Congenital laryngomucocoele: a rare cause for CHAOS

Area of Science:

• Neonatology
• Pediatric Surgery
• Medical Genetics

Background:

• Congenital high airway obstruction syndrome (CHAOS) is a rare and severe condition impacting newborns.
• Upper airway obstruction in neonates presents significant management challenges and carries a high mortality rate.
• Early diagnosis and intervention are critical for improving outcomes in neonates with airway obstruction.

Purpose of the Study:

• To report a rare case of congenital high airway obstruction syndrome (CHAOS) caused by a laryngomucocoele.
• To highlight the importance of antenatal diagnosis for timely intervention in cases of neonatal airway obstruction.
• To emphasize the potential of ex utero intrapartum treatment (EXIT) procedures for managing severe CHAOS.

Main Methods:

• Case report of a female newborn presenting with respiratory distress at birth.
• Detailed antenatal ultrasound findings including polyhydramnios.
• Post-mortem examination to identify the cause of laryngeal obstruction.

Main Results:

• The newborn exhibited severe respiratory distress, with unsuccessful intubation attempts due to laryngeal obstruction.
• Post-mortem examination revealed a total occlusion of the larynx by a laryngomucocoele.
• This case represents the first reported instance of a laryngomucocoele causing CHAOS.

Conclusions:

• Laryngomucocoele is a rare congenital laryngeal cyst that can cause fatal congenital high airway obstruction syndrome (CHAOS).
• Antenatal diagnosis of CHAOS is crucial for planning ex utero intrapartum treatment (EXIT) procedures, such as emergency tracheostomy.
• Prompt recognition and management strategies are vital for neonates with severe airway obstruction.