What is the management of Pierre Robin sequence?

Management of Pierre Robin Sequence

The management of Pierre Robin sequence 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 most cases and avoids the risks associated with invasive procedures. 1, 2, 3

Clinical Features and Assessment

Pierre Robin sequence is characterized by:

• Micrognathia (small mandible)
• Glossoptosis (posterior displacement of the tongue)
• Cleft palate (in 87% of cases) 4
• Upper airway obstruction

Initial Evaluation

• Assess severity of airway obstruction through:
  • Clinical observation of respiratory distress
  • Oxygen saturation monitoring
  • Polysomnography (PSG) when available 5
• Evaluate feeding difficulties
• Screen for associated syndromes or chromosomal abnormalities (present in ~42% of cases) 5
• Assess maxillary-mandibular discrepancy

Management Algorithm

Step 1: Conservative Management (First-Line)

• Prone positioning: Position the infant prone (on stomach) to allow the tongue to fall forward 2, 3
• Nasopharyngeal airway (NPA): Insert to bypass the area of obstruction 2
• Continuous monitoring: Monitor oxygen saturation and respiratory status
• Nutritional support:
  • Specialized feeding techniques
  • Modified nutrition plates may help in some cases 6
  • Nasogastric feeding if necessary

Step 2: Evaluation of Response

• If conservative measures are effective:

  • Continue until the infant grows out of respiratory difficulties
  • Most infants show good weight gain with this approach 2

• If conservative measures fail (persistent hypoxemia, failure to thrive):

  • Perform polysomnography to quantify severity of obstructive sleep apnea 1, 5
  • Consider direct assessment of upper and lower airways to identify secondary airway pathology (present in ~68% of cases) 5

Step 3: Surgical Interventions (If Conservative Management Fails)

1. Distraction Osteogenesis (DOG):

   • Mandibular lengthening to relieve airway obstruction
   • Shown to prevent tracheostomy in 96% of cases and achieve decannulation in 92% 1
   • Timing is controversial - some recommend early intervention to avoid tracheostomy, others delay until 18-24 months 1

2. Alternative surgical options:

   • Tongue-lip adhesion
   • Floor of mouth release
   • Tracheostomy (typically reserved for cases with coexisting airway morbidity or failed surgical interventions) 4

Special Considerations

Anesthesia Management

• Difficult airway anticipation: Pierre Robin sequence is associated with difficult intubation 1
• Endoscopic intubation: May be accomplished with flexible bronchoscope even in preterm neonates 1
• Modified rapid sequence induction: If needed, use gentle bag-mask ventilation with FiO2 0.8 and low peak inspiratory pressure (<15 cmH2O) if SpO2 falls below 95% 1, 7
• Extubation: Should be performed when the child is fully awake, after at least 3 minutes of spontaneous ventilation with 100% O2, under full monitoring, and with difficult airway equipment immediately available 1

Long-term Follow-up

• Monitor for:
  • Residual sleep-disordered breathing
  • Mandibular growth
  • Speech development
  • Feeding progress
  • Need for orthodontic intervention (Class II malocclusion is common) 3

Prognosis

With appropriate management, most infants with Pierre Robin sequence show improvement over time as mandibular growth occurs naturally. Conservative treatment has demonstrated remarkable results in managing respiratory distress with decreased obstructive sleep events as the patient grows 3.