Management of Funnel-Type Tracheal Stenosis in a 4-Year-Old

Objectives: Congenital tracheal stenosis is a rare condition, which may lead to sudden infant death and in other cases, is only recognized on the basis of respiratory symptoms and failure to thrive. Associations with other congenital anomalies, in particular cardiac defects, are frequent.

Methods: We report a case of a 4-year-old boy, presenting with congenital tracheal stenosis of the funnel type (complete cartilage rings) and patent ductus arteriosus.

Result: A 4-year-old male patient with congenital funnel-type tracheal stenosis was referred to our institution for surgical correction. With a body weight of 12.1 kg (<1% percentile) and a height of 0.96 m (2% percentile), the child had a medical history of recurrent severe pulmonary infections with temporary need for intubation and psychomotor retardation. At that time, the boy was already treated with high flow oxygen therapy (22 L/min) at night and supportive nutrition via percutaneous endoscopic gastrostomy. Surgery was performed with extracorporeal circulation for 113 minutes at mild hypothermia (34°C) on beating heart. After clipping of patent ductus arteriosus, a tracheal stenosis with a length of 4 cm, an outer diameter of 4 mm, and a lumen of 2.5 mm above the carina was approached with slide tracheoplasty. Intraoperative bronchoscopy revealed a residual stenosis with a length of 2 cm directly below the larynx with an unremarkable exterior, but a lumen less than 3.5 mm. The decision was taken to perform a second slide tracheoplasty. At first, the postoperative course was uneventful. Early extubation attempts, however, failed and bronchoscopy revealed a short-segment narrowing of 4 mm by mucosal edema and fibrin clots distal to the tracheal tube. After repeated endotracheal application of an ointment containing 0.05% betamethasone, the patient was successfully extubated in the third attempt. Invasive infections were an aggravating factor in the postoperative course. After 31 days of intensive care, the patient was transferred close to home and could finally be discharged after 56 days without further major complications.

Conclusion: Double-slide tracheoplasty is a technically feasible approach for long segment funnel-type tracheal stenosis. Local endotracheal therapy with betamethasone is applicable in the event of stenosing mucosal edema and fibrin clots after slide tracheoplasty. To the best of the authors’ knowledge, both therapeutic concepts have not yet been published.