Tracheobronchomalacia

Last updated
Tracheobronchomalacia
Other namesExcessive Dynamic Airway Collapse
Blausen 0865 TracheaAnatomy.png
Trachea anatomy
Specialty Pulmonology
Symptoms Chronic cough, stridor, inability to raise secretions, breathlessness
Usual onsetFrom birth (Congenital ), Adulthood (Acquired)
DurationCongenital: Significant improvement after 18-24 months although some symptoms may be present for life. Acquired: Long-term.
Risk factors Relapsing polychondritis, Chronic obstructive pulmonary disease, Asthma, Gastroesophageal reflux disease (GERD), Heritable connective tissue disorders (Particularly Ehlers-Danlos Syndrome), Prolonged tracheal intubation, Long-term use of inhaled corticosteroids
Diagnostic method Bronchoscopy, Dynamic Expiratory Computed Tomography
Differential diagnosis Asthma, Chronic Obstructive Pulmonary Disease (COPD), Bronchiectasis, Tracheal stenosis, Tracheal tumors, Laryngomalacia
PreventionTreatment of inflammatory disorders of the airway, avoiding hard impacts
Treatment Continuous Positive Airway Pressure (CPAP), Airway Stenting, Aortopexy, Tracheopexy, Tracheobronchoplasty
Prognosis Variable: Disease can range from asymptomatic to life-threatening

Tracheobronchomalacia (TBM) is a condition characterized by flaccidity of the tracheal support cartilage which leads to tracheal collapse. [1] This condition can also affect the bronchi. There are two forms of this condition: primary TBM and secondary TBM. Primary TBM is congenital and starts as early as birth. It is mainly linked to genetic causes. Secondary TBM is acquired and starts in adulthood. It is mainly developed after an accident or chronic inflammation. [2]

Contents

Tracheobronchomalacia may also occur in people who have normal cartilaginous structure of the trachea, but significant atrophy of the posterior wall, causing significant invagination of the trachea on expiration. In these cases it is more commonly known as excessive dynamic airway collapse (EDAC).

Signs and symptoms

Initially TBM may be asymptomatic or some patients do not experience symptoms at all. In a progressive TBM case symptoms include:

Symptoms may become worse if the patient is stressed, sick, lying down, or forcing a cough.

Cause

Congenital TBM is present from birth. Acquired TBM often has no clear cause but is frequently found together with other pulmonary diseases like asthma and Chronic obstructive pulmonary disease, as well as Gastroesophageal reflux disease. TBM can be caused by damage to the support cartilage or membranous wall of the trachea, this can be the result of physical trauma (such as from prolonged Tracheal intubation) or pathological changes caused by inflammatory diseases like Relapsing polychondritis. [5] In patients with TBM in one study, the number of longitudinal elastic fibers in the pars of membranacea was reduced throughout the whole trachea. [6]

People with heritable connective tissue disorders like Ehlers–Danlos syndrome seem to have at an increased risk of both congenital and acquired TBM, although the extent of that risk is unknown. [7]

There have been studies linking long-term use of inhaled Corticosteroids to Tracheobronchomalacia.[ citation needed ]

Diagnosis

Diagnosis is conducted according to the severity of the symptoms. Initially pulmonary function tests [8] are administered. These tests include the lungs' capability of air intake and outtake, and gas flow of oxygen and carbon dioxide between the body and environment. Following these function tests a special type of Chest CT scan or a bronchoscopy will be ordered. The results of the scan and bronchoscopy [9] will display the status of the condition. A mild case of tracheobronchomalacia would be if the patient's trachea condenses 50% of its normal space when exhaling. Moderate tracheobronchomalacia would be 25% of the normal trachea space constricting and a severe case would be if the walls touch each other. [10]

Tracheobronchomalacia is thought to be underdiagnosed as mild cases may be asymptomatic and symptoms are often mistaken for more common respiratory conditions like Asthma and Chronic obstructive pulmonary disease. [11]

Treatment

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 address 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 [12] that has a machine blow small amounts of air into the trachea to keep it open (mainly at night), or a tracheostomy, [13] which is surgically inserted into the patient's neck that leads to the trachea to help with breathing. Another form of treatment may include a tracheobronchoplasty which is a specific surgical procedure that helps control the airway by splinting the trachea. The splint helps strengthen the trachea with the hopes that the symptoms improve. [2] People with tracheobronchomalacia who do not experience symptoms do not need treatment and are often undiagnosed. [2]

On 28 May 2013, it was reported that a cure had been developed via a 3D printed windpipe. [14] This cure has currently saved the lives of at least 3 infants.

See also

Related Research Articles

<span class="mw-page-title-main">Trachea</span> Cartilaginous tube that connects the pharynx and larynx to the lungs

The trachea, also known as the windpipe, is a cartilaginous tube that connects the larynx to the bronchi of the lungs, allowing the passage of air, and so is present in almost all animals with lungs. The trachea extends from the larynx and branches into the two primary bronchi. At the top of the trachea, the cricoid cartilage attaches it to the larynx. The trachea is formed by a number of horseshoe-shaped rings, joined together vertically by overlying ligaments, and by the trachealis muscle at their ends. The epiglottis closes the opening to the larynx during swallowing.

Tracheomalacia is a condition or incident where the cartilage that keeps the airway (trachea) open is soft such that the trachea partly collapses especially during increased airflow. This condition is most commonly seen in infants and young children. The usual symptom is stridor when a person breathes out. This is usually known as a collapsed windpipe.

<span class="mw-page-title-main">Hemoptysis</span> Medical symptom consisting of bloody mucus from coughing

Hemoptysis or haemoptysis is the discharge of blood or blood-stained mucus through the mouth coming from the bronchi, larynx, trachea, or lungs. It does not necessarily involve coughing. In other words, it is the airway bleeding. This can occur with lung cancer, infections such as tuberculosis, bronchitis, or pneumonia, and certain cardiovascular conditions. Hemoptysis is considered massive at 300 mL. In such cases, there are always severe injuries. The primary danger comes from choking, rather than blood loss.

<span class="mw-page-title-main">Bronchiectasis</span> Permanent enlargement of the lung airways

Bronchiectasis is a disease in which there is permanent enlargement of parts of the airways of the lung. Symptoms typically include a chronic cough with mucus production. Other symptoms include shortness of breath, coughing up blood, and chest pain. Wheezing and nail clubbing may also occur. Those with the disease often get lung infections.

<span class="mw-page-title-main">Pulmonary aspiration</span> Entry of materials into the larynx (voice box) and lower respiratory tract

Pulmonary aspiration is the entry of material such as pharyngeal secretions, food or drink, or stomach contents from the oropharynx or gastrointestinal tract, into the larynx and lower respiratory tract, the portions of the respiratory system from the trachea (windpipe) to the lungs. A person may inhale the material, or it may be delivered into the tracheobronchial tree during positive pressure ventilation. When pulmonary aspiration occurs during eating and drinking, the aspirated material is often colloquially referred to as "going down the wrong pipe".

Stridor is an extra-thoracic high-pitched breath sound resulting from turbulent air flow in the larynx or lower in the bronchial tree. It is different from a stertor, which is a noise originating in the pharynx.

<span class="mw-page-title-main">Relapsing polychondritis</span> Medical condition

Relapsing polychondritis is a systemic disease characterized by repeated episodes of inflammation and in some cases deterioration of cartilage. The disease can be life-threatening if the respiratory tract, heart valves, or blood vessels are affected. The exact mechanism is poorly understood.

Tracheobronchitis is inflammation of the trachea and bronchi. It is characterised by a cough, fever, and purulent sputum and is therefore suggestive of pneumonia. It is classified as a respiratory tract infection.

<span class="mw-page-title-main">Respiratory disease</span> Disease of the respiratory system

Respiratory diseases, or lung diseases, are pathological conditions affecting the organs and tissues that make gas exchange difficult in air-breathing animals. They include conditions of the respiratory tract including the trachea, bronchi, bronchioles, alveoli, pleurae, pleural cavity, the nerves and muscles of respiration. Respiratory diseases range from mild and self-limiting, such as the common cold, influenza, and pharyngitis to life-threatening diseases such as bacterial pneumonia, pulmonary embolism, tuberculosis, acute asthma, lung cancer, and severe acute respiratory syndromes, such as COVID-19. Respiratory diseases can be classified in many different ways, including by the organ or tissue involved, by the type and pattern of associated signs and symptoms, or by the cause of the disease.

<span class="mw-page-title-main">Laryngotracheal stenosis</span> Medical condition

Laryngotracheal stenosis refers to abnormal narrowing of the central air passageways. This can occur at the level of the larynx, trachea, carina or main bronchi. In a small number of patients narrowing may be present in more than one anatomical location.

<span class="mw-page-title-main">Bronchoscopy</span> Procedure allowing a physician to look at a patients airways

Bronchoscopy is an endoscopic technique of visualizing the inside of the airways for diagnostic and therapeutic purposes. An instrument (bronchoscope) is inserted into the airways, usually through the nose or mouth, or occasionally through a tracheostomy. This allows the practitioner to examine the patient's airways for abnormalities such as foreign bodies, bleeding, tumors, or inflammation. Specimens may be taken from inside the lungs. The construction of bronchoscopes ranges from rigid metal tubes with attached lighting devices to flexible optical fiber instruments with realtime video equipment.

<span class="mw-page-title-main">Obstructive lung disease</span> Category of respiratory disease characterized by airway obstruction

Obstructive lung disease is a category of respiratory disease characterized by airway obstruction. Many obstructive diseases of the lung result from narrowing (obstruction) of the smaller bronchi and larger bronchioles, often because of excessive contraction of the smooth muscle itself. It is generally characterized by inflamed and easily collapsible airways, obstruction to airflow, problems exhaling, and frequent medical clinic visits and hospitalizations. Types of obstructive lung disease include asthma, bronchiectasis, bronchitis and chronic obstructive pulmonary disease (COPD). Although COPD shares similar characteristics with all other obstructive lung diseases, such as the signs of coughing and wheezing, they are distinct conditions in terms of disease onset, frequency of symptoms, and reversibility of airway obstruction. Cystic fibrosis is also sometimes included in obstructive pulmonary disease.

Williams–Campbell syndrome (WCS) is a disease of the airways where cartilage in the bronchi is defective. It is a form of congenital cystic bronchiectasis. This leads to collapse of the airways and bronchiectasis. It acts as one of the differential to allergic bronchopulmonary aspergillosis. WCS is a deficiency of the bronchial cartilage distally.

<span class="mw-page-title-main">Bronchomalacia</span> Medical condition

Bronchomalacia is a term for weak cartilage in the walls of the bronchial tubes, often occurring in children under a day. 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 tracheobronchomalacia has been described in older adults.

Pulmonary hygiene, also referred to as pulmonary toilet, is a set of methods used to clear mucus and secretions from the airways. The word pulmonary refers to the lungs. The word toilet, related to the French toilette, refers to body care and hygiene; this root is used in words such as toiletry that also relate to cleansing.

<span class="mw-page-title-main">Tracheobronchial injury</span> Damage to the tracheobronchial tree

Tracheobronchial injury is damage to the tracheobronchial tree. It can result from blunt or penetrating trauma to the neck or chest, inhalation of harmful fumes or smoke, or aspiration of liquids or objects.

Double aortic arch is a relatively rare congenital cardiovascular malformation. DAA is an anomaly of the aortic arch in which two aortic arches form a complete vascular ring that can compress the trachea and/or esophagus. Most commonly there is a larger (dominant) right arch behind and a smaller (hypoplastic) left aortic arch in front of the trachea/esophagus. The two arches join to form the descending aorta which is usually on the left side. In some cases the end of the smaller left aortic arch closes and the vascular tissue becomes a fibrous cord. Although in these cases a complete ring of two patent aortic arches is not present, the term ‘vascular ring’ is the accepted generic term even in these anomalies.

A laryngeal cleft or laryngotracheoesophageal cleft is a rare congenital abnormality in the posterior laryngo-tracheal wall. It occurs in approximately 1 in 10,000 to 20,000 births. It means there is a communication between the oesophagus and the trachea, which allows food or fluid to pass into the airway.

Tracheal agenesis is a rare birth defect with a prevalence of less than 1 in 50,000 in which the trachea fails to develop, resulting in an impaired communication between the larynx and the alveoli of the lungs. Although the defect is normally fatal, occasional cases have been reported of long-term survival following surgical intervention.

<span class="mw-page-title-main">Tracheobronchopathia osteochondroplastica</span> Medical condition

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.

References

  1. Lee, EY; Boiselle, PM (July 2009). "Tracheobronchomalacia in infants and children: multidetector CT evaluation". Radiology. 252 (1): 7–22. doi:10.1148/radiol.2513081280. PMID   19561247.
  2. 1 2 3 "Tracheobronchomalacia". Genetic and Rare Diseases Information Center.
  3. "Tracheaobronchomalacia - Cedars-Sinai". www.cedars-sinai.edu. Archived from the original on 2015-12-08. Retrieved 2015-12-03.
  4. Baroni, RH; Feller-Kopman, D; Nishino, M; Hatabu, H; Loring, SH; Ernst, A; Boiselle, PM (May 2005). "Tracheobronchomalacia: comparison between end-expiratory and dynamic expiratory CT for evaluation of central airway collapse". Radiology. 235 (2): 635–41. doi:10.1148/radiol.2352040309. PMID   15798155.
  5. Oryoji, Daisuke; Ono, Nobuyuki; Himeji, Daisuke; Yoshihiro, Kyoko; Kai, Yasufumi; Matsuda, Motohiro; Tsukamoto, Hiroshi; Ueda, Akira (2017-12-15). "Sudden Respiratory Failure due to Tracheobronchomalacia by Relapsing Polychondritis, Successfully Rescued by Multiple Metallic Stenting and Tracheostomy". Internal Medicine. 56 (24): 3369–3372. doi:10.2169/internalmedicine.8778-16. PMC   5790730 . PMID   29021454.
  6. Jokinen, K; Palva, T; Sutinen, S; Nuutinen, J (April 1977). "Acquired tracheobronchomalacia". Annals of Clinical Research. 9 (2): 52–7. PMID   883758.
  7. Bascom, Rebecca; Dhingra, Radha; Francomano, Clair A. (2021-11-22). "Respiratory manifestations in the Ehlers–Danlos syndromes". American Journal of Medical Genetics Part C: Seminars in Medical Genetics. 187 (4). Wiley: 533–548. doi:10.1002/ajmg.c.31953. ISSN   1552-4868. PMID   34811894. S2CID   244491275.
  8. MedlinePlus Encyclopedia : Pulmonary function tests
  9. MedlinePlus Encyclopedia : Bronchoscopy
  10. Kamran, Ali; Zendejas, Benjamin; Jennings, Russell W. (2021-06-01). "Current concepts in tracheobronchomalacia: diagnosis and treatment". Seminars in Pediatric Surgery. 30 (3): 151062. doi:10.1016/j.sempedsurg.2021.151062. ISSN   1055-8586. PMID   34172207. S2CID   235647748 . Retrieved 2022-03-26.
  11. Maestu, Luis Puente; Poch, Eduardo de Miguel (2016). "Tracheobronchomalacia treatment: how far have we come?". Journal of Thoracic Disease. 8 (12): 3490–3493. doi: 10.21037/jtd.2016.12.91 . PMC   5227206 . PMID   28149540.
  12. "What Is CPAP? - NHLBI, NIH". www.nhlbi.nih.gov. Archived from the original on 2015-12-08. Retrieved 2015-12-03.
  13. "What Is a Tracheostomy? - NHLBI, NIH". www.nhlbi.nih.gov. Retrieved 2015-12-03.
  14. Fessenden, Marissa (2013). "3-D printed windpipe gives infant breath of life". Nature. doi: 10.1038/nature.2013.13085 . S2CID   61819094 . Retrieved 2015-09-09.