/j.1460-9592.2006.02044.x. PMID   17238893. S2CID   21128728.
  2. 1 2 Heneghan MA, Sosulski R, Alarcon MB (1987). "Early pulmonary interstitial emphysema in the newborn: a grave prognostic sign". Clin Pediatr (Phila). 26 (7): 361–5. doi:10.1177/000992288702600707. PMID   3595042. S2CID   26285767.
  3. Morisot C, Kacet N, Bouchez MC, Rouland V, Dubos JP, Gremillet C, et al. (1990). "Risk factors for fatal pulmonary interstitial emphysema in neonates". Eur J Pediatr. 149 (7): 493–5. doi: 10.1007/bf01959402 . PMID   2347343. S2CID   23256278.
  4. Hart SM, McNair M, Gamsu HR, Price JF (1983). "Pulmonary interstitial emphysema in very low birthweight infants". Arch Dis Child. 58 (8): 612–5. doi:10.1136/adc.58.8.612. PMC   1628320 . PMID   6351760.
  5. Yu VY, Wong PY, Bajuk B, Szymonowicz W (1986). "Pulmonary interstitial emphysema in infants less than 1000 g at birth". Aust Paediatr J. 22 (3): 189–92. doi:10.1111/j.1440-1754.1986.tb00221.x. PMID   3767787. S2CID   22291777.
  6. Gaylord MS, Thieme RE, Woodall DL, Quissell BJ (1985). "Predicting mortality in low-birth-weight infants with pulmonary interstitial emphysema". Pediatrics. 76 (2): 219–24. doi:10.1542/peds.76.2.219. PMID   4022695. S2CID   38763776.