Early MDO with a Virtually Planned Distractor in a Neonate with Pierre Robin Sequence

Area of Science:

• Craniofacial Surgery
• Pediatric Surgery
• Genetics

Background:

• Pierre Robin sequence (PRS) presents with micrognathia, glossoptosis, and airway obstruction.
• This case involves a female infant with PRS, 4q deletion, and cardiac insufficiency.

Observation:

• The infant experienced severe respiratory insufficiency, initially managed with CPAP, then endotracheal intubation after cardiac surgery.
• A Tuebingen plate was not tolerated, and CPAP became insufficient, necessitating intervention.

Findings:

• Early mandibular distraction with patient-specific internal distractors was performed at five weeks of life.
• Virtual surgery planning enabled precise vector planning and custom distractor fabrication.
• Mandibular advancement of 15 mm was achieved in two weeks, allowing successful extubation and resolution of respiratory failure.

Implications:

• Early mandibular distraction is a successful treatment for severe respiratory insufficiency in PRS.
• This technique can prevent the need for tracheostomy and its associated complications.
• Patient-specific distractors offer reduced surgical time and morbidity, protecting vital structures.