• Idiopathic Subglottic Stenosis

    (ISGS)

    Definition

    Subglottic stenosis is a narrowing of the windpipe in the area just below the vocal cords. It can make it difficult for air to get through the windpipe and into the lungs.
    The narrowing may also affect your voice.
    Air Pathway
    IMAGE
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    Causes

    The term idiopathic means the exact cause is unknown or the condition occurs spontaneously.

    Risk Factors

    ISGS almost always affects women. It usually affects those aged 30-50 years, but can occur in those who are younger. The connection between ISGS and these risk factors is not clear.

    Symptoms

    Symptoms of ISGS may include:
    • Difficulty breathing or shortness of breath, which may occur with or without activity
    • High-pitched noise heard during breathing—stridor
    • Persistent, barking cough
    • Hoarseness
    • Increase in phlegm
    Breathing problems may worsen over time. At first, breathing may only be difficult during physical activity. As ISGS progresses, you may also have a hard time breathing during rest. If you have acute difficulty breathing, call for emergency medical services right away.

    Diagnosis

    You will be asked about your symptoms and medical history. A physical exam will be done. Pulmonary function tests will also be done to test your ability to move air in and out of your lungs. Your doctor may suspect subglottic stenosis based on the pulmonary tests and medical history.
    Imaging tests of the airway may show narrowing. These may include:
    A scope may be passed down the throat to look for tissue problems. A sample of tissue can also be removed to look for signs of inflammation or disease.
    ISGS is graded from 1-4, with 1 being mild and 4 being the most severe. Other tests may be done to determine a specific cause.

    Treatment

    ISGS is rare. There is no standard treatment that works for everyone. A combination of treatments may work best.

    Mild Stenosis

    Treatment for mild stenosis may include:
    Treatment for mild stenosis may include:
    • Watchful waiting—Stenosis is monitored for the appearance of or changes in symptoms that may need treatment.
    • Medications to control factors that can make stenosis worse, like gastric reflux.
    • Oral, inhaled, or injected corticosteroids to reduce inflammation and swelling in the airway.
    • Bronchoscopy with surgery to help open the airway—A scope is passed down the throat to remove scar tissue or a balloon is used to press open the tissue.

    Severe Stenosis

    Treatment for severe stenosis may include:
    Treatment for severe stenosis may include:
    • Tracheostomy—An opening is made through the skin and throat below the narrowed section. A tube is inserted through the opening to allow airflow in and out of the lungs. Tracheostomy may be temporary or permanent.
    • Stent—Once the airway is opened during bronchoscopy, a mesh or plastic tube is placed into the narrowed section to hold it open.
    • Open surgery—An incision is made in the neck for 1 of the following:
      • Laryngotracheal reconstruction—A small section of damaged windpipe is removed. A piece of rib tissue is used to replace the removed tissue.
      • Cricotracheal resection—The section of damaged windpipe is removed. The remaining airway is reconnected.
    Some treatments may produce temporary results and need to be repeated.

    Prevention

    There are no current guidelines to prevent ISGS because the cause is unknown.

    RESOURCES

    American Academy of Otolaryngology—Head and Neck Surgery http://www.entnet.org

    American College of Chest Physicians http://www.chestnet.org

    CANADIAN RESOURCES

    Canadian Society of Otolaryngology—Head and Neck Surgery http://www.entcanada.org

    Canadian Thoracic Society http://www.cts.lung.ca

    References

    About subglottic stenosis. The Children’s Hospital of Philadelphia website. Available at: http://www.chop.edu/conditions-diseases/subglottic-stenosis/about#.V7b6iE2FPIV. Accessed August 18, 2016.

    Idiopathic subglottis stenosis. NORD—National Organization for Rare Diseases website. Available at: http://rarediseases.org/rare-diseases/idiopathic-subglottic-stenosis. Updated 2015. Accessed August 18, 2016.

    Subglottic stenosis. Baylor College of Medicine website. Available at: https://www.bcm.edu/healthcare/care-centers/institute-voice-swallowing/conditions/subglottic-stenosis. Accessed August 18, 2016.

    Subglottic stenosis. Rush University Medical Center website. Available at: https://www.rush.edu/services/conditions/subglottic-stenosis. Accessed August 18, 2016.

    Revision Information

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