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What is tracheomalacia?

Tracheomalacia is the collapse of the airway when breathing. This means that when your child exhales, the trachea narrows or collapses so much that it may feel hard to breathe. This may lead to a vibrating noise or cough.

Tracheomalacia can result in recurring respiratory illnesses or make it difficult to recover from a respiratory illness. In the long term, it can lead to progressive lung injury. Tracheomalacia has many different forms. Some children will only experience mild forms. For others, this condition can be life threatening and require immediate intervention to allow your child to breathe regularly again.

Tracheomalacia | Symptoms & Causes

What are the symptoms of tracheomalacia?

There are many types of tracheomalacia, but common symptoms include:

  • high-pitched breathing
  • rattling or noisy breathing (stridor)
  • frequent infections in the airway, such as bronchitis or pneumonia (because your child can’t cough effectively or otherwise clear their lungs)
  • frequent noisy cough
  • exercise intolerance
  • prolonged respiratory infections

More severe signs may include:

  • choking during feeding
  • a halt in breathing, particularly when crying or during strenuous activity
  • blue spells (child appears blue because they aren’t getting enough oxygen)

What causes tracheomalacia?

A healthy trachea is supported by a series of C-shaped rings made of cartilage that help your child’s airway to stay open during exhalation. The most common form of tracheomalacia occurs when the rings are wide and shaped more like a letter “U,” causing the membrane at the back of the airway to interfere with breathing and restrict airflow.

It is often incorrectly assumed that the condition results when the cartilage that goes around the trachea isn’t strong enough to fully support it. While this theory has long been taught, newer research and our extensive experience at Boston Children’s prove this to be an uncommon cause. Other types of tracheomalacia occur in the lower trachea or bronchi (the branching airways) — known as bronchomalacia — or are caused by a cyst (mass) in the chest or in the airway itself. Almost all babies with esophageal atresia have some degree of tracheomalacia. Sometimes, however, tracheomalacia can occur on its own, without another condition.

Tracheomalacia may be congenital (present at birth), or acquired later. Acquired tracheomalacia, also known as secondary tracheomalacia, can be caused by:

  • a previous treatment for esophageal atresia or another medical condition
  • heart anomalies, such as vascular rings
  • other internal structures or masses that push on the trachea, causing it to narrow
  • recurrent infection
  • tracheostomy tubes

Tracheomalacia | Diagnosis & Treatments

How is tracheomalacia diagnosed?

The first step in treating your child is forming an accurate and complete diagnosis. Sometimes a child is incorrectly diagnosed with severe asthma when they really have tracheomalacia. This is one reason why it's very important that your child's physician has lots of experience diagnosing and treating this condition.

There are multiple ways to diagnose tracheomalacia, but the best is a procedure called a three-phase dynamic bronchoscopy. During this test, your child's doctor will use a thin instrument called a bronchoscope to look in your child's airway in three different situations: during shallow breathing, during vigorous coughing, and when the airways have been distended with water.

The physician may also use a dynamic airway CT scan, a noninvasive procedure that uses x-ray equipment and computers to create detailed, cross-sectional images of your child's body.

How is tracheomalacia treated?

A common misconception is that children outgrow tracheomalacia over time. Your infant should be monitored closely by their care team and may benefit from humidified air, chest physical therapy, and perhaps a continuous positive airway pressure (CPAP) device and a pulmonary clearance regimen supervised by a pulmonologist.

However, if your child is having blue spells, choking when eating, episodes of choking, problems with noisy breathing, coughing, or exercise intolerance or recurrent pneumonia despite maximal medical treatment, surgery may be necessary. The treatments depend on the type and locations of the tracheomalacia, and each treatment is customized for the individual child. Some of these procedures were developed at Boston Children's and are not yet available elsewhere.

Surgical options include:

Aortopexy. This safe and reliable procedure provides immediate and permanent relief of some types of severe tracheomalacia. This surgery opens up the trachea by moving up the aorta (the body's main blood vessel) and attaching it to the back of the breastbone (sternum).

Tracheopexy. Similar to an aortopexy, this procedure opens up and supports the airway by suspending the front of the tracheal wall from the back of the sternum. Sometimes the thymus gland is removed to create more space between the aorta and the sternum.

Bronchopexy. This procedure may improve airflow in airway collapse that involves the bronchial airways.

Posterior aortopexy. In some situations, the aorta on the spine compresses the back of the airway. In these children, moving the aorta back can take the pressure off the airway and improve airflow.

Posterior tracheopexy. This operation was developed at Boston Children's. Our surgeons can support the flexible back wall of the trachea against the spine. It is much more effective than the other options alone and can be used in combination with the other procedures.

Tracheal diverticulum resection. After esophageal atresia with tracheoesophageal fistula (TEF) repair, a pouch or diverticulum usually remains that can trap secretions and collapse the airway. Our surgeons have developed techniques to completely resect (remove) these lesions to make the airway nearly normal.

Combined procedures. Our surgeons very often discover more than one problem with the airway, esophagus, or both. Using a variety of techniques, they will correct all the problems in one combined procedure. This procedure may include:

  • anterior/posterior tracheopexies
  • slide tracheoplasty: a technique to make the airway larger
  • rotational esophagolplasty: a technique to rotate the esophagus away from the trachea so that there is no chance for recurrence of a TEF
  • descending aortopexy

Placing a stent. Your child's doctor may also choose to temporarily place a stent in your child's trachea. This narrow tube holds the trachea open and encourages the tissues to grow around it. Stents are generally avoided in favor of other surgical options; however, our doctors have the most experience with tracheal stents in the country.

How we care for tracheomalacia

Tracheomalacia is often undetected or misdiagnosed, often as asthma, recurrent croup, or simply noisy breathing. You may have been told that this is the “new normal” for your child or that your child will eventually grow out of it. Many families — and even some doctors — do not know that there are surgical treatments for tracheomalacia.

Although this condition can improve on its own, some children don’t outgrow more severe forms of tracheomalacia. Instead, they adapt to it and learn to live with the discomfort and complications. Our doctors don’t want your child to have to adapt to discomfort or dysfunction. That’s why the Boston Children’s Hospital Esophageal and Airway Treatment Center offers advanced surgical options that can provide your child with complete relief.

Tracheomalacia | Programs & Services