Complete tracheal rings are a birth defect in the cartilage rings that keep the trachea, or windpipe, from collapsing. A normal tracheal cartilage is C-shaped with a softer, posterior membrane consisting of muscle. In complete tracheal rings, the trachea is made with several or more O-shaped rings.
This condition is thought by some to occur from abnormal growth of the cartilage as compared to the muscular part of the normal trachea. The affected airway may involve just a few rings to most of the trachea and can extend into the left or right lung through the bronchi.
Children with complete tracheal rings may have associated malformations, cardiac and pulmonary anomalies, Down syndrome, and Pfeiffer syndrome. It can be associated with a vascular anomaly known as a “ring-sling” complex in which the pulmonary artery due to an abnormal course can sling around the trachea and cause compression and narrowing of the tracheal opening.
Endoscopic view of complete tracheal rings
Doctors usually diagnose complete tracheal rings in newborns and infants. It is diagnosed less often later in childhood, with symptoms developing at a few months of age. Some children may present in acute respiratory distress. Affected children may experience rapidly worsening symptoms in the face of an upper respiratory infection. Symptoms include:
- Noisy breathing (stridor)
- Recurring pneumonias
- Wet-sounding biphasic noise (like a washing machine)
- Cyanosis (blue-spells)
- Apnea (breathing pauses)
- Chest congestion
X-rays and more advanced imaging such as CT or MR imaging will help your doctor diagnose complete tracheal rings. Imaging can provide a more complete picture of not only about the extent of airway narrowing, but also any other lesions that might be present such as cardiac or vascular problems.
Microlaryngoscopy and bronchoscopy is essential to fully diagnose complete tracheal rings. This diagnostic test provides clarification of the tracheal lesion and differentiates it from lesions in the larynx that can cause stridor and respiratory distress. Furthermore, the test can help show the degree of narrowing and length of narrowing in the trachea.
In addition to an airway evaluation, your child may also require evaluations by pulmonologists and gastroenterologists. Illnesses in the lungs or esophagus/stomach can affect the overall health of the patient and surgical outcomes. Advanced evaluation may include endoscopic examinations by these specialty services at the same time as a microlaryngoscopy and bronchoscopy.
Your child's treatment of complete tracheal rings depends on the extent of the narrowing and caliber of the airway.
Milder forms of complete tracheal rings can sometimes be monitored with close observation at your regular doctor’s visits. Larger caliber rings in children with less severe symptoms may grow and require no surgical intervention.
- Tracheal resection and primary anastomosis. If your child has a few complete tracheal rings arranged in a short segment, these surgical treatments may be an option. Tracheal resection involves surgically removing the defective tracheal segment and then repairing the airway by suturing the remaining ends back together.
- Slide tracheoplasty. Usually, your doctor cannot repair longer segment tracheal rings with a tracheal resection. Instead, the doctor may perform slide tracheoplasty surgery. In slide tracheoplasty, the narrow part of the trachea is cut through horizontally. Then a vertical incision is made in the back part of one segment and the front part of the other tracheal segment. Finally, the two sections are slid together and sutured so that they overlap, providing a wider tracheal airway.