Centers of Excellence

The Lung Center

Tracheal Disorders

The trachea, known as the airway or windpipe, is a tube that starts under the larynx (voice box) and runs behind the breastbone. It then divides into two smaller tubes, (bronchi) which lead to the lungs. When breathing, a normal trachea widens and lengthens with each breath. Inflammation can cause scarring and narrowing of the trachea, while birth defects or injury can cause the trachea to become soft and floppy. Tumors can also cause blockage of the trachea or the main bronchi. All of these conditions can severely affect your breathing.

Board-certified thoracic surgeons and pulmonologists at the Brigham and Women’s Hospital Lung Center (BWH) provide comprehensive, specialized care for patients experiencing tracheal disorders. Their expertise and collaboration with other specialists throughout BWH provide patients with a highly informed diagnosis and a cohesive treatment plan. Should surgery or a procedure be required, our surgeons and interventional pulmonologists are experts in tracheal resection and reconstruction surgery and collaborate with specialized otolaryngologists to ensure the best outcomes.

Tracheal Disorders Topics

Types of Tracheal Disorders

There are two main types of tracheal disorders:

  • Tracheal stenosis is a narrowing of the trachea, or “windpipe,” that prevents air from fully reaching the lungs. The effects of this narrowing can range in severity from mild to more severe. In the most severe cases, the patient may be dependent on a tracheotomy tube to breathe.
  • Tracheomalacia is a condition characterized by cartilage in the walls of the trachea that has broken down, semi-causing weakness or floppiness in the windpipe. The trachea should be rigid for normal breathing. Infants can be born with tracheomalacia, but adults can also acquire it in later life.

Tracheal tumors, while rare, can cause narrowing of the windpipe which prevents air from fully reaching the lungs. They can also cause coughing of blood. Types of tumors include:

  • Benign: pleomorphic adenoma, squamous cell papilloma, chondroma, granular cell tumor, glomus tumor, neurofibroma
  • Malignant: adenoid cystic carcinoma, squamous cell carcinoma, malignant epithelial tumor, carcinoid tumor, mucoepidermoid carcinoma, small cell and non-small cell lung cancer

Other tracheal disorders managed by The Lung Center include tracheo-esophageal fistula, an abnormal connection (fistula) between the esophagus and the trachea, and tracheobronchomalacia, a rare condition that occurs when the airway walls are weak, leading them to narrow or collapse.  

Causes of Tracheal Disorders

Tracheal Stenosis

The most common cause of tracheal stenosis is intubation, when a patient has had a breathing tube inserted into the trachea for surgery or other medical procedures. Other causes may include:

  • External trauma to the throat or chest
  • Thermal or caustic injuries
  • Chronic inflammatory disease
  • Infections, including tuberculosis
  • Tumors that press against the trachea
  • Radiation therapy
  • Idiopathic (unknown) cause common in young women
  • Pressure from other structures in the chest such as blood vessels
  • Autoimmune disorders, such as: sarcoidosisWegener’s granulomatosis and amyloidosis

Tracheomalacia

Tracheomalacia can have many causes, the most common are:

  • Damage from surgery or procedures on the trachea or esophagus
  • Damage from long-term breathing tube or tracheostomy
  • Chronic infections such as bronchitis
  • Polychondritis (inflammation of the cartilage in the trachea)
  • Emphysema
  • Gastroesophageal reflux disease (GERD)
  • Inhaling irritants
Symptoms of Tracheal Disorders

Tracheal Stenosis

Many patients do not experience any symptoms of tracheal stenosis. However, you may exhibit one or more of the following:

  • Wheezing
  • Stridor (a high-pitched, musical breathing sound)
  • Shortness of breath
  • Difficulty breathing/respiratory distress
  • Coughing
  • Hoarseness
  • Frequent upper respiratory infections, such as pneumonia
  • Asthma that resists treatment
  • Bluish color to the skin
  • Bluish color in the mucous membrane of the mouth or nose
  • Hemoptysis or coughing up blood

Tracheomalacia

  • Shortness of breath
  • Chronic cough
  • Hoarseness
  • Recurring respiratory tract infections
  • Abnormal breathing noises that improve when you change your position or are sleeping
  • High-pitched, noisy breathing, sometimes with a rattling sound
  • Difficulty swallowing food
Diagnosis of Tracheal Disorders

Tracheal Stenosis

In addition to a careful physical examination, The Lung Center team may perform the following procedures to determine if your symptoms are caused by tracheal stenosis:

  • Pulmonary Function Test measures how well the lungs work.
  • CT scan uses a combination of X-rays and computer technology to produce horizontal, or axial, images of any part of the body, including the bones, muscles, fat and organs.
  • Dynamic 3D chest CT scan takes a closer look at the area of stenosis while the patient inhales and exhales to outline the structures for surgical planning.
  • Chest X-ray uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs on film or digital media.
  • Bronchoscopy uses a bronchoscope to examine the inside of the trachea, bronchi (air passages that lead to the lungs)

Tracheomalacia

After taking your medical history and performing a careful physical examination, The Lung Center team may perform the following procedures to confirm a diagnosis of tracheal stenosis or tracheomalacia:

  • 6-minute walk test measures the distance an individual can walk over a period of 6 minutes on a hard, flat surface to determine functional exercise capacity.
  • Advanced cardiopulmonary exercise testing uses catheters during exercise (treadmill or stationary cycling) to measure heart and lung function.
  • Bronchoscopy uses a bronchoscope to examine the inside of the trachea, bronchi (air passages that lead to the lungs). It allows for precise diagnosis of the problem and measurement of the area affected to determine the best possible therapy options.
  • CT scan uses a combination of X-rays and computer technology to produce horizontal, or axial, images of any part of the body, including the bones, muscles, fat and organs.
  • Chest X-ray uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs on film or digital media.
  • Dynamic 3D chest CT scan takes a closer look at the area while the patient inhales and exhales to outline the structures for surgical planning.
  • Laryngoscopy allows your doctor to examine the back of your throat, larynx and vocal cords using a scope (laryngoscope).
  • Magnetic resonance imaging (MRI) uses strong magnetic fields, radio waves and field gradients to image the anatomy and physiological processes of the body. This procedure provides a non-invasive method of assessing the trachea.
  • Pulmonary Function Test measures how well the lungs work.
  • Pulmonary ventilation/perfusion scan involves two nuclear scan tests: injection of radioactive albumin into your veins and breathing radioactive gas through a mask to allow a machine to measure circulation (perfusion) and breathing (ventilation).
Treatment for Tracheal Disorders

Tracheal Stenosis

There are several surgical options to treat tracheal stenosis. Your thoracic surgeon will recommend the best option for you, based on the cause, location and severity of the narrowing. Possible treatments and procedures are:

  • Argon plasma coagulation (APC) is a form of non-contact electrocoagulation using a flexibile bronchoscopy which allows for rapid coagulation with minimal mechanical trauma to the tissue. This procedure is used for patients who are unable to undergo tracheal reconstruction for medical or personal reasons.
  • Bronchial artery embolization (BAE), a procedure involving X-rays to examine the bronchial arteries using a special dye to determine what is causing your haemoptysis so the bleeding can be stopped.
  • Bronchoscopic Tracheal dilation uses a balloon, tracheal dilator or electrocautery to widen the trachea. This procedure provides immediate relief for symptoms as well helps your thoracic surgeon determine the extent of the stenosis.
  • Laser bronchoscopy uses lasers to remove scar tissue and proves excellent short-term relief for symptoms.
  • Tracheobronchial airway stent or T-tube, a non-invasive procedure that uses a bronchoscope to place a stent to keep the airway open.
  • Tracheal resection and reconstruction, where the scarred and constricted section of the trachea is removed and the upper and lower sections are rejoined. This treatment has excellent long-term results and is the first indicated procedure for certain tumors and stenosis.
  • Tracheobronchoplasty, a procedure performed at few hospitals across the United States, that involves suturing mesh to the outside of a patient’s trachea through a series of knots, which opens the collapsed tissue in the tracheal wall to create an opening to process air. The goal is that the area will scar over, thereby strengthening the structure and making it permanent.  

Tracheomalacia

Often, tracheomalacia can improve without treatment. However, you should be monitored closely if you suffer from frequent respiratory infections. Treatments may include:

  • Continuous positive airway pressure (CPAP) uses mild pressure to keep the airway open.
  • Chest therapy, including deep breathing exercises and tapping the chest to break up mucus.
  • Speech and language therapy to adjust breathing patterns and swallowing issues.
  • Tracheobronchial airway stent or T-Tube, a non-invasive procedure that uses a bronchoscope to place a stent to keep the airway open.
  • Surgical Treatment
    • Tracheal resection and reconstruction, a surgical method that removes the constricted section of the trachea and rejoins the upper and lower sections. This treatment often has excellent long-term results.
    • Tracheostomy, a surgical procedure that creates an opening to the trachea through the neck.
    • Tracheoplasty, a surgical method to support the floppy airway and prevent its collapse. This can be achieved with the tightening of the loose trachea and the support of surrounding tissue or plastic mesh.

Tracheal Tumors

In addition to using some of the surgical procedures listed above, your physician may treat your tumor using some of the following therapies:

  • Brachytherapy involves locally-delivered radiation therapy to the airway.
  • Photodynamic therapy (PDT) uses the light of a specific wavelength to kill cells and damage tumor vasculature, with the goal of inducing an inflammatory reaction that helps to eliminate the tumor.
What You Should Expect

When you become a patient of The Lung Center you will meet with many members of the team who will carefully review your medical history and conduct a thorough diagnostic evaluation. You will receive a recommendation for a therapy tailored just for you, based on your specific disorder and other factors, as well as comprehensive monitoring.

Multidisciplinary Care

Patients with tracheal disorders benefit from the wide range of expertise at The Lung Center. Collaboration between thoracic surgeons, pulmonologists, otolaryngologists, radiologists, respiratory and speech therapists and other specialists and other specialists ensures comprehensive evaluation and effective treatment. If your medical team discovers an underlying illness or concern, you will be referred to a BWH physician for an expert evaluation.

Any surgery recommended will be performed by an experienced, board-certified thoracic surgeon, interventional pulmonologist or interventional radiologist who is an expert in tracheal disorders, in collaboration with the treatment team that including nurses and physician assistants who specialize in caring for patients with tracheal disorders. Your surgeon will also collaborate with pulmonologists and otolaryngologists to tailor a treatment plan for you. Our specialized care team has some of the best results in the country.  

Resources

Go to our online health library to learn more about thoracic diseases and tests.

Visit the Kessler Health Education Library in the Bretholtz Center for Patients and Families to access computers and knowledgeable staff.

Access a complete directory of patient and family services.

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