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What are complete tracheal rings?

A normal trachea (windpipe) has many rings made of cartilage (a strong and flexible tissue). These rings are C-shaped and support the trachea but also allow it to move and flex when your child breathes.

Complete tracheal rings are a birth defect in these rings that causes them to be O-shaped instead of C-shaped. This can result in airway stenosis — an abnormal narrowing of your child’s windpipe. The number of rings in the trachea varies from 16 to 20. This defect in the cartilage can affect one or many of those rings.

What are the symptoms of complete tracheal rings?

Some children with complete tracheal rings may show mild respiratory issues at first, while others may be in serious respiratory distress. Symptoms of a narrowed airway (stenosis) caused by complete tracheal rings include:

  • noisy breathing (stridor)
  • wet-sounding breathing
  • recurring pneumonias
  • wheezing
  • cyanosis (blue spells)
  • apnea (pauses in breathing)
  • chest congestion

What causes complete tracheal rings?

Complete tracheal rings are a rare disorder that is present at birth. It is usually associated with other abnormalities of the heart or lungs. It may also be associated with Down syndrome and Pfeiffer syndrome.

Tracheal rings can also be associated with a condition in which the pulmonary artery (the artery carrying blood from the right ventricle of the heart to the lungs) wraps around the trachea and causes a narrowing of the opening.

How we care for complete tracheal rings

Although mild cases of complete tracheal rings may not require surgery, more severe cases can seriously interfere with your child’s breathing and require immediate treatment. The Center for Airway Disorders at Boston Children's Hospital is specifically designed to care for children with this rare condition. Our team provides the most advanced testing and treatment available.

Complete Tracheal Rings | Diagnosis & Treatments

How are complete tracheal rings diagnosed?

Your child’s doctor will need to look at images of the trachea in order to make a diagnosis of tracheal rings. The visualization test that is used is called bronchoscopy. During this procedure, your child will receive general anesthesia so that the trachea can be examined using a tube called a bronchoscope. The doctor will view the airway and determine whether or not your child has complete tracheal rings.

This is a very safe procedure, and your child will recover quickly. After the test is complete, our team of experts will meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best treatment options.

What are the treatment options for complete tracheal rings?

Complete tracheal rings can vary in severity. Milder forms can sometimes be monitored with close observation. Children with less severe symptoms may not require surgery.

However, in many cases, surgery will be required to treat the disorder. Our doctors are experts in surgical interventions, which include:

  • Segmental Tracheal Resection: During this procedure, surgeons remove the defective tracheal segment and then repair the airway by suturing (attaching) the remaining ends back together. This surgery is an option if your child has a few complete tracheal rings arranged in a short segment.
  • Slide tracheoplasty: Our surgeons are experts in this complex procedure to make the airway larger. During this surgery, the narrowed trachea is divided across the middle of stenosis (the area where the airway is narrowed). A portion of the lower and upper tracheal segments are cut and then attached, resulting in an airway that is wider and shorter than before. 

Complete Tracheal Rings | Programs & Services