a male infant with a birth weight of 1,592 g was born by cesarean section at 31 weeks of gestation with a prenatal diagnosis of suspicious esophageal atresia . since the baby was hypotonic and did not cry , he was resuscitated by intubation , which was later determined to be accidental esophageal intubation and not endotracheal intubation . after intubation , the patient s respiratory distress temporarily improved ; however , he suffered repeated cycles of stabilization and deterioration . the g - tube was located in the left lung field on the anteroposterior view of the chest x - ray , not in the abdomen , and the e - tube deviated to the posterior side on the lateral view . 1a ) , and computed tomography scanning confirmed a diagnosis of floyd s type ii tracheal agenesis ( fig . furthermore , duodenal atresia ; hemi - vertebrae ; cardiac anomalies , including an atrial septal defect and a patent ductus arteriosus ; and a thickened filum terminale were observed . in order to stabilize the infant s airway , distal esophageal ligation and division with gastrostomy duodenoduodenostomy and diverticulum excision were performed concomitantly in order to correct duodenal atresia and meckel s diverticulum . a second operation involving separated cervical esophagostomies was performed 80 days after birth in order to perform a pseudotracheostomy . a proximal cervical esophagostomy was performed in the right side of the neck for salivary drainage and a distal cervical esophagostomy formed the airway . despite surgery , the patient suffered from repeated respiratory distress due to airway collapse and a narrow esophagobronchial fistula orifice . at 101 days of age , the stenotic esophagobronchial fistula orifice was widened using a pericardial patch under cardiopulmonary bypass support through a median sternotomy . a gore - tex external supportive stent ( wl gore & associates , flagstaff , az , usa ) was placed around the proximal esophagus using radial traction sutures to prevent the esophagus from collapsing ( figs . 2 , 3 ) . twenty - five days after the operation , he was transferred to a general ward without oxygen administration ( fig . the patient was readmitted to the intensive care unit at 155 days of age due to repeated desaturation events . we performed venovenous extracorporeal membrane oxygenation through the internal jugular vein due to severe hypoxia at 181 days of age . repeat surgery for airway reconstruction involving gore viabahn endoprosthesis stent ( wl gore & associates ) insertion and fixation was performed at 186 days of age . dehiscence of the esophageal traction sutures and the esophageal suture line around the site where the esophagobronchial fistula orifice had been widened was observed . despite supportive treatment , the patient s condition deteriorated due to recurrent respiratory infection , hemoptysis , and esophagobronchial fistula orifice restenosis , and he died of sepsis at 354 days of age . tracheal agenesis is an extremely rare congenital disorder ; approximately 150 cases have been reported since it was first described by payne in 1900 . reported the incidence of tracheal agenesis to be one case per 50,000 live births . the tracheal agenesis classification proposed by floyd et al .   has been universally accepted . it classifies tracheal agenesis into three types : type i involves a short segment of distal trachea arising from the esophagus ; type ii involves no trachea , but a carina is present with a fistula to the esophagus ; and in type iii , the two mainstem bronchi arise individually from the esophagus . the relative incidence of these three types is 13% , 65% , and 22% , respectively . fetal magnetic resonance imaging could be a useful modality for antenatal diagnosis , but it is not widely used . clinicians might overlook or completely misdiagnose this condition without a high level of clinical suspicion . tracheal agenesis should be suspected in cases of antenatal polyhydroamnios , preterm birth , and low birth weight . the most common sign is a lack of an audible cry with vigorous respiratory effort . esophageal intubation can temporarily improve respiratory distress only if a tracheoesophageal or bronchoesophageal fistula is present . chest x - ray findings include the absence of a tracheal shadow and the posterior location of the endotracheal tube . chest computed tomography is widely used to confirm the diagnosis of tracheal agenesis . in cases of tracheal agenesis , it is necessary to stabilize the infant s airway immediately . in floyd s type i agenesis , an endotracheal tube may be placed in the distal tracheal stump under direct visualization using a bronchoscope . in floyd s type ii and iii agenesis , esophageal intubation can maintain the infant s airway temporarily if a relatively large endotracheal tube is used at the level of the bronchoesophageal fistula . since the escape of gases into the stomach can lead to abdominal distension and consequent respiratory difficulty , surgery , including distal esophageal banding , we conducted distal esophageal ligation and division with gastrostomy to stabilize the airway of the infant in our case . after palliative surgery , surgical intervention for secure pseudotracheostomy and reconstruction of the alimentary tract should be scheduled . for the airway , only two patients with floyd s type i tracheal agenesis have ever remained alive without ventilator support . in these cases , external supportive stents with radial traction sutures around the esophagus were used to prevent the esophagus from collapsing . we used pericardium to widen the fistula orifice , but it did not demonstrate sufficient durability . reconstruction with a gore viabahn endoprosthesis stent was also unsuccessful . as the gore viabahn endoprosthesis stent does not have mucosa , it can not provide an appropriate airway , which leads to difficulties in clearing respiratory secretions and protecting the lungs from external stimuli . this was one of the reasons that the infant suffered repeated respiratory infections and hemoptysis , causing death . experimental tracheal reconstruction using a variety of materials , including silicone elastomer , dacron , marlex mesh , and bladder wall , have been attempted unsuccessfully in previous cases . recently , animal experiments using allographs and homographs have given hope that tracheal transplantation in humans may one day be clinically possible . however , long - term survival following surgical transplantation of the trachea has not been reported , even in an animal model . despite advancements in surgical techniques , tracheal reconstruction in floyd s type ii and iii tracheal agenesis is still regarded as an unsolved issue . however , the infant in the present case survived for almost a year and breathed without a ventilator for approximately 50 days . this case report contributes to a growing collection of data in the literature on tracheal agenesis and airway reconstruction using external supportive stents .
tracheal agenesis is an extremely rare and typically lethal congenital disorder . 
 approximately 150 cases have been described since 1900 , and very few cases of survival have been reported . 
 we describe tracheal reconstruction with external esophageal stenting in a patient with floyd s type ii tracheal agenesis . 
 neither long - term survival nor survival without mechanical ventilation for even a single day has previously been reported in patients with floyd s type ii tracheal agenesis . 
 the infant in the present case survived for almost a year and breathed without a ventilator for approximately 50 days after airway reconstruction using external supportive stents .