Mandibular Distraction in Robin Sequence With Multi-Level Airway Disease: Always Contraindicated?

Area of Science:

• Pediatric Surgery
• Otolaryngology
• Genetics

Background:

• Pierre Robin sequence (PRS) is characterized by micrognathia, glossoptosis, and airway obstruction.
• Concurrent airway anomalies, such as complete tracheal rings, complicate PRS management and often preclude mandibular distraction alone.
• Tracheostomy is frequently required for severe airway compromise in PRS with associated anomalies.

Observation:

• This report details the second case of PRS with concomitant complete tracheal rings.
• The patient presented with severe respiratory compromise at birth, unresponsive to initial interventions.
• A multi-level airway disease necessitated a staged surgical approach.

Findings:

• A two-stage surgical intervention, including sliding tracheoplasty followed by mandibular distraction osteogenesis, was successfully performed.
• This approach directly addressed both the tracheal anomaly and the micrognathia.
• The patient was successfully weaned to room air, avoiding tracheostomy.

Implications:

• Careful evaluation for concomitant airway anomalies is critical in PRS patients with respiratory distress.
• Even with multi-level airway disease, a staged surgical plan can secure the airway and prevent tracheostomy.
• Multidisciplinary collaboration is essential for optimal management of complex PRS cases.