Pneumothorax

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: ; Feham Tariq, MD [2] Hamid Qazi, MD, BSc [3]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Pneumothorax was first discovered by Hippocrates, about 2400 years ago who used a metal drain for treatment. In 1803, French physician Itard was the first to coin the term “pneumothorax.” By 1952, synthetic, more flexible drains replaced metal tubes that were used for pneumothorax treatment. By 1980s, flexible and plastic drains were used that ranged between 6 and 40 French (F) in size.

• In 1803, French physician Itard was the first to coin the term “pneumothorax.”^[1]
• By 1952, synthetic, more flexible drains replaced metal tubes that were used for pneumothorax treatment.^[2]
• By 1980, flexible and plastic drains were used that ranged between 6 and 40 French (F) in size^[3]

• Pneumothorax was first discovered by Hippocrates, about 2400 years ago who used a metal drain to treat it.^[4]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Feham Tariq, MD [2], Hamid Qazi, MD, BSc [3]

Pneumothorax can be classified into tension and non-tension pneumothorax. A tension pneumothorax is an acute medical emergency as air accumulates rapidly in the pleural space with each breath. The increase in intrathoracic pressure results in massive shifting of the mediastinum away from the affected lung compressing intrathoracic vessels. Non-tension pneumothorax is of lesser severity because there is slower accumulation of air and therefore slower increase in air pressure in the pleural cavity. In primary spontaneous pneumothorax, it is usually characterized by a rupture of a bleb in the lung while secondary spontaneous pneumothorax mostly occurs due to chronic obstructive pulmonary disease (COPD). There are several diseases that may lead to secondary spontaneous pneumothorax including tuberculosis, pneumonia, asthma, cystic fibrosis, lung cancer, interstitial lung disease, and marfan’s syndrome.

Pneumothorax is divided mainly into tension and non-tension pneumothorax:^[1]

• A tension pneumothorax is an acute medical emergency as air accumulates rapidly in the pleural space with each breath. The increase in intrathoracic pressure results in massive shifting of the mediastinum away from the affected lung compressing intrathoracic vessels.
• By contrast, non-tension pneumothorax is of lesser severity because there is slower accumulation of air and therefore slower increase in air pressure in the pleural cavity.

The accumulation of blood in the thoracic cavity (hemothorax) exacerbates the problem, creating a pneumohemothorax.

• Spontaneous pneumothorax can be classified as primary spontaneous pneumothorax and secondary spontaneous pneumothorax. In primary spontaneous pneumothorax, it is usually characterized by a rupture of a bleb in the lung while secondary spontaneous pneumothorax mostly occurs due to chronic obstructive pulmonary disease (COPD).

• A primary spontaneous pneumothorax may occur without either trauma to the chest or any kind of blast injury. This type of pneumothorax is caused when a bleb (an imperfection in the lining of the lung) bursts causing the lung to deflate. If a patient suffers two or more instances of a spontaneous pneumothorax, surgeons often recommend a bullectomy and pleurectomy.
• Primary spontaneous pneumothorax is most evident to people without any previous history of lung disease and in tall, thin men whose age is between 20 to 40 years old. But it can often occur in teenagers and young adults.

• A known lung disease is present in secondary spontaneous pneumothorax.^[2] The most common cause is chronic obstructive pulmonary disease (COPD). However, there are several diseases that may lead to secondary spontaneous pneumothorax:
  • COPD
  • Tuberculosis
  • Pneumonia
  • Asthma
  • Cystic fibrosis
  • Lung cancer
  • Interstitial lung disease
  • Marfan’s syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Feham Tariq, MD [2], Hamid Qazi, MD, BSc [3]

Pneumothorax is air in the pleural space under pressure resulting in lung collapse.The pathophysiology of each type depends on the underlying disease/etiology. Primary spontaneous pneumothorax most commonly results from the bleb (small air-filled lesions under pleural surface) rupture allowing the air to leak into the pleural space. A subclass of primary spontaneous pneumothorax is isolated familial primary spontaneous pneumothorax which is genetically associated with folliculin gene mutation. Secondary spontaneous pneumothorax occurs subsequent to underlying lung pathology such as obstructive lung disease, cystic fibrosis, diffuse parenchymal lung disease and lung cancer. In tension pneumothorax, the underlying pathophysiology most commonly is chest trauma forming a one-way valve in the pleura whereby air enters the pleural space when the pleural pressure is negative during inspiration. Pneumothorax can also result from several interventional procedures which cause penetrating or non-penetrating injury to the pleura resutling in abrupt increase in the alveolar pressure and hence, lead to alveolar rupture forming a communication with the pleura.

The normal anatomy and physiology of thoracic cavity is as follows:

• Thoracic cavity is defined as the space inside the chest that contains the heart, lungs, and, several major blood vessels.^[1][2][3]

• On either side of the cavity, a pleural membrane covers the outside surface of the lung (visceral pleura) and also lines the inside of the chest wall (parietal pleura).
• The two layers are separated by a small amount of lubricating serous fluid known as the pleural fluid.
• The lungs are fully inflated within the cavity as the pressure inside the airways is higher than the pressure inside the pleural space.
• The inhaled air does not enter the pleural space as there is no natural connections between them as well as the pressure of gases in the blood stream is too low for them to be forced into the pleural space.
• The pleural pressure is negative with respect to atmospheric pressure during spontaneous breathing.
• Air can enter the pleural space through the following mechanisms:^[4][5][6]
  • Damage to the pleura or the chest wall
  • Damage to the lungs
  • Microorganisms in the pleural space

The pathophysiology of pneumothorax depends on the underlying disease causing it.

• The most common underlying pathology of primary spontaneous pneumothorax is an apical subpleural bleb (small air-filled lesions under the pleural surface).^[7]
• In addition, smoking causes inflammation and obstruction of the small airways, which is responsible for the increased risk of primary spontaneous pneumothorax in smokers.

• Pneumothorax due to underlying lung disease is secondary spontaneous pneumothorax.

• Tension pneumothorax develops when a disruption involves the visceral pleura, parietal pleura, or the tracheobronchial tree.^[8][9]

• The disruption occurs when a one-way valve forms, allowing air inflow into the pleural space, and prohibiting air outflow.
• The volume of this nonabsorbable intrapleural air increases with each inspiration.
• As a result, pressure rises within the affected hemithorax; ipsilateral lung collapses and causes hypoxia.
• Further pressure causes the mediastinum shift toward the contralateral side and compresses both, the contralateral lung and the vasculature entering the right atrium of the heart.
• This leads to worsening hypoxia and compromised venous return.

Following procedures commonly cause iatrogenic pneumothorax:^[10][11]

• Central cannulation^[12]
• Transthoracic needle aspiration
• Thoracocentesis
• Mechanical ventilation
• Thoracic acupuncture^[13][14]
• Transbronchial lung biopsy or transpleural intervention
• Intravenous drug abusers using neck veins
• Aggressive cardiopulmonary resuscitation

Mechanism of injury:

• Iatrogenic pneumothorax causes penetrating or non-penetrating injury to the pleura resulting in abrupt increase in the alveolar pressure, which can lead to alveolar rupture.
• Once the alveolus is ruptured, air enters the interstitial space and dissects toward either the visceral pleura or the mediastinum.
• A pneumothorax occurs when either the visceral or the mediastinal pleura ruptures that allows air to enter the pleural space.

The genetic association of familial primary sponatneous pneumothorax is as follows:^{[15][16][17][18][19][20]}

• Primary spontaneous pneumothorax can result as a mutation in the FLCN (folliculin) gene.
• This gene codes for a protein called folliculin.
• It is produced by the cells lining the alveoli of the lung.
• Folliculin is found in the connective tissue cells that allow the lungs to contract and expand while breathing.
• It plays a role in repairing the lung tissue after damage.
• Nonsense mutation in the folliculin gene results in isolated familial sponataneous primary pneumothorax.^[21]
• Altered folliculin protein can trigger the inflammatory process within the lung tissue that can alter and damage the tissue, resulting in blebs formation.

Pneumothorax is associated with the following conditions:^[22][23]

• Homocystinuria
• Marfan’s syndrome
  • For Marfan’s syndrome genetics, please click here.
• Iatrogenic buffalo chest syndrome

On gross pathology, pneumothorax has the following findings:^[24][25]

• Bullae or blebs on the pleural surface

• Pleural fibrosis

• Pleuropulmonary adhesions

• Airway inflammation

• The microscopic findings associated with pneumothorax are as follows:^[26]
  • Pleural fibrosis with mesothelial thickening and hyperplasia.
  • Lymphocytes, eosinophils, mesothelial thickening, and proliferation of the pleura.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Pneumothorax can occur as part of medical procedures, such as the insertion of a central venous catheter in the subclavian vein or jugular vein. While rare, it is considered a serious complication and needs immediate treatment. Other causes include mechanical ventilation, emphysema and rarely other lung diseases such as pneumonia.

Pneumothorax can occur as part of medical procedures, such as the insertion of a central venous catheter in the subclavian vein or jugular vein. While rare, it is a serious complication and needs immediate treatment. Other causes include mechanical ventilation, emphysema and rarely other lung diseases such as pneumonia.^{[1][2][3][4][5][6][7][8]}

• Emphysema
• Trauma
• Mechanical ventilation
• Marfan’s syndrome

• Female gender
• Low body weight
• Homocystinuria
• Marijuana smoking

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Pneumothorax must be differentiated from other diseases that cause shortness of breath, difficulty breathing, and chest pain, such as [differential dx1], [differential dx2], and [differential dx3].

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

The incidence of primary spontaneous pneumothorax is approximately 7.4-18 per 100,000 individuals in males and approximately 1.2-6.0 per 100,000 individuals in females in USA. Patients of all age groups may develop pneumothorax. There is no racial predilection to pneumothorax. Males are more commonly affected by pneumothorax than females. The male to female ratio is approximately 3 to 1.

The epidemiology and demographics of pneumothorax are as follows:^[1][2]

• The incidence of primary spontaneous pneumothorax is approximately 7.4-18 per 100,000 individuals in males in USA.
• The incidence of primary spontaneous pneumothorax is approximately 1.2-6.0 per 100,000 individuals in females in USA.

• Incidence of males to females ratio is 6.3 to 2.0 per 100,000

• Patients of all age groups may develop pneumothorax.

• There is no racial predilection to pneumothorax.

• Males are more commonly affected by pneumothorax than females. The male to female ratio is approximately 3 to 1.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2],Feham Tariq, MD [3]

Common risk factors in the development of pneumothorax include smoking, underlying lung pathology such as obstructive lung diseases, female gender, thin and tall men, mechanical ventilation, low body weight, Marfan’s syndrome, and homocystinuria.

The risk factors for the development of pneumothorax include:^{[1][2][3][4][5][6][7][8]}

• Smoking
• Thin, lean, and tall population
• Family history
• Emphysema
• Trauma
• Mechanical ventilation
• Female gender
• Low body weight
• Marijuana smoking
• Marfans syndrome
• Homocystinuria
• Pulmonary tuberculosis

• Thoracic endometriosis
• Pneumocystis carinii
• Pneumonia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

There is insufficient evidence to recommend routine screening for pneumothorax.

• There is insufficient evidence to recommend routine screening for pneumothorax.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

The prognosis varies with the cause of pneumothorax; primary spontaneous pneumothorax have the most favorable prognosis. The symptoms of pneumothorax usually develop in any decade of life, and start with symptoms such as difficulty breathing, shortness of breath, and chest pain. Uncomplicated pneumothorax usually resolve within 10 days. Secondary pneumothorax is dependent on the underlying cause and can continue to reoccur. If tension pneumothorax is not recognized it will lead to death. Common complications of pneumothorax include recurrence, cardiovascular collapse, and pneumo-mediastinum.

• The symptoms of pneumothorax usually develop in any decade of life, and start with symptoms such as difficulty breathing, shortness of breath, and chest pain.
• Uncomplicated pneumothorax usually resolve within 10 days.
• Recurrence occurs within 6 months.^[1]

• If left untreated, patients with tension pneumothorax may progress to develop death.

• Uncomplicated pneumothorax usually resolve within 10 days.
• Recurrence occurs within 6 months.^[1]

• If left untreated, patients with tension pneumothorax may progress to develop death.

• Common complications of pneumothorax include:^[2]
  • Recurrence^[3]
  • Cardiovascular collapse
  • Pneumomediastinum^[4]

The prognosis of pneumothorax is as follows:^[5]

• The prognosis varies with the cause of pneumothorax; primary spontaneous pneumothorax have the most favorable prognosis.^[2]

• Primary spontaneous pneumothorax resolves within 10 days without treatment.
• Secondary pneumothorax is dependant on the underlying cause and can continue to reoccur.
• If tension pneumothorax is not recognized it will lead to death.

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