Pierre Robin sequence and double aortic arch: a case report

We present an 8-day-old female with two admissions for respiratory failure. On the first admission, the diagnosis of Pierre Robin sequence (PRS) and laryngomalacia was made after assessment with chest radiography, echocardiography, and flexible fiberoptic laryngoscopy. Four days after discharge, the child presented with stridor and respiratory distress, and a new cardiac murmur was noted after admission. Repeat echocardiography, with confirmatory direct laryngobronchoscopy, revealed a double aortic arch (DAA) with distal tracheal compression. This case illustrates the necessity of a complete otolaryngic evaluation, including direct laryngobronchoscopy, to search for a synchronous airway lesion in any neonate with severe respiratory distress associated with stridor.