Tracheal Stenosis

What is tracheal stenosis?

With tracheal stenosis, there is a narrowing of the airway anywhere in the trachea, or windpipe. The trachea begins at the bottom of the voice box and extends down to where it branches off to the lungs in airways called bronchi. Tracheal stenosis can also extend from the trachea into the bronchi, causing bronchial stenosis/narrowing. Children may be born with this narrowing (congenital tracheal stenosis), or it may be caused by a previous injury to the airway, often from a breathing tube or surgery.

Congenital tracheal stenosis

When children are born with tracheal stenosis, it is often caused by a condition called complete tracheal rings. Normally, the cartilage in the trachea is horseshoe shaped and located in the front of the airway. This keeps the airway from collapsing during breathing. The back wall (in the open part of the horseshoe) of the airway is soft, allowing it to expand during breathing. When a child has complete tracheal rings, the airway doesn’t expand. Instead, the flexible portion of the airway is missing, and the narrow, circular cartilage constricts the airway. The stenosis frequently becomes narrower the farther it extends down the trachea. This can cause severe and dangerous respiratory distress, even in very young babies. Usually, the condition is identified shortly after birth or early in childhood. Occasionally, it is identified during the teenage years or even during adulthood.

How is tracheal stenosis identified?

The gold-standard way to identify tracheal stenosis is through a procedure called microlaryngoscopy and bronchoscopy. This involves a minimally invasive procedure through the mouth, using a camera to look through the voice box into the windpipe; it is performed in the ICU or the operating room under anesthesia. This allows our team to measure the narrowing of the airway and determine whether any treatment is needed.

Tracheal stenosis may be suspected in the following circumstances:

  • When a child has difficulty breathing and/or noisy breathing shortly after birth.
  • If an airway malformation is detected in the fetus during pregnancy through an ultrasound and fetal magnetic resonance imaging (MRI).
  • When a fetal ultrasound identifies vascular (blood vessel) abnormalities that have a strong association with complete tracheal rings (e.g., pulmonary artery sling).
  • When imaging studies (such as computed tomography [CT] scan) identify a narrowing of the windpipe.
  • When a child has respiratory symptoms that are diagnosed as asthma but don’t respond to normal asthma treatment.

Who is at risk of tracheal stenosis?

Certain children are at higher risk of having tracheal stenosis. It is associated with certain heart and lung abnormalities, and certain genetic conditions or syndromes, including Down syndrome. For this reason, Lucile Packard Children’s Hospital Stanford’s Aerodigestive and Airway Reconstruction Center brings together the expertise of neonatologists, otolaryngologists, cardiothoracic (CT) surgeons, cardiac intensivists, and cardiologists in a child’s treatment to closely collaborate and develop a surgical plan, when necessary.

Tracheal stenosis commonly affects children who have a blood vessel abnormality called a left pulmonary artery sling (LPAS), a cardiac condition in which the misdirected left pulmonary artery passes between the trachea and the esophagus. More than 50% of children with LPAS also have tracheal stenosis.

At Packard Children’s Hospital, all patients with LPAS and some children with other heart conditions undergo a comprehensive airway evaluation after birth. If tracheal stenosis is identified and treatment is needed, we coordinate our team of specialists to perform the vascular surgery and airway surgery at the same time.

What is the treatment for tracheal stenosis?

For children who are born with tracheal stenosis but are breathing well on their own, our team closely follows them to make sure that their airway is growing appropriately as they get older. If symptoms develop over time, we are prepared to perform an airway reconstruction surgery using a safe, team-based approach.

When airway reconstruction is required, we use state-of-the-art photo and video documentation techniques to evaluate the airways, esophagus, and upper-GI tract for individualized preoperative planning. To reconstruct and enlarge the airway, the most effective operation is a slide tracheoplasty. This complex surgery uses the child’s own tracheal tissues to bridge and expand the narrow segments of trachea, enlarging the airway and restoring tracheal function and breathing. Because children often have associated lung and heart problems, an otolaryngologist (airway surgeon) specializing in airway reconstruction and a cardiothoracic (CT) surgeon work side by side to address the airway, heart, and lung components of the surgery in a single operation. We have a dedicated team of surgeons with specific training and expertise in performing this complicated operation.

What happens after the surgery?

After surgery, children go to a specialized cardiovascular intensive care unit (CVICU), where they are cared for by a team trained to manage patients who have undergone this operation. After a child has recovered from surgery, the Aerodigestive and Airway Reconstruction Center physicians and therapists continue to follow the child to optimize breathing, swallowing, and voice.