Tracheomalacia

What is tracheomalacia?

Tracheomalacia (TRAY-kee-oh-muh-LAY-shia) is when you have weak or floppy cartilage in your trachea (windpipe). The walls of your windpipe can collapse or fall in, causing symptoms like high-pitched breathing. It can also trap mucus in your lungs, making it difficult to clear them out. In severe cases, tracheomalacia may be life-threatening, but it’s curable with treatment.

Tracheomalacia usually affects newborns, but anyone can develop it. Babies born with the condition often develop symptoms when they’re 1 to 2 months old. Then, symptoms improve over the first three years of life. That’s because cartilage strengthens as your baby’s windpipe grows. But severe cases may need surgery.

Types of this condition

There are two types of tracheomalacia:

• Congenital tracheomalacia: “Congenital” means you’re born with the condition. It happens when a baby’s windpipe didn’t form properly during fetal development.
• Acquired tracheomalacia: “Acquired” means the condition developed after you were born. It happens when your windpipe breaks down or gets damaged. Injuries, surgeries or prolonged mechanical ventilation can cause this. Although it’s uncommon, acquired tracheomalacia can occur at any age.

Some people with tracheomalacia also have weak bronchi (the tubes that run from your windpipe to your lungs). Healthcare providers call this condition tracheobronchomalacia.

How common is tracheomalacia?

Congenital tracheomalacia is somewhat rare. Even so, it’s the most common birth defect affecting the windpipe. Approximately 1 in 2,100 children are born with the condition.

Acquired tracheomalacia (which can occur at any age) is also very uncommon.

What are the symptoms of tracheomalacia?

The most common tracheomalacia symptom is high-pitched or noisy breathing (stridor). Other symptoms include:

• Breathing issues that get worse when feeding, crying or coughing.
• Choking.
• Chronic coughing.
• Cyanosis (a condition where your skin, lips or nails turn blue from a lack of oxygen).
• Difficulty swallowing.
• Hoarseness.
• Recurring (returning) airway infections like pneumonia or bronchitis.
• Shortness of breath.
• Wheezing or rattling sounds.

What causes tracheomalacia?

Congenital tracheomalacia happens when the cartilage in your baby’s windpipe doesn’t develop the way it should. The walls of their windpipe are floppy instead of rigid.

Acquired tracheomalacia causes include:

• Chronic acid reflux (GERD).
• Damage from surgery or other medical procedures.
• Emphysema.
• Polychondritis (inflammation of the cartilage) in your windpipe.
• Tracheoesophageal fistula repair (fixing an abnormal connection between your esophagus and trachea).
• Tracheostomy, or long-term use of a breathing tube.
• Upper respiratory infections, like bronchitis.

Tracheomalacia may be associated with other conditions like:

• Developmental delays.
• Ehlers-Danlos syndrome.
• Heart defects.

What are the complications of tracheomalacia?

Without treatment, tracheomalacia can cause:

• Airway obstruction.
• Aspiration pneumonia.
• Growth faltering.
• Upper respiratory infections that linger or come back often.

How is tracheomalacia diagnosed?

A healthcare provider will do a physical exam and ask about your symptoms. They’ll also use laryngoscopy or bronchoscopy to look at your windpipe. To do this, they’ll guide a lighted scope with a camera down your throat.

Your healthcare provider may need to run more tests to diagnose tracheomalacia, like:

• Airway fluoroscopy.
• Barium swallow (esophagram).
• Chest X-ray.
• CT (computed tomography) scan.
• Lung function tests.
• MRI (magnetic resonance imaging).

How is tracheomalacia treated?

Healthcare providers can treat tracheomalacia with nonsurgical therapies, medications or surgery. What’s right for you depends on the extent of the condition.

Nonsurgical therapies

The following treatments help keep your airways open and your lungs clear:

• Breathing humidified air. Using a humidifier can thin out mucus so you can breathe comfortably.
• Chest physical therapy. A physical therapist can teach you breathing exercises to clear mucus from your lungs.
• Continuous positive airway pressure (CPAP). Using a CPAP machine can keep your windpipe from collapsing too much during sleep.

Medications

Healthcare providers use several different medications to treat tracheomalacia, including:

• Antibiotics to treat bacterial infections.
• Bronchodilators to relax the muscles that help you breathe.
• Corticosteroids to reduce inflammation.
• Mucolytics to thin out mucus.

Surgery

Severe tracheomalacia cases may need surgery. Some options include:

• Aortopexy. A surgeon moves your aorta up and away from your airway and attaches it to your breastbone. This keeps your windpipe from collapsing.
• Stenting. This involves placing a stent (hollow tube) in your airway to keep it open.
• Tracheopexy. A surgeon connects part of your windpipe to a nearby structure (like your breastbone or a ligament at the top of your spine). This keeps your windpipe open and prevents collapse.

Can tracheomalacia be prevented?

There’s nothing you can do to reduce your risk for tracheomalacia. But you can manage it with the help of your healthcare provider.

What’s the outlook for tracheomalacia?

After treatment, most people with tracheomalacia go on to live healthy lives with no complications.

Treatment isn’t always necessary. Congenital tracheomalacia usually improves on its own by age 3. As your baby’s tracheal cartilage grows stronger, their symptoms will likely improve. But in some cases, your baby may need medication and/or surgery.

People with tracheomalacia need close monitoring if they develop respiratory infections. Even minor colds can cause complications. Your healthcare provider may recommend treatments or medications to manage your symptoms.

When should I see my healthcare provider?

Schedule an appointment with your healthcare provider if your baby shows tracheomalacia symptoms. This includes things like frequent cough, noisy breathing or prolonged respiratory infections.

Tracheomalacia in adults can cause exercise intolerance and frequent respiratory infections. Tell your provider if you notice these things. They can confirm the diagnosis and recommend appropriate treatment.

What questions should I ask my doctor?

If you have tracheomalacia, here are some questions you may want to ask:

• How severe is the condition?
• What caused the condition?
• What treatment do you recommend?
• Will surgery be necessary?
• What can I do to ease symptoms?
• How soon do I need treatment?

What conditions might be confused for tracheomalacia?

The following conditions may be confused for tracheomalacia:

• Laryngomalacia: This condition refers to floppy tissue above your voice box. Your voice box sits above your windpipe.
• Tracheobronchomalacia: This describes weak bronchi (the tubes that run from your windpipe to your lungs). Some people with tracheomalacia also have tracheobronchomalacia.