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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).
There are three types of tracheomalacia:
- Type 1—congenital, sometimes associated with tracheoesophageal fistula or esophageal atresia
- Type 2—extrinsic compression sometimes due to vascular rings
- Type 3—acquired due to chronic infection or prolonged intubation or inflammatory conditions like relapsing polychondritis
A Saber-sheath trachea is a trachea that has an abnormal shape caused by chronic obstructive pulmonary disease. The posterior area of the trachea increases in diameter while the lateral measurement decreases.
If the symptoms are severe enough, treatment may be needed. These range from medical management over mechanical ventilation (both continuous positive airway pressure (CPAP), or bi-level positive airway pressure (BiPAP) to tracheal stenting and surgery.
Surgical techniques include aortopexy, tracheopexy, tracheobronchoplasty, and tracheostomy. The role of the nebulised recombinant human deoxyribonuclease (rhDNase) remains inconclusive.
Laryngotracheal stenosis (Laryngo-: Glottic Stenosis; Subglottic Stenosis; Tracheal: narrowings at different levels of the windpipe) is a more accurate description for this condition when compared, for example to subglottic stenosis which technically only refers to narrowing just below vocal folds or tracheal stenosis. In babies and young children however, the subglottis is the narrowest part of the airway and most stenoses do in fact occur at this level. Subglottic stenosis is often therefore used to describe central airway narrowing in children, and laryngotracheal stenosis is more often used in adults.
The differential of TO includes amyloidosis, which is typically circumferential, papillomatosis, though this usually occurs in younger patients and can cause lung cavitation when disseminated, granulomatosis with polyangiitis, though this is circumferential as well and often involves distal lung cavitation as well. Relapsing polychondritis can also spare the posterior wall, though it is not typically nodular in appearance.
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.
Laryngotracheal stenosis is an umbrella term for a wide and heterogeneous group of very rare conditions. The population incidence of adult post-intubation laryngotracheal stenosis which is the commonest benign sub-type of this condition is approximately 1 in 200,000 adults per year. The main causes of adult laryngotracheal stenosis are:
Tracheobronchopathia osteochondroplastica (TO) is a rare benign disease of unknown cause, in which multiple cartilaginous or bony submucosal nodules project into the trachea and proximal bronchi. The nodules usually spare the posterior wall of the airway because they are of cartilaginous origin, while the posterior wall of the airway is membranous (does not contain cartilage). This is as opposed to tracheobronchial amyloidosis, which does not spare the posterior wall.
It usually occurs in men around their fifth decade of life, as opposed to tracheobronchial papillomatosis due to HPV infection, which usually occurs in younger patients. TO can cause airway obstruction, bleeding and chronic cough. Treatment involves the use of bronchodilators, and physical dilatation by bronchoscopy. The patients are also more prone to post-obstructive pneumonia and chronic lung infection in severe cases.
Tracheobronchomegaly is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways. The abnormally widened trachea and mainstem bronchi are associated with recurrent lower respiratory tract infection and copious purulent sputum production, eventually leading to bronchiectasis and other respiratory complications.
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.
Woodring et al. (1991) suggested the following diagnostic criteria for tracheomegaly in adults based on chest radiography:
- Adult Males: Tracheal transverse diameter > 25 mm and sagittal diameter > 27 mm.
- Adult Females: Tracheal transverse diameter > 21 mm and sagittal diameter > 23 mm.
Tracheobronchomalacia or TBM is a condition characterized by flaccidity of the tracheal support cartilage which leads to tracheal collapse. This condition can also affect the bronchi. There are two forms of this rare condition: primary TB and secondary TB. Primary TB is congenital and starts as early as two years old. It is mainly linked to genetic causes. Secondary TB is acquired and starts in adulthood. It is mainly developed after an accident or chronic inflammation.
On 28 May 2013, it was reported that a cure had been developed via a 3D printed windpipe. This cure has currently saved the lives of at least 3 infants.
The innominate artery usually crosses the trachea at the ninth cartilage ring, however this can vary from the sixth to the thirteenth cartilage ring in patients. A TIF runs between the trachea and the innominate artery. Through this connection blood from within the artery may pass into the trachea or alternatively air from within the trachea may cross into the artery.
TIF is a late complication of a tracheotomy and is associated with prolonged endotracheal intubation, as a result of cuff over inflation or a poorly positioned tracheostomy tube. Over inflation of the cuff causes the tracheostomy tube to erode into the posterior aspect of the innominate artery leading to the formation of a fistula. The pathogenesis of an TIF by the aforementioned method is pressure necrosis by tracheostomy tube on the tracheal wall. An TIF can also occur due to innominate artery injury as a result of an bronchoscopy.
Patients whose tracheotomies are placed beneath the third tracheal ring cartilage and patients with innominate arteries crossing higher on the trachea have an increased risk of developing an TIF. Other factors contributing to the development of TIF include steroids, which weaken the endotracheal mucosa, episodes of hypotension in which the pressure in the tracheostomy tube exceeds that of the endotracheal mucosa, and radiation therapy.
An endotracheal tumor can mimic a TIF and present with massive bleeding during a rigid bronchoscopy.
Choking is the fourth most common cause of unintentional injury-related death in the US. Many episodes are not reported because they are brief and resolve without seeking medical attention. Among reported events, the majority of episodes (80%) occur among children younger than age 15, with fewer episodes (20%) among age 15 and older. The death rate from choking is low at most ages but increases starting around age 74. Choking due to a foreign object resulted in 162,000 deaths (2.5 per 100,000) in 2013, compared to 140,000 deaths (2.9 per 100,000) in 1990.
To properly treat a patient with tracheobronchomalacia, the subtype must be determined (primary or secondary). After the type is named, the cause must be identified, whether it is from genetics, a trauma accident, or chronic tracheal illness. If a trauma case or chronic tracheal illnesses were the cause, the first steps of treatment would be to fix or help these underlying issues. If the cause is genetic or the previous underlying issues could not be fixed, other treatments would be assessed. More severe treatments include silicone stenting to prevent tracheal constriction, surgery to strengthen or attempt to rebuild the walls, continuous positive airway pressure that has a machine blow small amounts of air into the trachea to keep it open (mainly at night), or a tracheostomy, which is surgically put into your neck that leads to your trachea to help with breathing. People with tracheobronchomalacia who do not experience symptoms do not need treatment and are often undiagnosed.
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.
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.
Rupture of the trachea or bronchus is the most common type of blunt injury to the airway. It is difficult to determine the incidence of TBI: in as many as 30–80% of cases, death occurs before the person reaches a hospital, and these people may not be included in studies. On the other hand, some TBI are so small that they do not cause significant symptoms and are therefore never noticed. In addition, the injury sometimes is not associated with symptoms until complications develop later, further hindering estimation of the true incidence. However, autopsy studies have revealed TBI in 2.5–3.2% of people who died after trauma. Of all neck and chest traumas, including people that died immediately, TBI is estimated to occur in 0.5–2%. An estimated 0.5% of polytrauma patients treated in trauma centers have TBI. The incidence is estimated at 2% in blunt chest and neck trauma and 1–2% in penetrating chest trauma. Laryngotracheal injuries occur in 8% of patients with penetrating injury to the neck, and TBI occurs in 2.8% of blunt chest trauma deaths. In people with blunt trauma who do reach a hospital alive, reports have found incidences of 2.1% and 5.3%. Another study of blunt chest trauma revealed an incidence of only 0.3%, but a mortality rate of 67% (possibly due in part to associated injuries). The incidence of iatrogenic TBI (that caused by medical procedures) is rising, and the risk may be higher for women and the elderly. TBI results about once every 20,000 times someone is intubated through the mouth, but when intubation is performed emergently, the incidence may be as high as 15%.
The mortality rate for people who reach a hospital alive was estimated at 30% in 1966; more recent estimates place this number at 9%. The number of people reaching a hospital alive has increased, perhaps due to improved prehospital care or specialized treatment centers. Of those who reach the hospital alive but then die, most do so within the first two hours of arrival. The sooner a TBI is diagnosed, the higher the mortality rate; this is likely due to other accompanying injuries that prove fatal.
Accompanying injuries often play a key role in the outcome. Injuries that may accompany TBI include pulmonary contusion and laceration; and fractures of the sternum, ribs and clavicles. Spinal cord injury, facial trauma, traumatic aortic rupture, injuries to the abdomen, lung, and head are present in 40–100%. The most common accompanying injury is esophageal perforation or rupture (known as Boerhaave syndrome), which occurs in as many as 43% of the penetrating injuries to the neck that cause tracheal injury.
Most people with TBI who die do so within minutes of the injury, due to complications such as pneumothorax and insufficient airway and to other injuries that occurred at the same time. Most late deaths that occur in TBI are attributed to sepsis or multiple organ dysfunction syndrome (MODS). If the condition is not recognized and treated early, serious complications are more likely to occur; for example, pneumonia and bronchiectasis may occur as late complications. Years can pass before the condition is recognized. Some TBI are so small that they do not have significant clinical manifestations; they may never be noticed or diagnosed and may heal without intervention.
If granulation tissue grows over the injured site, it can cause stenosis of the airway, after a week to a month. The granulation tissue must be surgically excised. Delayed diagnosis of a bronchial rupture increases risk of infection and lengthens hospital stay. People with a narrowed airway may suffer dyspnea, coughing, wheezing, respiratory tract infection, and difficulty with clearing secretions. If the bronchiole is completely obstructed, atelectasis occurs: the alveoli of the lung collapse. Lung tissue distal to a completely obstructed bronchiole often does not become infected. Because it is filled with mucus, this tissue remains functional. When the secretions are removed, the affected portion of the lung is commonly able to function almost normally. However, infection is common in lungs distal to a partially obstructed bronchiole. Infected lung tissue distal to a stricture can be damaged, and wheezing and coughing may develop due to the narrowing. In addition to pneumonia, the stenosis may cause bronchiectasis, in which bronchi are dilated, to develop. Even after an airway with a stricture is restored to normal, the resulting loss of lung function may be permanent.
Complications may also occur with treatment; for example a granuloma can form at the suture site. Also, the sutured wound can tear again, as occurs when there is excessive pressure in the airways from ventilation. However, for people who do receive surgery soon after the injury to repair the lesion, outcome is usually good; the long-term outcome is good for over 90% of people who have TBI surgically repaired early in treatment. Even when surgery is performed years after the injury, the outlook is good, with low rates of death and disability and good chances of preserving lung function.
Pulmonary artery sling is a rare condition in which the left pulmonary artery anomalously originates from a normally positioned right pulmonary artery. The left pulmonary artery arises anterior to the right main bronchus near its origin from the trachea, courses between the trachea and the esophagus and enters the left hilum. Symptoms include cyanosis, dyspnoea and apnoeic spells. It almost always requires surgical intervention. Rarely it is asymptomatic and is detected incidentally in asymptomatic adults.
The American Heart Association recommends chest thrusts rather than abdominal thrusts for pregnant or obese persons who are choking.
Chest thrusts are performed in a similar to the abdominal thrusts, but with a change in hand placement of the rescuer. The hands are placed on the lower part of the choking victim's chest, at the base of the breastbone or sternum, rather than over the middle of the abdomen, as in traditional abdominal thrusts. Strong inward thrusts are then applied.
A vascular ring is a congenital defect in which there is an abnormal formation of the aorta and/or its surrounding blood vessels. The trachea and esophagus are completely encircled and sometimes compressed by a "ring" formed by these vessels, which can lead to breathing and digestive difficulties.
Most often this is because of persistence of the double aortic arch after the second month of fetal life.
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.
Little is known regarding the exact causes of aortic arch anomalies. However, the association with chromosome 22q11 deletion (CATCH 22) implies that a genetic component is likely in certain cases. Esophageal atresia also occurs in some patients with double aortic arch.