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It is most commonly caused by:
- Oesophageal rupture, for example in Boerhaave syndrome
- Asthma or other conditions leading to alveolar rupture
- Bowel rupture, where air in the abdominal cavity tracts up into the chest.
It has also been associated with:
- "Mycoplasma pneumoniae" pneumonia
- obesity
It can be induced to assist thoracoscopic surgery. It can be caused by a pulmonary barotrauma resulting when a person moves to or from a higher pressure environment, such as when a SCUBA diver, a free-diver or an airplane passenger ascends or descends.
In rare cases, pneumomediastinum may also arise as a result of blunt chest trauma (e.g. car accidents, fights, over pressure of breathing apparatus), while still evolving in the same fashion as the spontaneous form.
Pneumomediastinum is most commonly seen in otherwise healthy young male patients and may not be prefaced by a relevant medical history of similar ailments.
There is still much debate to whether pulmonary sequestration is a congenital problem or acquired through reccurent pulmonary infection. It is widely believed that extralobar pulmonary sequestrations are a result of prenatal pulmonary malformation while intralobar pulmonary sequestrations can develop due to reccurent pulmonary infections in adolescents and young adults.
Failure to have a pulmonary sequestration removed can lead to a number of complications. These include:
- Hemorrhage that can be fatal.
- The creation of a left-right shunt, where blood flows in a shortcut through the feed off the aorta.
- Chronic infection. Diseases such as bronchiectasis, tuberculosis, aspergillosis, bronchial carcinoid and bronchogenic squamous cell carcinoma.
Pneumomediastinum (from Greek "pneuma" – "air", also known as mediastinal emphysema) is (abnormal presence of air or other gas) in the mediastinum. First described in 1819 by René Laennec, the condition can result from physical trauma or other situations that lead to air escaping from the lungs, airways, or bowel into the chest cavity.
Respiratory disease is a common and significant cause of illness and death around the world. In the US, approximately 1 billion "common colds" occur each year. A study found that in 2010, there were approximately 6.8 million emergency department visits for respiratory disorders in the U.S. for patients under the age of 18. In 2012, respiratory conditions were the most frequent reasons for hospital stays among children.
In the UK, approximately 1 in 7 individuals are affected by some form of chronic lung disease, most commonly chronic obstructive pulmonary disease, which includes asthma, chronic bronchitis and emphysema.
Respiratory diseases (including lung cancer) are responsible for over 10% of hospitalizations and over 16% of deaths in Canada.
In 2011, respiratory disease with ventilator support accounted for 93.3% of ICU utilization in the United States.
The mediastinum (from Medieval Latin "mediastinus", "midway") is the central compartment of the thoracic cavity surrounded by loose connective tissue, as an undelineated region that contains a group of structures within the thorax. The mediastinum contains the heart and its vessels, the esophagus, trachea, phrenic and cardiac nerves, the thoracic duct, thymus and lymph nodes of the central chest.
Pulmonary diseases may also impact newborns, such as pulmonary hyperplasia, pulmonary interstitial emphysema (usually preterm births), and infant respiratory distress syndrome,
Congenital diaphragmatic hernia has a mortality rate of 40–62%, with outcomes being more favorable in the absence of other congenital abnormalities. Individual rates vary greatly dependent upon multiple factors: size of hernia, organs involved, additional birth defects, and/or genetic problems, amount of lung growth, age and size at birth, type of treatments, timing of treatments, complications (such as infections) and lack of lung function.
Pneumopericardium is a medical condition where air enters the pericardial cavity. This condition has been recognized in preterm neonates, in which it is associated with severe lung pathology, after vigorous resuscitation, or in the presence of assisted ventilation. This is a serious complication, which if untreated may lead to cardiac tamponade and death. Pneumomediastinum, which is the presence of air in the mediastinum, may mimic and also coexist with pneumopericardium.
It can be congenital, or introduced by a wound.
Pulmonary hypoplasia is incomplete development of the lungs, resulting in an abnormally low number or size of bronchopulmonary segments or alveoli. A congenital malformation, it most often occurs secondary to other fetal abnormalities that interfere with normal development of the lungs. Primary (idiopathic) pulmonary hypoplasia is rare and usually not associated with other maternal or fetal abnormalities.
Incidence of pulmonary hypoplasia ranges from 9–11 per 10,000 live births and 14 per 10,000 births. Pulmonary hypoplasia is a relatively common cause of neonatal death. It also is a common finding in stillbirths, although not regarded as a cause of these.
The mechanism responsible for pneumopericardium is the ‘Macklin effect’ – There is initially an increased pressure gradient between the alveoli and the interstitial space. Increased pressure leads to alveolar rupture, resulting in air getting through to the pericapillary interstitial pulmonary space. This space is continuous with the peribronchial and pulmonary perivascular sheaths. From here, the air tracks to the hilum of the lung and then to the mediastinum. In case of a pericardial tear, this air enters the pericardial cavity and pneumopericardium develops. The condition may remain asymptomatic or may progress to life-threatening conditions like tension pneumopericardium or cardiac tamponade.
Causes of pulmonary hypoplasia include a wide variety of congenital malformations and other conditions in which pulmonary hypoplasia is a complication. These include congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, fetal hydronephrosis, caudal regression syndrome, mediastinal tumor, and sacrococcygeal teratoma with a large component inside the fetus. Large masses of the neck (such as cervical teratoma) also can cause pulmonary hypoplasia, presumably by interfering with the fetus's ability to fill its lungs. In the presence of pulmonary hypoplasia, the EXIT procedure to rescue a baby with a neck mass is not likely to succeed.
Fetal hydrops can be a cause, or conversely a complication.
Pulmonary hypoplasia is associated with oligohydramnios through multiple mechanisms. Both conditions can result from blockage of the urinary bladder. Blockage prevents the bladder from emptying, and the bladder becomes very large and full. The large volume of the full bladder interferes with normal development of other organs, including the lungs. Pressure within the bladder becomes abnormally high, causing abnormal function in the kidneys hence abnormally high pressure in the vascular system entering the kidneys. This high pressure also interferes with normal development of other organs. An experiment in rabbits showed that PH also can be caused directly by oligohydramnios.
Pulmonary hypoplasia is associated with dextrocardia of embryonic arrest in that both conditions can result from early errors of development, resulting in Congenital cardiac disorders.
PH is a common direct cause of neonatal death resulting from pregnancy induced hypertension.
The death rate of people with flail chest depends on the severity of their condition, ranging from 10 to 25%.
Baylor College of Medicine in Houston, Texas has conducted ACD research since 2001.
Congenital diaphragmatic hernia (CDH) is a birth defect of the diaphragm. The most common type of CDH is a Bochdalek hernia; other types include Morgagni hernia, diaphragm eventration and central tendon defects of the diaphragm. Malformation of the diaphragm allows the abdominal organs to push into the chest cavity, hindering proper lung formation.
CDH is a life-threatening pathology in infants and a major cause of death due to two complications: pulmonary hypoplasia and pulmonary hypertension. Experts disagree on the relative importance of these two conditions, with some focusing on hypoplasia, others on hypertension. Newborns with CDH often have severe respiratory distress which can be life-threatening unless treated appropriately.
The mediastinum is the cavity that separates the lungs from the rest of the chest. It contains the heart, esophagus, trachea, thymus, and aorta. The mediastinum has three main parts: the anterior mediastinum (front), the middle mediastinum, and the posterior mediastinum (back).
The most common mediastinal masses are neurogenic tumors (20% of mediastinal tumors), usually found in the posterior mediastinum, followed by thymoma (15-20%) located in the anterior mediastinum.
Masses in the anterior portion of the mediastinum can include thymoma, lymphoma, pheochromocytoma, germ cell tumors including teratoma, thyroid tissue, and parathyroid lesions. Masses in this area are more likely to be malignant than those in other compartments.
Masses in the posterior portion of the mediastinum tend to be neurogenic in origin, and in adults tend to be of neural sheath origin including neurilemomas and neurofibromas.
Lung cancer typically spreads to the lymph nodes in the mediastinum.
Lymphangiomatosis can occur at any age, but the incidence is highest in children and teenagers. Signs and symptoms are typically present before the age of 20 and the condition is often under-recognized in adults.
It affects males and females of all races and exhibits no inheritance pattern. The medical literature contains case reports from every continent.
Because it is so rare, and commonly misdiagnosed, it is not known exactly how many people are affected by this disease.
Approximately 1 out of 13 people admitted to the hospital with fractured ribs are found to have flail chest.
Lymphangiomatosis (LYMF) is a condition where a lymphangioma is not present in a single localised mass, but in a widespread or multifocal manner. It is a rare type of tumor which results from an abnormal development of the lymphatic system.
It is thought to be the result of congenital errors of lymphatic development occurring prior to the 20th week of gestation. Lymphangiomatosis is a condition marked by the presence of cysts that result from an increase both in the size and number of thin-walled lymphatic channels that are abnormally interconnected and dilated. 75% of cases involve multiple organs. It typically presents by age 20 and, although it is technically benign, these deranged lymphatics tend to invade surrounding tissues and cause problems due to invasion and/or compression of adjacent structures. The condition is most common in the bones and lungs and shares some characteristics with Gorham’s disease. Up to 75% of patients with lymphangiomatosis have bone involvement, leading some to conclude that lymphangiomatosis and Gorham’s disease should be considered as a spectrum of disease rather than separate diseases. When it occurs in the lungs, lymphangiomatosis has serious consequences and is most aggressive in the youngest children. When the condition extends into the chest it commonly results in the accumulation of chyle in the linings of the heart and/or lungs.
Chyle is composed of lymph fluid and fats that are absorbed from the small intestine by specialized lymphatic vessels called lacteals during digestion. The accumulations are described based on location: chylothorax is chyle in the chest; chylopericardium is chyle trapped inside the sack surrounding the heart; chyloascites is chyle trapped in the linings of the abdomen and abdominal organs. The presence of chyle in these places accounts for many of the symptoms and complications associated with both lymphangiomatosis and Gorham’s disease. The incidence of lymphangiomatosis is unknown and it is often misdiagnosed. It is separate and distinct from lymphangiectasis, lymphangioleiomyomatosis (LAM), pulmonary capillary hemangiomatosis, Kaposi’s sarcoma, and kaposiform hemangioendothelioma. Its unusual nature makes lymphangiomatosis (and Gorham’s disease) a diagnostic and therapeutic challenge. A multidisciplinary approach is generally necessary for optimal diagnosis and symptom management. The term literally means lymphatic system (lymph) vessel (angi) tumor or cyst (oma) condition (tosis).
Bochdalek hernia can be a life-threatening condition. Approximately 85.3% of newborns born with a Bochdalek hernia are immediately high risk. Infants born with a Bochdalek hernia have a "high mortality rate due to respiratory insufficiency". Between 25–60% of infants with a Bochdalek hernia die. The lungs, diaphragm, and digestive system are all forming at the same time, so when a Bochdalek hernia permits the abdominal organs to invade the chest cavity rather than remain under the diaphragm in the correct position, it puts the infant in critical condition. These "foreign bodies" in the chest cavity compress the lungs, impairing their proper development and causing pulmonary hypoplasia. Since the lungs of infants suffering from a Bochdalek hernia have fewer alveoli than normal lungs, Bochdalek hernias are life-threatening conditions due to respiratory distress. Also, if the invasion of the intestine or stomach punctures the lung, then the lungs cannot fill completely with air. The baby will not be healthy or stable with this condition because he or she cannot take in enough air and oxygen to keep the body operating properly. Like the lungs, the intestines may also have trouble developing correctly. If the intestines are trapped within the lungs, then the lungs and intestines may not be receiving the amount of blood they need to stay healthy and function properly.
Bochdalek hernias make up about 0.17% to 6% of all diaphragmatic hernia cases and about one in every 2200 to 12,500 births every year. Babies who are born with a Bochdalek hernia are more than likely to have another birth defect caused by the hernia. About twenty percent of those children born with a Bochdalek hernia, also have a congenital heart defect. In addition, infants born with this condition may also have other abnormalities. "Between five and sixteen [percent of infants] have a chromosomal abnormality." In most cases, left-sided hernias or Bochdalek hernias have a ratio of 3:2 of males to females. In other words, Bochdalek hernias are more common in men.
Injury to the lung may also cause pulmonary edema through injury to the vasculature and parenchyma of the lung. The acute lung injury-acute respiratory distress syndrome (ALI-ARDS) covers many of these causes, but they may include:
- Inhalation of hot or toxic gases
- Pulmonary contusion, i.e., high-energy trauma (e.g. vehicle accidents)
- Aspiration, e.g., gastric fluid
- Reexpansion, i.e. post large volume thoracocentesis, resolution of pneumothorax, post decortication, removal of endobronchial obstruction, effectively a form of negative pressure pulmonary oedema.
- Reperfusion injury, i.e. postpulmonary thromboendartectomy or lung transplantation
- Swimming induced pulmonary edema also known as immersion pulmonary edema
- Transfusion Associated Circulatory Overload (TACO) occurs when multiple blood transfusions or blood-products (plasma, platelets, etc.) are transfused over a short period of time.
- Transfusion associated Acute Lung Injury (TRALI) is a specific type of blood-product transfusion injury that occurs when the donors plasma contained antibodies against the donor, such as anti-HLA or anti-neutrophil antibodies.
- Severe infection or inflammation which may be local or systemic. This is the classical form of ALI-ARDS.
Some causes of pulmonary edema are less well characterised and arguably represent specific instances of the broader classifications above.
- Arteriovenous malformation
- Hantavirus pulmonary syndrome
- High altitude pulmonary edema (HAPE)
- Envenomation, such as with the venom of Atrax robustus
"Widened mediastinum/mediastinal widening" is where the mediastinum has a width greater than 6 cm on an upright PA chest X-ray or 8 cm on supine AP chest film.
A widened mediastinum can be indicative of several pathologies:
- aortic aneurysm
- aortic dissection
- aortic unfolding
- aortic rupture
- hilar lymphadenopathy
- anthrax inhalation - a widened mediastinum was found in 7 of the first 10 victims infected by anthrax ("Bacillus anthracis") in 2001.
- esophageal rupture - presents usually with pneumomediastinum and pleural effusion. It is diagnosed with water-soluble swallowed contrast.
- mediastinal mass
- mediastinitis
- cardiac tamponade
- pericardial effusion
- thoracic vertebrae fractures in trauma patients.
Mediastinal fibrosis most common cause is idiopathic mediastinal fibrosis; less commonly histoplasmosis tuberculosis or unknown. It is characterized by invasive, calcified fibrosis centered on lymph nodes that block major vessels and airways. In Europe, this disease is exceptionally rare. More cases are seen
in USA where the disease may often be associated with histoplasmosis.
The following are causes of BHL:
- Sarcoidosis
- Infection
- Tuberculosis
- Fungal infection
- Mycoplasma
- Intestinal Lipodystrophy (Whipple's disease)
- Malignancy
- Lymphoma
- Carcinoma
- Mediastinal tumors
- Inorganic dust disease
- Silicosis
- Berylliosis
- Extrinsic allergic alveolitis
- Such as bird fancier's lung
- Less common causes also exist:
- Eosinophilic granulomatosis with polyangiitis
- Human immunodeficiency virus
- Extrinsic allergic alveolitis
- Adult-onset Still's disease