Objective: a) To report on an adult patient with congenital long-segment tracheal stenosis from complete tracheal rings complicated by tracheomalacia; b) to highlight the fact that some patients with airway narrowing could be misdiagnosed as having bronchial asthma; and c) to discuss our management with a custom-made tracheostomy tube extending to the carina.
Design: Case report.
Setting: A university hospital's 14-bed medical/surgical intensive care unit.
Patient: A 21-yr-old patient, with a history of what was labeled as asthma, was admitted to the intensive care unit with diabetic ketoacidosis, pneumonia, respiratory failure, and septic shock.
Interventions: Her therapy included assisted mechanical ventilation through an endotracheal tube. Initially, a size 6.0 endotracheal tube was used. Finally, a custom-made tracheostomy tube extending to the carina was inserted to manage her persistent infantile trachea.
Measurement and Main Results: During 4 months in the intensive care unit, she suffered numerous airway problems from her narrow trachea that were eventually attributed to congenital long-segment tracheal stenosis from complete tracheal rings. Bacterial pneumonia, viral tracheobronchitis, and tracheomalacia complicated her course. Multiple attempts at extubation failed and, after translaryngeal endotracheal tubes and tracheostomy tubes of decreasing size, her airway was managed with a size 5.0 custom-made tracheostomy tube with the tip extending to her carina. She was totally dependent on this tube.
Conclusion: Airway narrowing may masquerade as asthma. Congenital tracheal stenosis is rare and is associated with a high mortality rate. Complete tracheal rings presenting in adulthood are extremely rare, and we report the first case of long-segment pantracheal stenosis presenting in adulthood. Surgical treatment with tracheoplasty is difficult. A custom-made tracheostomy tube to stent the entire trachea is one management option. Tracheal stenosis should be excluded in patients with a chronic lack of response to therapy for asthma.