Management of Pierre Robin Sequence
The management of Pierre Robin sequence (PRS) should follow a stepwise approach, starting with conservative measures like prone positioning and nasopharyngeal airway placement before considering surgical interventions, as non-surgical management is effective in approximately 70% of cases. 1, 2
Assessment and Evaluation
- Assess severity of airway obstruction through:
- Clinical observation of respiratory distress
- Continuous oxygen saturation monitoring
- Polysomnography (PSG) when available 1
- Evaluate feeding difficulties
- Screen for associated syndromes or chromosomal abnormalities
- Assess maxillary-mandibular discrepancy
Management Algorithm
Step 1: Conservative Management (First-line)
- Prone positioning to allow the tongue to fall forward
- Continuous monitoring of oxygen saturation and respiratory status
- Nutritional support with specialized feeding techniques and modified nutrition plates 1
Step 2: Minimally Invasive Interventions (If Step 1 fails)
- Nasopharyngeal airway (NPA) insertion to bypass the area of obstruction 1, 3
- Effectively relieves airway obstruction until babies grow out of respiratory difficulties
- Requires monitoring but avoids need for surgery 3
Step 3: Surgical Management (If Steps 1-2 fail)
- Distraction osteogenesis (DOG)
- Can prevent tracheostomy in 96% of cases
- Can achieve decannulation in 92% of cases 1
- Tongue-lip adhesion (for temporary management)
- Tracheostomy (reserved for approximately 10% of cases with severe obstruction) 2
Anesthesia and Airway Management
- Anticipate difficult airway
- Use endoscopic intubation with flexible bronchoscope even in preterm neonates
- Employ modified rapid sequence induction with:
- Perform extubation when the child is fully awake, after at least 3 minutes of spontaneous ventilation with 100% O2 1
- Have difficult airway equipment immediately available during extubation 1
Post-Management Monitoring
- Monitor for residual sleep-disordered breathing
- Track mandibular growth
- Assess speech development
- Monitor feeding progress
- Evaluate need for orthodontic intervention 1
Clinical Pearls and Pitfalls
- Important distinction: Differentiate between isolated PRS and syndromic PRS, as management approach may differ 5
- Catch-up growth: Likely occurs in deformational (isolated) PRS but not in syndromic cases 2
- Treatment individualization: Consider that patients with potential catch-up growth may only need temporary measures like NPA, while those without catch-up growth potential may benefit from early distraction osteogenesis 2
- Avoid unnecessary tracheostomy: While necessary in severe cases, tracheostomy should be avoided when possible due to associated complications 2
- Grading system: Consider using a grading system based on symptom severity to clarify care pathways and enhance communication between healthcare professionals 6
The evidence strongly supports a conservative approach first, with surgical interventions reserved for cases that fail to respond to less invasive measures. With appropriate management, most infants with Pierre Robin sequence show improvement over time as natural mandibular growth occurs.