A 26-day-old boy with no relevant family, prenatal or perinatal history. At around three weeks of age, he began to present vomiting, which increased, and the day before his admission he began to vomit with each feed, abundantly and in projectile form. The patient was eager to eat and was in good general condition.
On presentation to the Emergency Department, a clinical suspicion of hypertrophic pyloric stenosis was raised, supported by a blood gas analysis showing metabolic alkalosis and an ultrasound with a characteristic target image. The patient underwent open surgery using the Ramstedt technique without incident. Prior to the procedure the patient was intubated without complications.
At 14 hours after surgery, inspiratory stridor was heard for the first time, which did not change with sleep, position or feeding. The mother was re-interviewed and denied having heard it before. The patient had no evidence of respiratory distress or oxygen desaturation on pulse oximetry.
As this was a previously healthy patient who had not heard stridor before, it was suspected that the operative intubation and associated post-traumatic oedema might be the cause. Adrenaline nebulisation and a dose of intravenous dexamethasone were administered. After two hours, the stridor had not changed, and as the patient continued without signs of respiratory distress, this problem was started to be addressed.
A plain chest X-ray showed no abnormalities in the lung parenchyma, neck structures or tracheal air column. Subsequently, a flexible nasolaryngoendoscopy showed the supraglottic structures and the vocal cords to be intact, without morphological alterations or abnormal movements. The next step was a fluoroscopic series with oral contrast, which showed a notch in the posterior wall of the upper third of the oesophagus. During this time the patient showed no change in his symptoms. After the pylorus surgery, he had not presented vomiting again. At no time did he present signs of respiratory distress associated with stridor.
A few days later, a bronchoscopy was performed, in which an extrinsic compression 2 cm above the carina was observed, pulsatile, obstructing more than 50% of the tracheal lumen and unchanged by positive pressure. Given the strong suspicion of a vascular malformation responsible for the decrease in tracheal calibre, computed tomography angiography (CT angiography) was performed, which identified a complete double aortic arch, with predominance of the right arch as the cause of the tracheal constriction. Echocardiography was normal.

The patient underwent surgery via a left lateral thoracic approach, which was performed without mishap.

In the first hours after surgery, a significant decrease in the intensity of inspiratory noise was noted. He was discharged and during the following weeks the residual stridor continued to decrease. He had no further respiratory or digestive symptoms, and at a follow-up consultation two months after surgery the stridor eventually disappeared completely.
