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. One typically develops during infancy or early childhood (primary TBM). The other is an acquired form usually seen in adults (acquired or secondary TBM).^[2] Some people with TBM may initially have no signs or symptoms. However, the condition typically is progressive (worsening over time) and most people will eventually develop shortness of breath, cough, sputum retention (inability to clear mucus from the respiratory tract), and/or 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 acquired form may occur due to trauma, chronic inflammation, and/or prolonged compression of the airways.^[2]^[1] The acquired form is often seen in adults with common respiratory conditions such as asthma, chronic bronchitis, and emphysema. In many cases, the cause is not known. Treatment may only be needed if signs and symptoms are present, of there is complete or near-complete collapse of the airway.^[4] Depending on severity, treatment options may include continuous positive airway pressure (CPAP), stenting, surgical correction (tracheobronchoplasty), and tracheostomy.^[1]^[5]^[6]^[4]

Last updated: 3/22/2018

Symptoms Symptoms

Tracheobronchomalacia (TBM) 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 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]^[7]^[2]

Last updated: 3/5/2015

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Cause Cause

The cause of tracheobronchomalacia (TBM) varies depending on whether a person has primary TBM (also called congenital TBM) or acquired TBM (also called secondary TBM). Most cases of primary TBM are caused by underlying genetic conditions that weaken the walls of the airway (the trachea and bronchi). For example, primary 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 (such as a tracheoesophageal fistula).^[3]^[1]^[5] A small proportion of adults with TBM have the primary form but are not diagnosed until adulthood.^[4]

Acquired TBM is generally caused by the degeneration (break down) of cartilage that typically supports the airways. In most adults with acquired TBM, the underlying cause of this cannot be identified.^[4] Many adults diagnosed with acquired TBM have common respiratory conditions such as asthma, chronic bronchitis and emphysema.^[4] Acquired TBM may be associated with inflammatory conditions (such as relapsing polychondritis), exposure to toxins (e.g. mustard gas), enlargement of structures near the airway (such as goiter or a tumor), and complications from medical procedures (such as endotracheal intubation).^[3]^[1]^[5]^[4]

Last updated: 3/22/2018

Inheritance Inheritance

Primary tracheobronchomalacia (TBM) is often associated with certain genetic conditions.^[3]^[1]^[6] 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

TBM ideally should be managed by a team of specialists working together, which includes interventional pulmonologists, radiologists, and thoracic surgeons. Treatment may only be needed if signs and symptoms are present, quality of life is impaired, and/or there is complete or near-complete collapse of the airway.^[4] Initial management of symptoms may depend on whether an underlying condition is contributing to symptoms, and whether that condition can be managed.^[1] In some cases, treating the underlying condition improves the symptoms of TBM.^[8] Depending on severity, treatment options for TBM that continues to progress may include:^[1]^[5]^[6]^[4]^[8]

Silicone and/or long-term stenting.
Tracheobronchoplasty - this is a surgical procedure used to stabilize the central airway by splinting the posterior wall of the trachea with mesh. The mesh becomes incorporated into the wall of the trachea and then stiffens. A person may be considered a candidate for this procedure depending on results of a short-term trial of stenting.
Resection and reconstruction - this is a surgical procedure that may be used when TBD is more confined (focal).
Continuous positive airway pressure (CPAP) - this has shown variable success and requires an external device which may interfere with daily activities.
Tracheostomy - this may be used as a last resort and can sometimes worsen TBM.

Even with treatment, continued surveillance is recommended to identify any progression or recurrence of symptoms.^[4]

Last updated: 3/22/2018

Prognosis Prognosis

The long-term outlook associated with tracheobronchomalacia (TBM) varies depending on whether it is primary or acquired, and the underlying cause. In general, the prognosis is good in children with primary TBM who do not have any associated problems. Most 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]

Acquired TBM typically is progressive and tends to worsen over time for most people.^[3]^[1]^[9] TBM can cause significant breathing problems and reduced quality of life. Fortunately, treatment options have progressed significantly in recent decades, leading to improvements in prognosis and quality of life.^[9]^[5] TBD can cause sudden respiratory failure, which can be life-threatening; prompt evaluation of symptoms and appropriate treatment is important.^[10]

Last updated: 3/22/2018

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 related to Tracheobronchomalacia. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Coping With Laryngomalacia, Inc
PO Box 313
Port Monmouth, NJ 07758
E-mail: CopingWithLM@yahoo.com
Website: http://www.copingwithlm.org
Cure TBM
c/o Jennifer Champy
140-A Amicks Ferry Road
PMB 225
Chapin, SC 29036
Telephone: 1-973-534-7158
E-mail: info@curetbm.org
Website: https://www.curetbm.org/

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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.

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.

Selected Full-Text Journal Articles

Buitrago DH, Wilson JL, Parikh M, Majid A, Gangadharan SP. Current concepts in severe adult tracheobronchomalacia: evaluation and treatment. J Thorac Dis. January, 2017; 9(1):E57-E66.

News & Events News & Events

News

NCATS Rare Diseases Are Not Rare! Challenge
October 9, 2018
The NIH Undiagnosed Diseases Network Expands
September 26, 2018

GARD Answers GARD Answers

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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; 2015; https://www.uptodate.com/contents/tracheomalacia-and-tracheobronchomalacia-in-adults.
Christopher M Oermann, MD. Congenital anomalies of the intrathoracic airways and tracheoesophageal fistula. UpToDate. 2015; https://www.uptodate.com/contents/congenital-anomalies-of-the-intrathoracic-airways-and-tracheoesophageal-fistula.
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.
Buitrago DH, Wilson JL, Parikh M, Majid A, Gangadharan SP. Current concepts in severe adult tracheobronchomalacia: evaluation and treatment. J Thorac Dis. January, 2017; 9(1):E57-E66. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5303067/.
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.
Majid A. Tracheomalacia and tracheobronchomalacia in adults. UpToDate. Waltham, MA: UpToDate; June 3, 2016; https://www.uptodate.com/contents/tracheomalacia-and-tracheobronchomalacia-in-adults.
Choo EM, Seaman JC, Musani AI. Tracheomalacia/Tracheobronchomalacia and hyperdynamic airway collapse. Immunol Allergy Clin North Am. February, 2013; 33(1):23-34. https://www.ncbi.nlm.nih.gov/pubmed/23337062.
de Mello RA, Magalhães A, Vilas-Boas AJ. Stridor and respiratory failure due to tracheobronchomalacia: case report and review of the literature. Sao Paulo Med J. 2012; 130(1):61-64.