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Pierre Robin sequence and double aortic arch: a case report.

Edward T Waters¹, James P Oberman, Abhik K Biswas

¹Department of Pediatrics, Naval Hospital Beaufort, One Pinckney Blvd., Beaufort, SC 29902, USA. taguas@pol.net

International Journal of Pediatric Otorhinolaryngology

|January 4, 2005

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A neonate with respiratory failure initially diagnosed with Pierre Robin sequence (PRS) and laryngomalacia was later found to have a double aortic arch (DAA). This highlights the importance of thorough airway evaluation in infants with severe stridor.

Area of Science:

Pediatric Otolaryngology
Neonatal Respiratory Medicine
Congenital Cardiovascular Surgery

Background:

Severe respiratory distress in neonates can stem from various congenital anomalies.
Pierre Robin sequence (PRS) and laryngomalacia are common causes of upper airway obstruction in infants.
Early diagnosis and management are crucial for improving outcomes in neonates with respiratory failure.

Observation:

An 8-day-old female presented with recurrent respiratory failure.
Initial diagnosis included PRS and laryngomalacia, but symptoms persisted.
A new cardiac murmur prompted further investigation, revealing a double aortic arch (DAA) causing tracheal compression.

Findings:

Direct laryngobronchoscopy confirmed a double aortic arch (DAA) as the cause of distal tracheal compression.

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The DAA was synchronous with the initial diagnosis of PRS and laryngomalacia.

This case underscores the potential for co-existing airway lesions in neonates.

Implications:

A comprehensive otolaryngic evaluation, including direct laryngobronchoscopy, is essential for neonates with severe respiratory distress and stridor.
Failure to identify synchronous airway lesions like DAA can lead to delayed or incorrect diagnosis and treatment.
This case emphasizes the need for a multidisciplinary approach in managing complex neonatal respiratory conditions.