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A pneumatocele results when a lung laceration, a cut or tear in the lung tissue, fills with air. A rupture of a small airway creates the air-filled cavity. Pulmonary lacerations that fill with blood are called pulmonary hematomas. In some cases, both pneumatoceles and hematomas exist in the same injured lung. A pneumatocele can become enlarged, for example when the patient is mechanically ventilated or has acute respiratory distress syndrome, in which case it may not go away for months.
A pneumatocele is a cavity in the lung parenchyma filled with air that may result from pulmonary trauma during mechanical ventilation.
As with other chest injuries such as pulmonary contusion, hemothorax, and pneumothorax, pulmonary laceration can often be treated with just supplemental oxygen, ventilation, and drainage of fluids from the chest cavity. A thoracostomy tube can be used to remove blood and air from the chest cavity. About 5% of cases require surgery, called thoracotomy. Thoracotomy is especially likely to be needed if a lung fails to re-expand; if pneumothorax, bleeding, or coughing up blood persist; or in order to remove clotted blood from a hemothorax. Surgical treatment includes suturing, stapling, oversewing, and wedging out of the laceration. Occasionally, surgeons must perform a lobectomy, in which a lobe of the lung is removed, or a pneumonectomy, in which an entire lung is removed.
A pulmonary laceration can cause air to leak out of the lacerated lung and into the pleural space, if the laceration goes through to it. Pulmonary laceration invariably results in pneumothorax (due to torn airways), hemothorax (due to torn blood vessels), or a hemopneumothorax (with both blood and air in the chest cavity). Unlike hemothoraces that occur due to pulmonary contusion, those due to lung laceration may be large and long lasting. However, the lungs do not usually bleed very much because the blood vessels involved are small and the pressure within them is low. Therefore, pneumothorax is usually more of a problem than hemothorax. A pneumothorax may form or be turned into a tension pneumothorax by mechanical ventilation, which may force air out of the tear in the lung.
The laceration may also close up by itself, which can cause it to trap blood and potentially form a cyst or hematoma. Because the lung is elastic, the tear forms a round cyst called a "traumatic air cyst" that may be filled with air, blood, or both and that usually shrinks over a period of weeks or months. Lacerations that are filled with air are called pneumatoceles, and those that are filled with blood are called pulmonary hematomas. In some cases, both pneumatoceles and hematomas exist in the same injured lung. A pneumatocele can become enlarged, for example when the patient is mechanically ventilated or has acute respiratory distress syndrome, in which case it may not go away for months. Pulmonary hematomas take longer to heal than simple pneumatoceles and commonly leave the lungs scarred.
Over time, the walls of lung lacerations tend to grow thicker due to edema and bleeding at the edges.
A pulmonary hematoma is a collection of blood within the tissue of the lung. It may result when a pulmonary laceration fills with blood. A lung laceration filled with air is called a pneumatocele. In some cases, both pneumatoceles and hematomas exist in the same injured lung. Pulmonary hematomas take longer to heal than simple pneumatoceles and commonly leave the lungs scarred. A pulmonary contusion is another cause of bleeding within the lung tissue, but these result from microhemorrhages, multiple small bleeds, and the bleeding is not a discrete mass but rather occurs within the lung tissue. An indication of more severe damage to the lung than pulmonary contusion, a hematoma also takes longer to clear. Unlike contusions, hematomas do not usually interfere with gas exchange in the lung, but they do increase the risk of infection and abscess formation.
"Fire-eater's lung" is an important variant of hydrocarbon pneumonitis, which typically involves adolescents or young adults who are exposed through mishap during flame-blowing performances using a variety of different flammable materials. The substances used overlap with some of the pediatric exposures (kerosene, gasoline) but can also include other hydrocarbons such as jet fuel and, in France, an aromatic hydrocarbon enriched petroleum-distillate called "kerdan". There has also been a case of citronella oil aspiration in a fire-eater. As with hydrocarbon pneumonitis in children, fire-eater's lung can also be complicated by pneumatocele. Although the term "acute lipoid pneumonia" has been used to refer to the "fire-eater's lung" syndrome, this is a misnomer.
Hydrocarbon pneumonitis is a kind of chemical pneumonitis which occurs with oral ingestion of hydrocarbons and associated aspiration. It occurs prominently among children, accounting for many hospital admissions each year. Common hydrocarbons involved are mineral spirits, mineral seal oil (common in furniture polish), lamp oil, kerosene (paraffin), turpentine (pine oil), gasoline, and lighter fluid. Pneumatocele is a complication of hydrocarbon pneumonitis. In both childhood and adult pneumonitis, hydrocarbon aspiration occurs at the time of initial ingestion event or subsequently with vomiting. Low viscosity of an ingested hydrocarbon is considered a major factor promoting aspiration (presumably for mechanical reasons). Contrary to aspiration hydrocarbon pneumonitis, hydrocarbon (solvent) vapor inhalation manifests primarily in either central nervous system or cardiac effects.
Children with DOCK8 deficiency do not tend to live long; sepsis is a common cause of death at a young age. CNS and vascular complications are other common causes of death.
DOCK8 deficiency is very rare, estimated to be found in less than one person per million; there have been 32 patients diagnosed as of 2012.