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Idiopathic Subglottic Stenosis

Authors:
  • Cynthia M. Johnson, MA
Publication Type:

Condition

Idiopathic Subglottic Stenosis

(ISGS)

Definition

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

Air Pathway.

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Causes

The exact cause of ISGS is not known.

Risk Factors

ISGS almost always affects women. It is more common in women who are 20 to 60 years old.

Symptoms

Symptoms of ISGS may be:

  • Problem breathing 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

The doctor will ask about symptoms and past health. A physical exam will be done.

Lung function tests may 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 scope may be used to assess the upper and lower airway. A tissue sample may be taken with bronchoscopy to look for signs of ISGS.

Treatment

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

For mild to moderate stenosis, options may be:
  • Watchful waiting. The doctor looks for changes that may need treatment.
  • Medicines are used to manage problems that can make stenosis worse, such as gastric reflux.
  • Corticosteroids can help ease swelling in the airway.
  • Bronchoscopy with surgery can help open the airway. The surgeon passes a scope down the throat. The scope is used to remove scar tissue—or a balloon is used to press open the tissue.
  • Endoscopic expansion surgery. The surgeon passes a scope with a laser or knife down the throat. It is used to open part of the windpipe to add a piece of cartilage and widen it.

Severe problems may be treated with:

  • Tracheostomy—The surgeon makes an opening 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:

CANADIAN RESOURCES:

CANADIAN RESOURCES:

References

  • About subglottic stenosis. The Children’s Hospital of Philadelphia website. Available at: http://www.chop.edu/conditions-diseases/subglottic-stenosis/about#.V7b6iE2FPIV.
  • Idiopathic subglottis stenosis. NORD—National Organization for Rare Diseases website. Available at: http://rarediseases.org/rare-diseases/idiopathic-subglottic-stenosis.
  • Subglottic stenosis. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/subglottic-stenosis.
  • Subglottic stenosis. Rush University Medical Center website. Available at: https://www.rush.edu/services/conditions/subglottic-stenosis.

Contributors

  • Daniel A. Ostrovsky, MD
Last Updated:
2022-09-01

(C) Copyright 2022 EBSCO Information Services

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.