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Tracheoplasty for congenital long-segment intrathoracic tracheal stenosis
C H Van Meter1, R M Lusk, H Muntz
1Children's Hospital, Washington University Medical Center, St. Louis, Missouri.
Insights
Congenital long-segment intrathoracic tracheal stenosis (CTS) requires prompt surgical repair. Rigid repair using rib cartilage is preferred over pericardium for better long-term outcomes in infants.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Congenital Malformations
Background:
- Congenital long-segment intrathoracic tracheal stenosis (CTS) is a rare, life-threatening condition in infants and children.
- Symptoms vary from stridor and wheezing to severe respiratory compromise.
- Diagnosis often requires bronchoscopy due to limitations of routine imaging.
Purpose of the Study:
- To evaluate surgical repair outcomes for congenital long-segment intrathoracic tracheal stenosis.
- To compare the efficacy of different graft materials (pericardium vs. rib cartilage).
- To identify critical factors for successful surgical intervention.
Main Methods:
- Retrospective review of six infants diagnosed with CTS between 1983 and 1988.
- Surgical repair techniques included anterior tracheoplasty using pericardium or rib cartilage grafts.
- Intraoperative bronchoscopy and cardiopulmonary bypass were utilized in some cases.
Main Results:
- Complete tracheal rings were observed in all patients, causing severe tracheobronchial lumen compromise.
- Three patients repaired with pericardium had one early death and two late deaths unrelated to repair.
- Three patients repaired with rib grafts, including one with prior tetralogy of Fallot repair, survived with no postoperative symptoms.
Conclusions:
- Prompt surgical intervention for CTS is crucial to prevent complications associated with long-term ventilation.
- Rigid repair with rib cartilage demonstrates superior outcomes compared to pericardium.
- Successful outcomes depend on meticulous surgical technique, including proper graft harvesting and alignment.
Abstract:
Congenital long-segment intrathoracic tracheal stenosis (CTS) is a rare life-threatening obstruction in infancy and childhood. From July 1983 to March 1988 six infants aged 14 days to 14 months with CTS were identified. Symptoms ranged from recurrent stridor and wheezing to severe respiratory compromise and hypercarbia. Routine chest x-rays were not diagnostic. Definitive diagnosis was made by bronchoscopy, which showed complete tracheal rings in all patients with severely compromised tracheobronchial lumens. In three patients, pericardium was successfully used for anterior tracheoplasty with one early death due to fulminant sepsis in an infant with undiagnosed sickle cell disease. The other two died late, at 3 and 9 months from problems unrelated to the repair. In three patients a rib graft was used for repair; in one, tracheoplasty was required after earlier repair of tetralogy of Fallot. All are late survivors with no postoperative symptoms. After recognition of CTS, prompt surgery is warranted to avoid the late complications of tracheostomy for long-term ventilatory support. Rigid repair with rib cartilage is preferable to use of pericardium. Proper rib harvesting with intact perichondrium, intraoperative bronchoscopy, oxygenation by cardiopulmonary bypass, and meticulous graft alignment are necessary for successful postoperative outcome.