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Updated: Jun 20, 2025
Author Spotlight: Advancing Awake Nasotracheal Intubation with Flexible Video Rhino-Laryngoscopes
Published on: August 2, 2024
Airway management in infants with Robin sequence in the United Kingdom and Ireland: A prospective population-based
Marie Wright1,2,3, Rachel L Knowles3, Mario Cortina-Borja3
1Division of Paediatric Respiratory Medicine, BC Children's Hospital, Vancouver, Canada.
Insights
Most infants with Robin sequence (RS) in the UK/Ireland are managed with nasopharyngeal airways (NPA) for upper airway obstruction (UAO). NPA is a safe first-line treatment, but longitudinal monitoring of growth and neurodevelopment is crucial.
Area of Science:
- Pediatric Otolaryngology
- Craniofacial Anomalies
- Neonatal Intensive Care
Background:
- Robin sequence (RS) presents challenges in infant upper airway obstruction (UAO) management.
- Current treatment efficacy and clinical outcomes lack consensus.
- Understanding UAO management in RS is critical for improving infant care.
Purpose of the Study:
- To describe UAO management strategies for infants with RS in the UK and Ireland.
- To explore the relationship between patient characteristics, UAO management, and clinical outcomes.
- To evaluate the safety and feasibility of different UAO interventions.
Main Methods:
- Active surveillance of RS cases across the UK and Ireland.
- Clinical data collection at initial notification and 12-month follow-up.
- Analysis of patient characteristics, interventions, and clinical outcomes.
Main Results:
- 173 infants with RS were identified; 47% had non-isolated RS.
- Two-thirds required airway intervention beyond prone positioning; 84% were non-surgical (primarily nasopharyngeal airway - NPA).
- Surgical management was linked to longer hospital stays, neurodevelopmental delay, and feeding issues; NPA showed safe and feasible outcomes.
Conclusions:
- Nasopharyngeal airway (NPA) is the predominant and a safe first-line treatment for UAO in infants with RS in the UK/Ireland.
- Tracheostomy is reserved for severe, refractory UAO.
- Longitudinal assessment of growth and neurodevelopment is essential for all RS infants, including isolated cases, highlighting the need for evidence-based guidelines.
Objective:
There is currently no consensus about managing upper airway obstruction (UAO) in infants with Robin sequence (RS), in terms of treatment efficacy or clinical outcomes. This study describes UAO management in UK/Ireland, and explores relationships between patient characteristics, UAO management, and clinical outcomes in the first 2 years of life.
Methods:
Active surveillance of RS throughout UK/Ireland via the British Paediatric Surveillance Unit and nationally commissioned cleft services. Clinical data were collected at initial notification and 12-month follow-up.
Results:
173 infants with RS were identified, of which 47% had additional congenital anomalies or an underlying syndrome (non-isolated RS). Two-thirds (n = 119) required an airway intervention other than prone positioning: non-surgical in 84% and surgical (tracheostomy) in 16%. Nasopharyngeal airway (NPA) was the most common intervention, used in 83% (n = 99) for median 90 days (IQR 136). Surgical UAO management was associated with prolonged hospital admission, higher prevalence of neurodevelopmental delay (NDD), lower weight-for-age z-scores, and delayed oral feeding. These findings were not attributable to a higher prevalence of non-isolated RS in this group. Although more commonly associated with non-isolated RS, growth faltering was also identified in 48%, and NDD in 18%, of cases of isolated RS.
Conclusions:
In UK/Ireland, most infants with RS are managed with NPA, and tracheostomy is reserved for refractory severe UAO. Clinical outcomes and duration of use indicate that NPA is a safe and feasible first-line approach to UAO. Longitudinal assessment of neurodevelopment and growth is imperative, including in children with isolated RS. Current variations in practice reinforce the need for evidence-based treatment guidelines.
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