Tracheobronchomalacia

Title

Other Names:

TBM

Categories:

Lung Diseases

Summary Summary

Tracheobronchomalacia (TBM) is a rare condition that occurs when the walls of the airway (specifically the trachea and bronchi) are weak. This can cause the airway to become narrow or collapse.^[1] There are two forms of TBM: a congenital form (called primary TBM) that typically develops during infancy or early childhood and an acquired form (called secondary TBM) that is usually seen in adults.^[2] Some affected people may initially have no signs or symptoms. However, the condition is typically progressive (becomes worse overtime) and most people will eventually develop characteristic features such as shortness of breath, cough, sputum retention (inability to clear mucus from the respiratory tract), and wheezing or stridor with breathing.^[1]^[3] Most cases of primary TBM are caused by genetic conditions that weaken the walls of the airway, while the secondary form often occurs incidentally due to trauma, chronic inflammation and/or prolonged compression of the airways.^[2]^[1] Treatment is generally only required in those who have signs and symptoms of the condition and may include stenting, surgical correction, continuous positive airway pressure (CPAP), and tracheostomy.^[1]^[4]^[5]

Last updated: 3/6/2015

Symptoms Symptoms

Tracheobronchomalacia (TBM) is a condition that occurs when the walls of the airway (specifically the trachea and bronchi) are weak. This can cause the airway to become narrow or collapse. There are two forms of TBM. Primary TBM (also called congenital TBM) typically develops during infancy or early childhood, while secondary TBM (also called acquired TBM) is usually seen in adults.^[1]

Some affected people may initially have no signs or symptoms. However, the condition is typically progressive (becomes worse overtime) and many people will eventually develop characteristic features such as shortness of breath, cough, sputum retention (inability to clear mucus from the respiratory tract), and wheezing or stridor with breathing. Symptoms may become worse during periods of stress (i.e. illness), when reclining, or when forcing a cough. Infants and young children with TBM tend to have more frequent respiratory infections and delayed recovery from these illnesses.^[1]^[6]^[2]

Last updated: 3/5/2015

Cause Cause

The underlying cause of tracheobronchomalacia (TBM) varies by subtype. Most cases of primary TBM (also called congenital TBM) are caused by genetic conditions that weaken the walls of the airway (specifically the trachea and bronchi). For example, TBM has been reported in people with mucopolysaccharidoses (such as Hunter syndrome and Hurler syndrome), Ehlers-Danlos Syndrome, and a variety of chromosome abnormalities. Primary TBM can also be idiopathic (unknown cause) or associated with prematurity and certain birth defects (i.e. tracheoesophageal fistula).^[3]^[1]^[4]

The secondary form (also called acquired TBM) is caused by the degeneration (break down) of cartilage that typically supports the airways. It is most commonly associated with:^[3]^[1]^[4]

Certain medical procedures such as endotracheal intubation or tracheostomy
Conditions that lead to chronic (persisting or progressing for a long period of time) inflammation such as relapsing polychondritis or chronic obstructive pulmonary disease (COPD)
Cancers, tumors, or cysts that cause prolonged compression of the airway

Last updated: 3/5/2015

Inheritance Inheritance

Primary tracheobronchomalacia (TBM) is often associated with certain genetic conditions.^[3]^[1]^[5] In some cases, an affected person inherits the condition from an affected parent. Other cases may result from new (de novo) gene mutations. These cases occur in people with no history of the disorder in their family. When TBM is part of a genetic condition, it can be passed on to future generations.

Secondary TBM (also called acquired TBM) is not inherited. It generally occurs incidentally due to trauma, chronic inflammation and/or prolonged compression of the airways.^[1]

Last updated: 3/5/2015

Diagnosis Diagnosis

A diagnosis of tracheobronchomalacia (TBM) may be suspected based on the presence of characteristic signs and symptoms or abnormal pulmonary function tests. Additional testing such as CT scan and bronchoscopy can then be performed to confirm the diagnosis and evaluate the severity of the condition. TBM is considered mild if the trachea narrows to 50% of its initial size while the affected person is breathing out, moderate if it narrows to 25%, and severe if the walls of the trachea touch.^[1]

Last updated: 3/6/2015

Treatment Treatment

Treatment is only medically necessary in people who have signs and symptoms of tracheobronchomalacia (TBM). Management of symptomatic TBM first involves identifying underlying conditions contributing to symptoms, such as chronic inflammation, compression, or injury. Initial treatment will target these underlying medical concerns.^[1]

If symptoms persist, people with TBM may undergo pulmonary function tests or other assessments to help guide therapy choice and allow monitoring of the response to treatment.^[1]

Treatment options may include:^[1]^[4]^[5]

Silicone and/or long-term stenting
Surgical correction
Continuous positive airway pressure (CPAP)
Tracheostomy (often used as a last resort as it can sometimes worsen TBM)

We strongly recommend that you discuss your treatment options with a healthcare provider.

Last updated: 3/6/2015

Prognosis Prognosis

The long-term outlook (prognosis) for people with tracheobronchomalacia (TBM) varies depending on the underlying cause. In general, the prognosis is good in children with primary TBM (also called congenital TBM) who do not have any associated problems. Most of these cases that develop during infancy resolve on their own when the cartilage of the trachea naturally stiffens (often between ages one and two). However, intervention may be needed in children with episodes of airway obstruction, frequent infections, respiratory failure, and/or failure to thrive. When primary TBM is part of a genetic condition, the prognosis largely depends on the severity of the condition and the other associated signs and symptoms.^[2]

Secondary TBM (also called acquired TBM) can remain stable over time, but tends to worsen in the majority of affected people.^[3]^[1] Without treatment, TBM may cause significant breathing problems, but it is rarely life-threatening. Fortunately, treatment options have progressed significantly in recent decades which have lead to improvements in prognosis and quality of life.^[4]

Last updated: 3/6/2015

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Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are studying or have studied Tracheobronchomalacia. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

Cedars-Sinai has an information page on Tracheobronchomalacia. Please click the link to access this resource.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
PubMed is a searchable database of medical literature and lists journal articles that discuss Tracheobronchomalacia. Click on the link to view a sample search on this topic.

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I have just been diagnosed with TBM and I want to find out as much as possible about this disease, specifically regarding diagnosis, treatment, and prognosis. See answer
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References References

Ernst A, Carden K, Gangadharan SP. Tracheomalacia and tracheobronchomalacia in adults. In: Basow, DS. UpToDate. Waltham, MA: UpToDate; 2013;
Christopher M Oermann, MD. Congenital anomalies of the intrathoracic airways and tracheoesophageal fistula. UpToDate. April 2014; Accessed 3/5/2015.
Carden KA, Boiselle PM, Waltz DA, and Ernst A. . Tracheomalacia and Tracheobronchomalacia in Children and Adults: An In-Depth Review. Chest. 2005; 127(3):984-1005. http://www.ncbi.nlm.nih.gov/pubmed/15764786. Accessed 9/12/2013.
Ridge CA, O'donnell CR, Lee EY, Majid A, Boiselle PM. Tracheobronchomalacia: current concepts and controversies. Journal of Thoracic Imaging. 2011; 26:278-289. http://www.ncbi.nlm.nih.gov/pubmed/22009081. Accessed 11/20/2012.
Choo EM, Seaman JC, Musani AI.. Tracheomalacia/Tracheobronchomalacia and hyperdynamic airway collapse. Immunol Allergy Clin North Am. February 2013; 33(1):23-34.
Daniel S Schwartz, MD, FACS. Tracheomalacia. Medscape Reference. March 2014; http://emedicine.medscape.com/article/426003-overview.