Tracheal agenesis type B: further evidence to a lethal congenital tracheal malformation

Tracheal agenesis and esophageal atresia with proximal and distal bronchoesophageal fistulas

“…Fewer than 100 cases have been reported in the literature until today. The incidence of it was reported to be less than 1:50,000 with a male predominance [1,4]. It presents immediately at birth with cyanosis and respiratory distress.…”

“…Numerous attempts at neonatal resuscitation with endotracheal intubation meet with failure, although the larynx and vocal cords are well visualized. The classification system of Faro [1,5] seems to have the most practical application from a surgical standpoint. Faro et al [1,5] divided tracheal atresia into categories A to G, representing various types of airway anomalies decreasing in severity, with category A representing total pulmonary atresia and category G representing tracheal stenosis.…”

“…Typically, TA is fatal in the neonatal period, and the diagnosis is not made until a postmortem examination. Very few reports have demonstrated long-term survival with surgically reconstructed TA [1,8]. If surgical treatment is planned, then initial stabilization of these infants requires esophageal banding to prevent regurgitation of gastric fluid and abdominal distension owing to positive pressure mechanical ventilation [9].…”

“…Definitive surgical reconstruction for patients with a distal tracheal remnant continues to be challenging. Esophageal diversion followed by reconstruction with colonic interposition or gastric pull-up has already been demonstrated to be feasible [1,12]. However, at present, there is no suitable autologous tissue to assume the function of the normal trachea with its rigid cartilaginous framework.…”

“…An early diagnosis may also help the parents and the medical team to take appropriate decisions. Currently, there is no effective therapy that guarantees long-term survival [1].…”