Tracheal agenesis and esophageal atresia with proximal and distal bronchoesophageal fistulas

Anesthetic management of congenital tracheoesophageal fistula

“…Although the provision of anesthesia for rigid bronchoscopy in a spontaneously ventilating neonate is challenging, this is an opportunity to characterize the airway. Firstly, these patients are at risk of concomitant airway anomalies, including tracheo‐bronchomalacia, tracheal upper pouch and tracheal stenosis/agenesis, which may alter the decision to proceed with muscle relaxation (1,20,21). Secondly, the position of the fistula affects management options: it may be difficult to intubate beyond a pericarinal fistula.…”

Tracheal agenesis, a frightening scenario

“…Survival was very rare, although some attempts at reconstruction were made. Reported cases of survived tracheal agenesis (in which reconstruction attempts led to survival, even if just for a short time) are summarised in Table I 1 , 4 – 11 Table IReported cases of survived tracheal agenesis* and their management…”

“…A diagnosis of tracheal agenesis should be considered in cases of respiratory distress with immediate hypoxia in a baby with no audible cry and where there is a mechanical inability to intubate 1 . If tracheal agenesis is suspected postnatally, then a trial of intubation of the oesophagus is recommended.…”

“…Tracheal agenesis incidence is reported to be less than 1 in 50 000 births 1 . Most cases represent part of either the ‘VATER’ association (vertebral defects, anal atresia, tracheoesophageal fistula with oesophageal atresia, and radial or renal dysplasia), the ‘VACTERL’ association (vertebral defects, anal atresia, tracheoesophageal fistula with oesophageal atresia, and radial or renal dysplasia, plus cardiovascular and limb defects) or the ‘TARCD’ association (tracheal agenesis or atresia, radial ray defects, complex congenital cardiac abnormalities, and duodenal atresia) 2 .…”

Prenatal diagnosis of tracheal atresia in a twin pregnancy

“…The level of airway obstruction in tracheal atresia varies, and distal atresia with the upper half of the trachea and larynx being normal has been described. Tracheal atresia is usually associated with tracheoesophageal or bronchoesophageal fistula, but isolated forms have also been reported.…”