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(ISGS)

Definition

Idiopathic subglottic stenosis (ISGS) is a narrowing of the windpipe in the area just below the vocal cords. It can make it hard to breathe and speak. It is rare.

Air Pathway
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Causes

The exact cause is not known.

Risk Factors

This problem almost always affects women. It more common in women who are 30 to 50 years of age.

Symptoms

Problems may be:

  • Shortness of breath with or without activity
  • Breathing that worsens over time
  • A high-pitched noise when breathing
  • A lasting, barking cough
  • A hoarse voice
  • Increased mucus

Diagnosis

You will be asked about your symptoms and health history. A physical exam will be done. Lung function tests will also be done to test how well air moves in and out of the lungs. This may be enough to suspect the diagnosis.

Images will be taken to look for signs of airway narrowing. This can be done with:

A tissue sample may be taken to look for signs of ISGS. This can be done with a bronchoscopy.

Treatment

There is no one treatment that works for everyone. It may need to be treated using more than one method. Options are:

Mild to Moderate Stenosis

It may be treated with:

It may be treated with:

  • Watchful waiting—Looking for changes that may need treatment
  • Medicines to manage problems that can make stenosis worse, such as gastric reflux
  • Corticosteroids to ease 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
  • Endoscopic expansion surgery—A scope with a laser or knife is passed down the throat and used to open part of the windpipe to add a piece of cartilage and widen it

Severe Stenosis

Severe problems may be treated with:

Severe problems may be treated with:

  • Tracheostomy —An opening is made through the skin and throat below the narrowed section. A tube is inserted through the opening to allow air to flow in and out of the lungs. It may be used in the short or long-term.
  • 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 one 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 need to be repeated.

Prevention

There are no current guidelines to prevent ISGS. The cause is not known.

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 July 27, 2020.

Idiopathic subglottis stenosis. NORD—National Organization for Rare Diseases website. Available at: http://rarediseases.org/rare-diseases/idiopathic-subglottic-stenosis. Updated 2018. Accessed July 27, 2020.

Subglottic stenosis. Rush University Medical Center website. Available at: https://www.rush.edu/services/conditions/subglottic-stenosis. Accessed July 27, 2020.

Revision Information