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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
Tracheomalacia is a condition where the cartilage that keeps the airway (trachea) open is soft such that the trachea partly collapses especially during increased airflow. The usual symptom is stridor when a person breathes out.
The trachea normally opens slightly during breathing in and narrows slightly during breathing out. These processes are exaggerated in tracheomalacia, leading to airway collapse on breathing out.
If the condition extends further to the large airways (bronchi) (if there is also bronchomalacia), it is termed tracheobronchomalacia. The same condition can also affect the larynx, which is called laryngomalacia. The term is from "trachea" and the Greek μαλακία, "softening"
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.
Although this is a congenital lesion, airway sounds typically begin at age 4–6 weeks. Until that age, inspiratory flow rates may not be high enough to generate the sounds. Symptoms typically peak at age 6–8 months and remit by age 2 years.
Late-onset laryngomalacia may be a distinct entity, which can present after age of 2 years
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.
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.
In some cases (particularly where whole litters are affected) FCKS can be due to genetic factors: certain bloodlines are known to produce a preponderance of kittens with the condition, and close matings very commonly produce it in the offspring. Isolated cases are more likely due to environmental factors or slight prematurity of the kittens concerned, and even complete litters suffering from FCKS may have no genetic component—repeat matings that have produced flat kittens in one instance have often not produced it again, and recovered FCKS that have been bred from have likewise not necessarily produced offspring that suffered from the condition.
A kitten that has difficulty in breathing is very likely also to suffer from colic (which can cause weight loss in the early development of a normal kitten), and a very small daily (or twice daily) dose of liquid paraffin (one or two drops placed on the tongue of the kitten, or 0.1 ml) should help to alleviate this problem. FCKS kittens who do not maintain weight are usually among the group which die, but many of them may simply be unable to feed properly due to colic, becoming increasingly weak and lethargic, and fading due to malnutrition as much as to the thoracic problems.
Colic has many causes, but in a kitten with respiratory difficulty it is possible that a malfunction during the breathing process leads the kitten to swallow air instead of taking it into its lungs. The GI tract fills with air while the lungs do not receive a proper air supply, preventing them from inflating fully. Pressure from the stomach exacerbates the condition. Treating for colic with liquid paraffin seems to shorten recovery time from 4–10 weeks to a matter of days.
Choking is caused by an object from outside the body, also called a foreign body, blocking the airway. The object can block the upper or lower airway passages. The airway obstruction is usually partial but can also be complete.
Among children, the most common causes of choking are food, coins, toys, and balloons. In one study, peanuts were the most common object found in the airway of children evaluated for suspected foreign body aspiration. Foods that pose a high risk of choking include hot dogs, hard candy, nuts, seeds, whole grapes, raw carrots, apples, popcorn, peanut butter, marshmallows, chewing gum, and sausages. The most common cause of choking death in children is latex balloons. Small, round non-food objects such as balls, marbles, toys, and toy parts are also associated with a high risk of choking death because of their potential to completely block a child's airway.
Children younger than age three are especially at risk of choking because they explore the environment by putting objects in their mouth. Also, young children are still developing the ability to chew food completely. Molar teeth, which come in around 1.5 years of age, are necessary for grinding food. Even after molar teeth are present, children continue developing the ability to chew food completely and swallow throughout early childhood. In addition, a child's airway is smaller in diameter than an adult's airway, which means that smaller objects can cause an airway obstruction in children. Infants and young children generate a less forceful cough than adults, so coughing may not be as effective in relieving an airway obstruction. Finally, children with neuromuscular disorders, developmental delay, traumatic brain injury, and other conditions that affect swallowing are at an increased risk of choking.
In adults, choking often involves food blocking the airway. Risk factors include using alcohol or sedatives, undergoing a procedure involving the oral cavity or pharynx, wearing oral appliances, or having a medical condition that causes difficulty swallowing or impairs the cough reflex. Conditions that can cause difficulty swallowing and/or impaired coughing include neurologic conditions such as strokes, Alzheimer disease, or Parkinson disease. In older adults, risk factors also include living alone, wearing dentures, and having difficulty swallowing.
Children and adults with neurologic, cognitive, or psychiatric disorders may experience a delay in diagnosis because there may not be a known history of a foreign body entering the airway.
Although laryngomalacia is not associated with a specific gene, there is evidence that some cases may be inherited. Relaxation or a lack of muscle tone in the upper airway may be a factor. It is often worse when the infant is on his or her back, because the floppy tissues can fall over the airway opening more easily in this position.
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.
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.
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.
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.
Airway obstruction may cause obstructive pneumonitis or post-obstructive pneumonitis.
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.
Acute respiratory distress syndrome (ARDS) has some similarities to IRDS. Transient tachypnea of the newborn presents with respiratory distress syndrome in the preterm newborn.
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 annual incidence of ARDS is 13–23 people per 100,000 in the general population. Its incidence in the mechanically ventilated population in intensive care units is much higher. According to Brun-Buisson "et al" (2004), there is a prevalence of acute lung injury (ALI) of 16.1% percent in ventilated patients admitted for more than 4 hours.
Worldwide, severe sepsis is the most common trigger causing ARDS. Other triggers include mechanical ventilation, sepsis, pneumonia, Gilchrist's disease, drowning, circulatory shock, aspiration, traumaespecially pulmonary contusionmajor surgery, massive blood transfusions, smoke inhalation, drug reaction or overdose, fat emboli and reperfusion pulmonary edema after lung transplantation or pulmonary embolectomy. Pneumonia and sepsis are the most common triggers, and pneumonia is present in up to 60% of patients and may be either causes or complications of ARDS. Alcohol excess appears to increase the risk of ARDS. Diabetes was originally thought to decrease the risk of ARDS, but this has shown to be due to an increase in the risk of pulmonary edema. Elevated abdominal pressure of any cause is also probably a risk factor for the development of ARDS, particularly during mechanical ventilation.
The death rate varies from 25–40% in centers using up-to-date ventilatory strategies and up to 58% in all centers.
Pulmonary contusion can result in respiratory failure—about half of such cases occur within a few hours of the initial trauma. Other severe complications, including infections and acute respiratory distress syndrome (ARDS) occur in up to half of cases. Elderly people and those who have heart, lung, or kidney disease prior to the injury are more likely to stay longer in hospital and have complications from the injury. Complications occur in 55% of people with heart or lung disease and 13% of those without. Of people with pulmonary contusion alone, 17% develop ARDS, while 78% of people with at least two additional injuries develop the condition. A larger contusion is associated with an increased risk. In one study, 82% of people with 20% or more of the lung volume affected developed ARDS, while only 22% of people with less than 20% did so.
Pneumonia, another potential complication, develops in as many as 20% of people with pulmonary contusion. Contused lungs are less able to remove bacteria than uninjured lungs, predisposing them to infection. Intubation and mechanical ventilation further increase the risk of developing pneumonia; the tube is passed through the nose or mouth into the airways, potentially tracking bacteria from the mouth or sinuses into them. Also, intubation prevents coughing, which would clear bacteria-laden secretions from the airways, and secretions pool near the tube's cuff and allow bacteria to grow. The sooner the endotracheal tube is removed, the lower the risk of pneumonia, but if it is removed too early and has to be put back in, the risk of pneumonia rises. People who are at risk for pulmonary aspiration (e.g. those with lowered level of consciousness due to head injuries) are especially likely to get pneumonia. As with ARDS, the chances of developing pneumonia increase with the size of the contusion. Children and adults have been found to have similar rates of complication with pneumonia and ARDS.
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.
Lower airway obstruction is mainly caused by increased resistance in the bronchioles (usually from a decreased radius of the bronchioles) that reduces the amount of air inhaled in each breath and the oxygen that reaches the pulmonary arteries. It is different from airway restriction (which prevents air from diffusing into the pulmonary arteries because of some kind of blockage in the lungs). Diseases that cause lower airway obstruction are termed obstructive lung diseases.
Lower airway obstruction can be measured using spirometry. A decreased FEV1/FVC ratio (versus the normal of about 80%) is indicative of an airway obstruction, as the normal amount of air can no longer be exhaled in the first second of expiration. An airway restriction would not produce a reduced FEV1/FVC ratio, but would reduce the vital capacity. The ventilation is therefore affected leading to a ventilation perfusion mismatch and hypoxia.
Patients with plastic bronchitis that is being caused due to a co-morbid condition generally have a good prognosis once the underlying disease is treated.
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 contusion is found in 30–75% of severe cases of chest injury, making it the most common serious injury to occur in association with thoracic trauma. Of people who have multiple injuries with an injury severity score of over 15, pulmonary contusion occurs in about 17%. It is difficult to determine the death rate (mortality) because pulmonary contusion rarely occurs by itself. Usually, deaths of people with pulmonary contusion result from other injuries, commonly traumatic brain injury. It is controversial whether pulmonary contusion with flail chest is a major factor in mortality on its own or whether it merely contributes to mortality in people with multiple injuries. The estimated mortality rate of pulmonary contusion ranges from 14–40%, depending on the severity of the contusion itself and on associated injuries. When the contusions are small, they do not normally increase the chance of death or poor outcome for people with blunt chest trauma; however, these chances increase with the size of the contusion. One study found that 35% of people with multiple significant injuries including pulmonary contusion die. In another study, 11% of people with pulmonary contusion alone died, while the number rose to 22% in those with additional injuries. Pulmonary contusion is thought to be the direct cause of death in a quarter to a half of people with multiple injuries (polytrauma) who die. An accompanying flail chest increases the morbidity and mortality to more than twice that of pulmonary contusion alone.
Pulmonary contusion is the most common cause of death among vehicle occupants involved in accidents, and it is thought to contribute significantly in about a quarter of deaths resulting from vehicle collisions. As vehicle use has increased, so has the number of auto accidents, and with it the number of chest injuries. However an increase in the number of airbags installed in modern cars may be decreasing the incidence of pulmonary contusion. Use of child restraint systems has brought the approximate incidence of pulmonary contusion in children in vehicle accidents from 22% to 10%.
Differences in the bodies of children and adults lead to different manifestations of pulmonary contusion and associated injuries; for example, children have less body mass, so the same force is more likely to lead to trauma in multiple body systems. Since their chest walls are more flexible, children are more vulnerable to pulmonary contusion than adults are, and thus suffer from the injury more commonly. Pulmonary contusion has been found in 53% of children with chest injuries requiring hospitalization. Children in forceful impacts suffer twice as many pulmonary contusions as adults with similar injury mechanisms, yet have proportionately fewer rib fractures. The rates of certain types of injury mechanisms differ between children and adults; for example, children are more often hit by cars as pedestrians. Some differences in children's physiology might be advantageous (for example they are less likely to have other medical conditions), and thus they have been predicted to have a better outcome. However, despite these differences, children with pulmonary contusion have similar mortality rates to adults.