Subglottic Stenosis

Overview

What is subglottic stenosis?

Subglottic stenosis is a condition in which your subglottis (a portion of your windpipe, just below your vocal cords) narrows. It’s usually characterized by inflammation and fibrosis (scar tissue) in the area. Sometimes, the condition is called idiopathic subglottic stenosis (iSGS). The term idiopathic means that experts don’t know the underlying cause.

What's the difference between congenital subglottic stenosis and acquired subglottic stenosis?

There are two main types of subglottic stenosis — congenital and acquired:

  • Congenital subglottic stenosis occurs when a baby is born with an airway that’s smaller than usual.
  • Acquired subglottic stenosis is not present at birth. It develops later because of trauma, infection or complications due to intubation (when a breathing tube is inserted into the airway).

Who does subglottic stenosis affect?

Subglottic stenosis can affect newborn babies, children and adults. People are either born with the condition or they develop it as a complication later on. Statistically, idiopathic subglottic stenosis affects females almost exclusively (98%) — most of them white (95%).

How common is subglottic stenosis?

Subglottic stenosis is considered a rare disease. It occurs in about 1 out of every 400,000 people.

Symptoms and Causes

What are the symptoms of subglottic stenosis?

People with subglottic stenosis may experience a few different symptoms, including:

Some subglottic stenosis symptoms are similar to those of asthma, bronchitis or other bronchi-related conditions. For this reason, some people with subglottic stenosis are misdiagnosed.

What are common subglottic stenosis symptoms in infants?

Babies with subglottic stenosis often exhibit noisy breathing. Additional symptoms may include:

  • Cyanosis (when the skin has a blue hue).
  • Poor weight gain.
  • Respiratory distress.
  • Croup or lung infections that keep coming back.

What causes subglottic stenosis?

Though subglottic stenosis can be caused by trauma or complications of intubation, the causes of many cases are not known. This is called idiopathic subglottic stenosis. Researchers don’t know exactly what causes idiopathic subglottic stenosis. Though there are many theories that take genetic, environmental and immunologic factors into account, there isn’t currently enough evidence to provide conclusive answers.

Diagnosis and Tests

How is subglottic stenosis diagnosed?

Your healthcare provider will perform a physical examination and ask you about your symptoms. If they suspect subglottic stenosis, they may perform a number of tests, which may include:

  • Flexible laryngoscopy. A flexible tube with a tiny camera attached is inserted down your throat. This allows your healthcare provider to view your voice box and windpipe directly.
  • Pulmonary function test. During this assessment, you’ll be asked to breathe into a spirometer (a device that measures the amount of air you can breathe in or out). If your airflow is reduced, a pulmonary function test can detect it.
  • Imaging tests. Sometimes, CT (computed tomography) scans may be taken to view structures inside your body.

Management and Treatment

How is subglottic stenosis treated?

Subglottic stenosis is most commonly treated with steroid injections or surgery. In some mild cases, periodic observation is sufficient to see if narrowing of your airway worsens. Because subglottic stenosis is a rare disorder, there is no standardized treatment. Healthcare providers take many factors into account when treating it, including age and severity of the condition.

Steroid injections for subglottic stenosis

Steroid injections are sometimes recommended after endoscopic dilations, or as a first line of treatment in some select people. During this in-office procedure, your healthcare provider numbs your neck, windpipe and nose with local anesthesia. Next, a laryngoscope (a special camera used to look at your voice box and windpipe) is placed through your nose to view the subglottis. A needle is then placed through your neck and into your airway to inject steroid medications. This procedure is only recommended for people who can tolerate a small amount of airway swelling. Furthermore, a series of three injections are typically needed, given every four to six weeks.

Surgery for subglottic stenosis

Subglottic stenosis is surgically treated using one of four methods:

  • Endoscopic dilation. During this procedure, the affected section of your windpipe is dilated (stretched) using a balloon or other surgical instrument. In some cases, a laser may also be used to remove small amounts of tissue from the narrowed portion of your windpipe. It’s important to note that endoscopic dilation usually only offers temporary relief. At some point, the procedure will need to be performed again to maintain results.
  • Endoscopic resection. This procedure is similar to endoscopic dilation. However, a laser is also used to cut away a significant portion of your windpipe. Post-surgical care may involve antibiotics, anti-reflux medications and corticosteroids.
  • Cricotracheal resection. Unlike dilation and endoscopic resection, cricotracheal resection is an open surgery. Rather than using an endoscope to view your windpipe, an incision is made on the front of your neck. Scarred tissue is removed and the problematic areas of your larynx and windpipe are reconstructed.
  • Laryngotracheoplasty. This is another type of open surgery that involves adding a piece of cartilage to your windpipe once it’s been stretched. This piece of cartilage is often taken from your rib, and it’s used to provide support. People who undergo this procedure may need a temporary stent or breathing tube.

Can subglottic stenosis go away on its own?

Yes, sometimes. Mild cases of subglottic stenosis can potentially go away without treatment. That’s why close monitoring is often recommended in these instances.

Care at Cleveland Clinic

Prevention

How can I reduce my risk for subglottic stenosis?

Because all underlying causes of subglottic stenosis have not been discovered, there is no known way to fully prevent the condition from developing.

Outlook / Prognosis

Is subglottic stenosis life threatening?

Severe cases of subglottic stenosis can be dangerous and life-limiting. Therefore, it’s important to seek immediate medical attention if you develop symptoms.

Can subglottic stenosis be cured?

Yes. Most cases of subglottic stenosis can be treated with prompt and proper treatment. However, recurrences do occur and may require multiple treatments down the road.

Living With

When should I see my healthcare provider?

Anytime you have difficulty breathing, call your healthcare provider right away. They may refer you to an ear, nose and throat specialist for testing.

A note from Cleveland Clinic

Whether you have a baby who was born with subglottic stenosis or you developed the condition yourself later in life, it can be challenging to determine which treatment is right for you. Your healthcare provider can answer any questions you have and offer insight regarding your long-term health and recovery.

Last reviewed by a Cleveland Clinic medical professional on 11/01/2021.

References

  • Aravena C, Almeida FA, Mukhopadhyay S, et al. Idiopathic subglottic stenosis: a review. (https://pubmed.ncbi.nlm.nih.gov/32274178/) J Thorac Dis. 2020 Mar;12(3):1100-1111. Accessed 10/1/21.
  • Ferdinande P, Kim DO. Prevention of postintubation laryngotracheal stenosis. (https://pubmed.ncbi.nlm.nih.gov/8525831/) Acta Otorhinolaryngol Belg. 1995;49(4):341-6. Accessed 10/1/21.
  • Fiz I, Bittar Z, Piazza C, et al. Hormone receptors analysis in idiopathic progressive subglottic stenosis. (https://pubmed.ncbi.nlm.nih.gov/28990664/) Laryngoscope. 2018 Feb;128(2):E72-E77. Accessed 10/1/21.
  • Jagpal N, Shabbir N. Subglottic Stenosis. (https://www.ncbi.nlm.nih.gov/books/NBK563265/) [Updated 2021 Jul 25]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Accessed 10/1/21.
  • National Organization for Rare Disorders. Idiopathic Subglottic Stenosis. (https://rarediseases.org/rare-diseases/idiopathic-subglottic-stenosis/) Accessed 10/1/21.
  • Pan DR, Rosow DE. Office-based corticosteroid injections as adjuvant therapy for subglottic stenosis. (https://pubmed.ncbi.nlm.nih.gov/31453351/) Laryngoscope Investig Otolaryngol. 2019 Jun 10;4(4):414-419. Accessed 10/1/21.

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