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When a pleural effusion has been determined to be exudative, additional evaluation is needed to determine its cause, and amylase, glucose, pH and cell counts should be measured.
- Red blood cell counts are elevated in cases of bloody effusions (for example after heart surgery or hemothorax from incomplete evacuation of blood).
- Amylase levels are elevated in cases of esophageal rupture, pancreatic pleural effusion, or cancer.
- Glucose is decreased with cancer, bacterial infections, or rheumatoid pleuritis.
- pH is low in empyema (<7.2) and may be low in cancer.
- If cancer is suspected, the pleural fluid is sent for cytology. If cytology is negative, and cancer is still suspected, either a thoracoscopy, or needle biopsy of the pleura may be performed.
- Gram staining and culture should also be done.
- If tuberculosis is possible, examination for "Mycobacterium tuberculosis" (either a Ziehl–Neelsen or Kinyoun stain, and mycobacterial cultures) should be done. A polymerase chain reaction for tuberculous DNA may be done, or adenosine deaminase or interferon gamma levels may also be checked.
The most common causes of exudative pleural effusions are bacterial pneumonia, cancer (with lung cancer, breast cancer, and lymphoma causing approximately 75% of all malignant pleural effusions), viral infection, and pulmonary embolism.
Another common cause is after heart surgery, when incompletely drained blood can lead to an inflammatory response that causes exudative pleural fluid.
Conditions associated with exudative pleural effusions:
- Parapneumonic effusion due to pneumonia
- Malignancy (either lung cancer or metastases to the pleura from elsewhere)
- Infection (empyema due to bacterial pneumonia)
- Trauma
- Pulmonary infarction
- Pulmonary embolism
- Autoimmune disorders
- Pancreatitis
- Ruptured esophagus (Boerhaave's syndrome)
- Rheumatoid pleurisy
- Drug-induced lupus
Tumor-like disorders of the lung pleura are a group of conditions that on initial radiological studies might be confused with malignant lesions. Radiologists must be aware of these conditions in order to avoid misdiagnosing patients. Examples of such lesions are: pleural plaques, thoracic splenosis, catamenial pneumothorax, pleural pseudotumor, diffuse pleural thickening, diffuse pulmonary lymphangiomatosis and Erdheim-Chester Disease.
The most common causes of transudative pleural effusions in the United States are heart failure and cirrhosis. Nephrotic syndrome, leading to the loss of large amounts of albumin in urine and resultant low albumin levels in the blood and reduced colloid osmotic pressure, is another less common cause of pleural effusion. Pulmonary emboli were once thought to cause transudative effusions, but have been recently shown to be exudative.
The mechanism for the exudative pleural effusion in pulmonary thromboembolism is probably related to increased permeability of the capillaries in the lung, which results from the release of cytokines or inflammatory mediators (e.g. vascular endothelial growth factor) from the platelet-rich blood clots. The excessive interstitial lung fluid traverses the visceral pleura and accumulates in the pleural space.
Conditions associated with transudative pleural effusions include:
- Congestive heart failure
- Liver cirrhosis
- Severe hypoalbuminemia
- Nephrotic syndrome
- Acute atelectasis
- Myxedema
- Peritoneal dialysis
- Meigs' syndrome
- Obstructive uropathy
- End-stage kidney disease
Ectopic endometrial tissue reaches the pleural space of the lung or the right hemi-diaphragmatic region and erodes the visceral pleura, causing the formation of a spontaneous pneumothorax. The condition is often cyclical, due to its associations with the beginning of the menstrual cycle.
Affected persons usually present with recurrent spontaneous pneumothorax associated with the onset of the menstrual cycle. Additionally, chest/scapular pain and/or evidence of endometriosis in the abdominopelvic cavity are other manifestations.
On radiological studies, pneumothorax is visualized using conventional chest x-rays and CT scans. In 90% of the cases, the pneumothorax is located on the right side. In some cases, small nodules can be seen in the pleura using CT scans. Confirmation can be done using video assisted thoracoscopic surgery (VATS).
Treatment for the pneumothorax is with chest tube placement. As for the ectopic endometrial tissue, therapy with gonadotropin-releasing–hormone or resection of the lesions can improve symptoms.
Asbestos can cause lung cancer that is identical to lung cancer from other causes. Exposure to asbestos is associated with all major histological types of lung carcinoma (adenocarcinoma, squamous cell carcinoma, large-cell carcinoma and small-cell carcinoma). The latency period between exposure and development of lung cancer is 20 to 30 years. It is estimated that 3%-8% of all lung cancers are related to asbestos. The risk of developing lung cancer depends on the level, duration, and frequency of asbestos exposure (cumulative exposure). Smoking and individual susceptibility are other contributing factors towards lung cancer. Smokers who have been exposed to asbestos are at far greater risk of lung cancer. Smoking and asbestos exposure have a multiplicative (synergistic) effect on the risk of lung cancer. Symptoms include chronic cough, chest pain, breathlessness, haemoptysis (coughing up blood), wheezing or hoarseness of the voice, weight loss and fatigue. Treatment involves surgical removal of the cancer, chemotherapy, radiotherapy, or a combination of these (multimodality treatment). Prognosis is generally poor unless the cancer is detected in its early stages. Out of all patients diagnosed with lung cancer, only 15% survive for five years after diagnosis.
Fibrothorax is diffuse fibrosis of the pleural space surrounding the lungs. It can have several causes including hemothorax, pleural effusion and tuberculosis. It may also be induced by exposure to certain substances, as with asbestos-induced diffuse pleural fibrosis. Idiopathic fibrothorax may also occur.
In fibrothorax, scar tissue is formed around the visceral pleura following inflammation due to pleural effusion or other pathology. The scar tissue lies in a sheet between the pleura, then fuses with the parietal pleura and the chest wall. Over time, generally the course of years, the fibrotic scar tissue slowly tightens, which results in the contraction of the entire hemithorax, and leaves the ribs immobilized. Within the chest, the lung is compressed and unable to expand, making it vulnerable to collapse. At the microscopic level, the scar tissue is composed of collagen fibers deposited in a basket weave pattern. The treatment for fibrothorax is decortication, the surgical removal of the fibrous layer of scar tissue. However, since many of the diseases and conditions resulting in fibrothorax are treatable, prevention remains the preferred method of managing fibrothorax.
Secondary spontaneous pneumothorax occurs in the setting of a variety of lung diseases. The most common is chronic obstructive pulmonary disease (COPD), which accounts for approximately 70% of cases. Known lung diseases that may significantly increase the risk for pneumothorax are
In children, additional causes include measles, echinococcosis, inhalation of a foreign body, and certain congenital malformations (congenital cystic adenomatoid malformation and congenital lobar emphysema).
11.5% of people with a spontaneous pneumothorax have a family member who has previously experienced a pneumothorax. The hereditary conditions – Marfan syndrome, homocystinuria, Ehlers–Danlos syndrome, alpha 1-antitrypsin deficiency (which leads to emphysema), and Birt–Hogg–Dubé syndrome—have all been linked to familial pneumothorax. Generally, these conditions cause other signs and symptoms as well, and pneumothorax is not usually the primary finding. Birt–Hogg–Dubé syndrome is caused by mutations in the "FLCN" gene (located at chromosome 17p11.2), which encodes a protein named folliculin. "FLCN" mutations and lung lesions have also been identified in familial cases of pneumothorax where other features of Birt–Hogg–Dubé syndrome are absent. In addition to the genetic associations, the HLA haplotype AB is also a genetic predisposition to PSP.
Spontaneous pneumothoraces are divided into two types: "primary", which occurs in the absence of known lung disease, and "secondary", which occurs in someone with underlying lung disease. The cause of primary spontaneous pneumothorax is unknown, but established risk factors include male sex, smoking, and a family history of pneumothorax. Smoking either cannabis or tobacco increases the risk. The various suspected underlying mechanisms are discussed below.
Its cause is usually traumatic, from a blunt or penetrating injury to the thorax, resulting in a rupture of the serous membrane either lining the thorax or covering the lungs. This rupture allows blood to spill into the pleural space, equalizing the pressures between it and the lungs. Blood loss may be massive in people with these conditions, as each side of the thorax can hold 30 to 40% of a person's blood volume or 1.5 to 2 L per side in the average adult. Even minor injury to the chest wall can lead to significant hemothorax.
Less frequently, hemothorax occurs spontaneously. A major vascular cause of hemothorax is aortic dissection or rupture of thoracic aortic aneurysms. It may also follow surgical intervention in the thoracic area. Infrequently, patients with pneumothorax may develop spontaneous hemothorax. Spontaneous hemothorax or hemopneumothorax may be occur with endometriosis, if endometrial tissue implants on the pleural surface, then bleeds in response to cyclical hormonal changes in menstruating women.
If left untreated, the condition can progress to a point where the blood accumulation begins to put pressure on the mediastinum and the trachea, effectively limiting the amount that the heart's ventricles are able to fill. The condition can cause the trachea to deviate, or move, toward the unaffected side.
Some sources claim this entity represents 3-6% of pneumothorax in women. In regard of the low incidence of primary spontaneous pneumothorax ("i.e." not due to surgical trauma "etc.") in women (about 1/100'000/year), this is a very rare condition. Hence, many basic textbooks do not mention it, and many doctors have never heard of it. Therefore, catamenial pneumothorax is probably under-recognized.
Pneumothorax can be a medical emergency, as it can become associated with decreased lung function, and if progressed to tension pneumothorax, potentially fatal. A chest tube should be inserted after clinical assessment. This releases the air and menstrual blood, and the lung can re-expand.
Surgery, hormonal treatments and combined approaches have all been proposed, with variable results in terms of short and long term outcome. Surgical removal of the endometrial tissue should be endeavoured during menstruation for optimal visualisation of the cyst. Pleurodesis may also be helpful. Menstruation and accompanying lung collapse can be suppressed with hormone therapy, like with Lupron Depot, danazol or extended cycle combined oral contraceptive pills.
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.
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.
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.
Malignant pleural effusion is a condition in which cancer causes an abnormal amount of fluid to collect between the thin layers of tissue (pleura) lining the outside of the lung and the wall of the chest cavity. Lung cancer and breast cancer account for about 50-65% of malignant pleural effusions. Other common causes include pleural mesothelioma and lymphoma.
Working with asbestos is the most common risk factor for mesothelioma. However, mesothelioma has been reported in some individuals without any known exposure to asbestos.
The most common cause is post-surgical atelectasis, characterized by splinting, i.e. restricted breathing after abdominal surgery.
Another common cause is pulmonary tuberculosis. Smokers and the elderly are also at an increased risk. Outside of this context, atelectasis implies some blockage of a bronchiole or bronchus, which can be within the airway (foreign body, mucus plug), from the wall (tumor, usually squamous cell carcinoma) or compressing from the outside (tumor, lymph node, tubercle). Another cause is poor surfactant spreading during inspiration, causing the surface tension to be at its highest which tends to collapse smaller alveoli. Atelectasis may also occur during suction, as along with sputum, air is withdrawn from the lungs. There are several types of atelectasis according to their underlying mechanisms or the distribution of alveolar collapse; resorption, compression, microatelectasis and contraction atelectasis.
A Simon focus is a tuberculosis (TB) nodule that can form in the apex of the lung when a primary TB infection elsewhere in the body spreads to the lung apex via the bloodstream. Simon focus nodules are often calcified.
The initial lesion is usually a small focus of consolidation, less than 2cm in diameter and located within 1 to 2 cm of the apical pleura. In adolescence, Simon foci may become reactivated and develop into Assmann foci. Such foci are sharply circumscribed, firm, gray-white to yellow areas that have a variable amount of central caseation and peripheral fibrosis.
Air in subcutaneous tissue does not usually pose a lethal threat; small amounts of air are reabsorbed by the body. Once the pneumothorax or pneumomediastinum that causes the subcutaneous emphysema is resolved, with or without medical intervention, the subcutaneous emphysema will usually clear. However, spontaneous subcutaneous emphysema can, in rare cases, progress to a life-threatening condition, and subcutaneous emphysema due to mechanical ventilation may induce ventilatory failure.
The goal of treatment of malignant pleural effusions is relief of breathlessness. Occasionally, treatment of the underlying cancer can cause resolution of the effusion. This may be the case with types of cancer that respond well to chemotherapy, such as small cell carcinoma or lymphoma. Simple aspiration of pleural fluid can relieve breathlessness rapidly but fluid and symptoms will usually recur within a couple of weeks. For this reason, more permanent treatments are usually used to prevent fluid recurrence. Standard treatment involves chest tube insertion and pleurodesis. However, this treatment requires an inpatient stay of approximately 2–7 days, can be painful and has a significant failure rate. This has led to the development of tunneled pleural catheters (e.g., Pleurx Catheters), which allow outpatient treatment of effusions.
Subcutaneous emphysema is a common result of certain types of surgery; for example it is not unusual in chest surgery. It may also occur from surgery around the esophagus, and is particularly likely in prolonged surgery. Other potential causes are positive pressure ventilation for any reason and by any technique, in which its occurrence is frequently unexpected. It may also occur as a result of oral surgery, laparoscopy, and cricothyrotomy. In a pneumonectomy, in which an entire lung is removed, the remaining bronchial stump may leak air, a rare but very serious condition that leads to progressive subcutaneous emphysema. Air can leak out of the pleural space through an incision made for a thoracotomy to cause subcutaneous emphysema. On infrequent occasions, the condition can result from dental surgery, usually due to use of high-speed tools that are air driven. These cases result in usually painless swelling of the face and neck, with an immediate onset, the crepitus (crunching sound) typical of subcutaneous emphysema, and often with subcutaneous air visible on X-ray.
One of the main causes of subcutaneous emphysema, along with pneumothorax, is an improperly functioning chest tube. Thus subcutaneous emphysema is often a sign that something is wrong with a chest tube; it may be clogged, clamped, or out of place. The tube may need to be replaced, or, when large amounts of air are leaking, a new tube may be added.
Since mechanical ventilation can worsen a pneumothorax, it can force air into the tissues; when subcutaneous emphysema occurs in a ventilated patient, it is an indication that the ventilation may have caused a pneumothorax. It is not unusual for subcutaneous emphysema to result from positive pressure ventilation. Another possible cause is a ruptured trachea. The trachea may be injured by tracheostomy or tracheal intubation; in cases of tracheal injury, large amounts of air can enter the subcutaneous space. An endotracheal tube can puncture the trachea or bronchi and cause subcutaneous emphysema.
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.
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.
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.