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A bronchopleural fistula (BPF) is a fistula between the pleural space and the lung. It can develop following Pneumonectomy, post traumatically, or with certain types of infection. It may also develop when large airways are in communication with the pleural space following a large pneumothorax or other loss of pleural negative pressure, especially during positive pressure mechanical ventilation. On imaging, the diagnosis is suspected indirectly on radiograph. Increased gas in the pneumonectomy operative bed, or new gas within a loculated effusion are highly suggestive of the diagnosis. Infectious causes include tuberculosis, "Actinomyces israelii", "Nocardia", and "Blastomyces dermatitidis". Malignancy and trauma can also result in the abnormal communication.
In the post-antibiotic era pattern of frequency is changing. In older studies anaerobes were found in up to 90% cases but they are much less frequent now.
TIF is a rare condition with a .7% frequency, and an mortality rate approaching 100% without surgical intervention. Immediate diagnosis and intervention of an TIF is critical for the surgical intervention success. 25-30% of TIF patients who reach the operating room survive. Recently, the incidence of TIF may have declined due to advances in tracheostomy tube technology and the introduction of the bedside percutaneous dilatational tracheostomy (PDT).
Most cases respond to antibiotics and prognosis is usually excellent unless there is a debilitating underlying condition. Mortality from lung abscess alone is around 5% and is improving.
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.
Conditions which commonly involve hemoptysis include bronchitis and pneumonia, lung cancers and tuberculosis. Other possible underlying causes include aspergilloma, bronchiectasis, coccidioidomycosis, pulmonary embolism, pneumonic plague, and cystic fibrosis. Rarer causes include hereditary hemorrhagic telangiectasia (HHT or Rendu-Osler-Weber syndrome), Goodpasture's syndrome, and granulomatosis with polyangiitis. In children, hemoptysis is commonly caused by the presence of a foreign body in the airway. The condition can also result from over-anticoagulation from treatment by drugs such as warfarin.
Blood-laced mucus from the sinus or nose area can sometimes be misidentified as symptomatic of hemoptysis (such secretions can be a sign of nasal or sinus cancer, but also a sinus infection). Extensive non-respiratory injury can also cause one to cough up blood. Cardiac causes like congestive heart failure and mitral stenosis should be ruled out.
The origin of blood can be identified by observing its color. Bright-red, foamy blood comes from the respiratory tract, whereas dark-red, coffee-colored blood comes from the gastrointestinal tract. Sometimes hemoptysis may be rust-colored.
The most common cause of minor hemoptysis is bronchitis.
- Lung cancer, including both non-small cell lung carcinoma and small cell lung carcinoma.
- Sarcoidosis
- Aspergilloma
- Tuberculosis
- Histoplasmosis
- Pneumonia
- Pulmonary edema
- Pulmonary embolism
- Foreign body aspiration and aspiration pneumonia
- Goodpasture's syndrome
- Granulomatosis with polyangiitis
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
- Bronchitis
- Bronchiectasis
- Pulmonary embolism
- Anticoagulant use
- Trauma
- Lung abscess
- Mitral stenosis
- Tropical eosinophilia
- Bleeding disorders
- Hughes-Stovin Syndrome and other variants of Behçet's disease
- Squamous Cell Carcinoma Of Esophagus
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.
Patients, families, and caregivers are encouraged to join the NIH Rare Lung Diseases Consortium Contact Registry. This is a privacy protected site that provides up-to-date information for individuals interested in the latest scientific news, trials, and treatments related to rare lung diseases.
A pulmonary consolidation is a region of (normally compressible) lung tissue that has filled with liquid, a condition marked by induration (swelling or hardening of normally soft tissue) of a normally aerated lung. It is considered a radiologic sign. Consolidation occurs through accumulation of inflammatory cellular exudate in the alveoli and adjoining ducts. Simply, it is defined as alveolar space that contains liquid instead of gas. The liquid can be pulmonary edema, inflammatory exudate, pus, inhaled water, or blood (from bronchial tree or hemorrhage from a pulmonary artery). It must be present to diagnose pneumonia: the signs of lobar pneumonia are characteristic and clinically referred to as consolidation.
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.
Hemoptysis is the coughing up of blood or blood-stained mucus from the bronchi, larynx, trachea, or lungs. This can occur with lung cancer, infections such as tuberculosis, bronchitis, or pneumonia, and certain cardiovascular conditions. Hemoptysis is considered massive at . In such cases, there are always severe injuries. The primary danger comes from choking, rather than blood loss.
To prevent an TIF, intubation time should be limited to less than 2 weeks and proper techniques should be used when performing tracheotomies. The occurrence of an TIF can be reduced by using more flexible and blunt tracheostomy tubes and insuring that the tubes are properly aligned in the patients. Placing the tracheostomy between the second and third tracheal rings can minimize the risk of an TIF. Repetitive head movements, especially, hyperextension of the neck should be avoided as since this movement results in contact between the innominate artery and the underside of the tube.
Signs that consolidation may have occurred include:
- Expansion of the thorax on inspiration is reduced on the affected side
- Vocal fremitus is increased on the side with consolidation
- Percussion is dull in affected area
- Breath sounds are bronchial
- Possible medium, late, or pan-inspiratory crackles
- Vocal resonance is increased. Here, the patient's voice (or whisper, as in whispered pectoriloquy) can be heard more clearly when there is consolidation, as opposed to in the healthy lung where speech sounds muffled.
- A pleural rub may be present.
- A lower expected Pa02 than calculated in the alveolar gas equation
To date, about 420 cases have been reported in the medical literature. Given its unusual nature, the true prevalence of PB is unknown, and it is likely that many patients are undiagnosed. PB does affect patients of all age groups and both genders.
Sources of such lipids could be either exogenous or endogenous.
Exogenous: from outside the body. For example, inhaled nose drops with an oil base, or accidental inhalation of cosmetic oil. Amiodarone is an anti-arrythmic known to cause this condition. Oil pulling has also been shown to be a cause. At risk populations include the elderly, developmentally delayed or persons with gastroesophageal reflux. Switching to water-soluble alternatives may be helpful in some situations.
Endogenous: from the body itself, for example, when an airway is obstructed, it is often the case that distal to the obstruction, lipid-laden macrophages (foamy macrophages) and giant cells fill the lumen of the disconnected airspace.
A bronchocele is a segment of bronchus that is filled with mucus and completely enclosed so the mucus cannot drain out. This segment is usually dilated. It is also called bronchial mucocele. If there is no obstruction to the flow of mucus, it is called mucoid impaction of bronchus. Bronchocele results from obstruction of bronchus. Overproduction of mucus can also contribute. Obstruction could be from scarring, tumor, congenital atresia, etc.
Bronchomalacia is a term for weak cartilage in the walls of the bronchial tubes, often occurring in children under six months. Bronchomalacia means 'floppiness' of some part of the bronchi. Patients present with noisy breathing and/or wheezing. There is collapse of a main stem bronchus on exhalation. If the trachea is also involved the term tracheobronchomalacia (TBM) is used. If only the upper airway the trachea is involved it is called tracheomalacia (TM). There are two types of bronchomalacia. Primary bronchomalacia is due to a deficiency in the cartilaginous rings. Secondary bronchomalacia may occur by extrinsic compression from an enlarged vessel, a vascular ring or a bronchogenic cyst. Though uncommon, idiopathic (of unknown cause) tracheobronchomalacia has been described in older adults.
Bronchomalacia can best be described as a birth defect of the bronchus in the respiratory tract. Congenital malacia of the large airways is one of the few causes of irreversible airways obstruction in children, with symptoms varying from recurrent wheeze and recurrent lower airways infections to severe dyspnea and respiratory insufficiency. It may also be acquired later in life due to chronic or recurring inflammation resulting from infection or other airway disease.
Laughlen first described lipid pneumonia in 1925 with infants that inhaled oil droplets. It is a condition that has been seen as an occupational risk for commercial diving operations but documented cases are rare.
The morbidity associated with DIPNECH is due to the associated obstructive lung disease. The lung disease tends to be slowly progressive, but given enough time can lead to significant disability and require supplemental oxygen therapy. There have been reports of lung transplantation in the setting of end-stage DIPNECH.
Neonates with TEF or esophageal atresia are unable to feed properly. Once diagnosed, prompt surgery is required to allow the food intake. Some children do experience problems following TEF surgery; they can develop dysphagia and thoracic problems. Children with TEF can also be born with other abnormalities, most commonly those described in VACTERL association - a group of anomalies which often occur together, including heart, kidney and limb deformities. 6% of babies with TEF also have a laryngeal cleft.
Tuberculosis, pneumonia, inhaled foreign bodies, allergic bronchopulmonary aspergillosis and bronchial tumours are the major acquired causes of bronchiectasis. Infective causes associated with bronchiectasis include infections caused by the Staphylococcus, Klebsiella, or Bordetella pertussis, the causative agent of whooping cough and nontuberculous mycobacteria.
Aspiration of ammonia and other toxic gases, pulmonary aspiration, alcoholism, heroin (drug use), various allergies all appear to be linked to the development of bronchiectasis.
Various immunological and lifestyle factors have also been linked to the development of bronchiectasis:
- Childhood Acquired Immune Deficiency Syndrome (AIDS), which predisposes patients to a variety of pulmonary ailments, such as pneumonia and other opportunistic infections.
- Inflammatory bowel disease, especially ulcerative colitis. It can occur in Crohn's disease as well, but does so less frequently. Bronchiectasis in this situation usually stems from various allergic responses to inhaled fungal spores. A Hiatal hernia can cause Bronchiectasis when the stomach acid that is aspirated into the lungs causes tissue damage.
- People with rheumatoid arthritis who smoke appear to have a tenfold increased rate of the disease. Still, it is unclear as to whether or not cigarette smoke is a specific primary cause of bronchiectasis.
- Case reports of Hashimoto's thyroiditis and bronchiectasis occurring in the same persons have been published.
No cause is identified in up to 50% of non-cystic-fibrosis related bronchiectasis.
The radiocephalic arteriovenous fistula (RC-AVF) is a shortcut between cephalic vein and radial artery at the wrist. It is the recommended first choice for hemodialysis access. Possible underlying causes for failure are stenosis and thrombosis especially in diabetics and those with low blood flow such as due to narrow vessels, arteriosclerosis and advanced age. Reported patency of fistulae after 1 year is about 62.5%.
In people with renal failure, requiring dialysis, a cimino fistula is often deliberately created in the arm by means of a short day surgery in order to permit easier withdrawal of blood for hemodialysis.
As a radical treatment for portal hypertension, surgical creation of a portacaval fistula produces an anastomosis between the hepatic portal vein and the inferior vena cava across the omental foramen (of Winslow). This spares the portal venous system from high pressure which can cause esophageal varices, caput medusae, and hemorrhoids.