Surgical management of tracheal stenosis in an infant with multiple congenital anomalies: when is baby inoperable?

Congenital tracheal stenosis is a rare disorder that carries a significant morbidity and mortality. It is an even greater challenge when present in combination with other life-threatening congenital anomalies. We present an infant with multiple congenital anomalies, including trisomy 21, who was diagnosed with congenital tracheal stenosis at 3 months of age. At age 5 months, the infant underwent tracheoplasty with costal cartilage graft and pulmonary artery banding. Bronchoscopy 3 weeks later revealed a patent airway. However, the patient died 2 months postoperatively of cardiac complications. At autopsy, whole organ and histologic specimens revealed excellent incorporation of the graft with no evidence of re-stenosis. Our documentation of graft incorporation in this patient is evidence that long segments of stenotic trachea can be successfully treated by tracheoplasty utilizing rib cartilage grafts.