Congenital absence of cartilaginous tracheal rings associated with esophageal atresia and trifurcated carina: a novel anomaly?

Tracheomalacia associated with esophageal atresia (EA) is a well-known condition. However, complete absence of tracheal rings (TRs) is extremely rare. Our aim is to describe a novel triad of conditions and to discuss the best treatment. An expremature male operated for EA presented with severe respiratory distress. The diagnosis of absent cartilage rings, suspected on bronchoscopy, was confirmed by optical coherence tomography. The absence of TRs was localized to a short tracheal segment, and the carina trifurcated into right upper lobe, right intermediate, and left main bronchus. The patient was treated with resection and anastomosis with a completely satisfactory course. Absence of TRs was previously reported by us in 2 other cases, both with associated EA and trifurcation of the carina. One child was treated with tracheostomy and the other with a stent, but the outcome was far from optimal. The patient with tracheostomy eventually underwent resection and anastomosis with tracheostomy closure. Congenital absence of TRs is extremely rare. Although localized, it is responsible for severe symptoms owing to complete tracheal collapse and may be misdiagnosed as tracheomalacia. In our experience, it has been associated with EA and trifurcated carina. Our limited experience suggests resection of the abnormal segment and tracheal anastomosis as the best treatment.