Subglottic Stenosis

What is subglottic stenosis?

Subglottic stenosis is when the upper section of your trachea (windpipe) is narrower than usual so that there’s a whistling noise when you breathe, or you feel short of breath. “Subglottic” means the part of your trachea just below your vocal cords and “stenosis” means there’s a narrowing.

Anyone can have subglottic stenosis. Your newborn baby may be born with an unusually narrow airway (congenital subglottic stenosis). Or you could develop it after you’re born (acquired subglottic stenosis). Several things may cause subglottic stenosis in adults, but it can happen for no known reason (idiopathic subglottic stenosis).

Without treatment, severe subglottic stenosis can be life-threatening. But treatment to widen the narrow area in your windpipe can effectively treat the condition.

How common is subglottic stenosis?

Subglottic stenosis is a rare condition, affecting 1 in every 400,000 people.

What are the symptoms of subglottic stenosis?

Subglottic stenosis symptoms can be mild, moderate or severe, depending on the narrowness of your airway. Think of sipping water through a flexible straw. You only get a sip of water when the straw is wide open. Squeeze it a little, and there’s less to drink. The more you squeeze, the less water flows through the straw.

In mild or moderate subglottic stenosis, part of your airway is narrower than usual. In severe subglottic stenosis, that part of your airway is nearly closed shut so that very little air that comes in through your nose makes it through your windpipe and into your lungs.

If your baby has this condition, they may have:

• Stridor, which is a high-pitched whistling noise when your baby breathes in or out.
• Dyspnea (difficulty breathing).
• Recurring croup.

If you or your older child has subglottic stenosis, symptoms may include:

• A cough that doesn’t go away or you bring up more mucus than usual when you cough.
• Shortness of breath (dyspnea) that doesn’t improve with inhalers or other treatments
• Stridor.

What causes subglottic stenosis?

The condition can happen if your baby’s airway is narrower than usual (congenital subglottic stenosis). A child or an adult may develop it if they:

• Need intubation on a ventilator. People who need intubation for more than two weeks can sometimes develop subglottic stenosis.
• Have an injury that damages their airway.
• Have a rare autoimmune disorder or vasculitis that causes scar tissue to build up in their airway.

Some of the time, however, there’s no known reason why people develop subglottic stenosis. These cases tend to affect people assigned female at birth (AFAB) ages 30 to 60.

How is subglottic stenosis diagnosed?

Your healthcare provider will perform a physical examination. They’ll ask about your medical history and your symptoms. If they suspect subglottic stenosis, they may refer you to an ear, nose and throat specialist (ENT or otolaryngologist). The specialist may perform tests, including:

• Flexible laryngoscopy: Your provider inserts a flexible tube down your throat. There’s a tiny camera on the tube that allows your provider to look at your larynx (voice box) and windpipe.
• Pulmonary function test: This test measures the amount of air that you can breathe in or out. Reduced airflow is a sign of subglottic stenosis.
• Computed tomography (CT) scan: Your provider may order a CT scan so they can look at the structure of your airway.

How is subglottic stenosis treated?

Subglottic stenosis is rare, so there’s no standard or common treatment for it. Treatments may include checking on your condition, steroid injections and surgery.

Monitoring

If tests show subglottic stenosis is causing mild narrowing of your airway, your provider may schedule regular appointments so they can check your airway. They may do more tests to see if your airway is narrowing.

Steroid injections

Your provider may do steroid injections as initial or first-line treatment. The treatment involves injecting a steroid through your neck into your airway. (Your provider will use local anesthesia to numb your neck, airway and nose so you won’t have pain during treatment.) Typically, people who have this treatment have a series of injections every four to six weeks.

Surgery for subglottic stenosis

There are a few different surgical procedures to treat subglottic stenosis:

• Endoscopic dilation: In this treatment, your provider uses an endoscope to place a tiny balloon in the narrow area of your windpipe. The balloon dilates (stretches) the section. An endoscopic dilation isn’t a permanent solution: at some point, you’ll need to have the procedure again to keep your airway open.
• Cricotracheal resection: This surgery involves removing the narrowed part of your airway just below your voice box and then reconnecting your airway and voice box.
• Endoscopic laryngotracheoplasty: In this surgery, providers widen your airway by placing pieces of cartilage in your airway. Surgeons may call this surgery laryngotracheal reconstruction.

What can I expect if I have subglottic stenosis?

Often, prompt treatment can effectively treat the condition. But subglottic stenosis can come back, which means you or your child will need more treatment.

When should I see my healthcare provider?

If you or your child has treatment for subglottic stenosis, contact your provider as soon as you notice changes or symptoms, like stridor or breathing issues, that may mean the condition is coming back. You or your child may need more treatment to prevent symptoms from getting worse.

What questions should I ask my doctor?

If you or your child has subglottic stenosis, you may have the following questions:

• How severe is my condition/my child’s condition?
• Will I need treatment right away?
• What treatment do you recommend for my child?

A note from Cleveland Clinic

There’s a noisy, whistling sound when your baby breathes. They have croup that keeps coming back, along with rasping breath and a distinctive cough that sounds like a seal barking. Or you may feel short of breath for no reason and have a cough that won’t go away. Tests show subglottic stenosis is the reason why breathing is such hard work for you and/or your child.

It can be scary to learn your airway isn’t working like it should. Fortunately, there are many treatments for subglottic stenosis that can ease symptoms and sometimes cure the issue. Your healthcare provider will recommend the treatment that’s right for you and/or your child. And they’ll be happy to answer any questions you have about what you can expect after treatment.