Complete Tracheal Rings


Children's Memorial Hospital, Chicago, IL

Spring 1998


Complete tracheal rings occur with congenital absence of the posterior membranous trachea (Figure 8). This is associated with tracheal stenosis that nearly always leads to respiratory distress in infancy. The medical management of this lesion is associated with a 50% mortality rate. Many patients are referred when even the smallest endo-tracheal tube cannot be passed very far below the vocal cords because of the stenosis. The diagnosis is confirmed by rigid bronchoscopy. In many patients the bronchoscope itself cannot be passed, only a fine telescope may be possible. In patients diagnosed with complete tracheal rings, pulmonary artery sling is present in 30% and intracardiac defects in 15%, and these should be ruled out with echocardiography.

A total of 38 patients have now undergone repair of complete tracheal rings at Children's Memorial. Twenty-eight have had pericardial tracheoplasty, 2 have had resection with end-to-end anastomosis, 2 have had slide tracheoplasty,10 and 6 have had a new technique developed at Children'sa free tracheal autograft.11

FIGURE 8. Complete tracheal rings

FIGURE 9. Pericardial patch tracheoplasty.

The pericardial patch tracheoplasty is performed through a median sternotomy with the use of cardiopulmonary bypass for respiratory support. The trachea is opened anteriorly the entire extent of the stenosis and then patched open with autologous pericardium as shown in Figure 9. The patch is stented with an endotracheal tube for 1014 days at which time the child is extubated. Bronchoscopy is performed before extubation to remove secretions and granulation tissue and perform dilation if necessary.12 The pericardial patch technique was the first operation successful in the treatment of children with complete tracheal rings. However, a number of patients had prolonged hospitalizations secondary to granulation tissue and scar tissue, and 6 of 28 patients required reoperation and surgical revision.13 Four patients required placement of balloon expandable metallic stents for collapsing tracheal or bronchial segments.

FIGURE 10. The trachea has been opened anteriorly and the autograft resected.

In an effort to improve the outcome of these patients, we developed the tracheal autograft technique.11 Using a median sternotomy and cardiopulmonary bypass, the trachea is incised anteriorly throughout the length of the stenosis. Then approximately 6 to 8 tracheal rings or 1520 mm of trachea are harvested from the mid-portion of the trachea (Figure 10). The trachea is reanastomosed posteriorly and the autograft is used as an anterior patch (Figure 11). In children with a shorter segment of tracheal stenosis, the autograft completes the patch. In patients with a longer stenosis, the autograft has been augmented superiorly with pericardium. This technique has now been successful in 6 consecutive infants.

Results and Conclusions

There has been no operative mortality from an isolated vascular ring or pulmonary artery sling in the Division of Cardiovascular-Thoracic Surgery at Children's Memorial since 1959. The survival rate of infants with complete tracheal rings in our series is 84% with 100% survival using our new tracheal autograft technique. Ninety-two percent of the infants who undergo vascular ring repair are free of respiratory symptoms one year post-operatively.

Vascular rings are rare congenital anomalies that cause compression of the trachea and esophagus. Infants present with stridor, barky cough, respiratory distress, cyanosis, and apnea. Diagnosis is best established by barium esophagram for double aortic arch and right aortic arch with left ligamentum. Bronchoscopy is used to diagnose innominate artery compression syndrome and complete tracheal rings. Echocardiogram is the diagnostic procedure of choice for pulmonary artery sling.

The surgical approaches to vascular rings include a left thoracotomy for double aortic arch and right aortic arch with ligamentum; right thoracotomy for innominate artery suspension; and median sternotomy with cardiopulmonary bypass for pulmonary artery sling and complete tracheal rings. Close cooperation between the divisions of Cardiovascular-Thoracic Surgery and Otolaryngology have enabled the development of a program with the largest published single institutional experience with vascular rings in North America.

Copyright  2000 by Childrens Memorial Hospital.