INTRODUCTION: Pierre Robin sequence (PRS) is a congenital anomaly presenting with micrognathia, glossoptosis and a cleft palate. This study describes a decade's experience of the management of upper airway obstruction (UAO) in PRS patients with a nasopharyngeal airway (NPA).
METHODS: This study was conducted by paediatric respiratory and otolaryngology departments. Children with PRS referred with UAO were evaluated according to a standard protocol. Data collected included the degree of airway obstruction, method of airway management, polysomnography data before and after intervention, and longer term follow-up.
RESULTS: Data were collected on 104 PRS patients referred to us for airway assessment in 2000-2010. 64/104 were aged
CONCLUSION: There is a spectrum of UAO in PRS. This study reports on long-term outcomes in 104 children with PRS and airway obstruction. In most children (86.5%), airway obstruction was managed by conservative measures or with an NPA for a few months. The natural history shows that with normal growth, airway compromise resolves without immediate surgical intervention as advocated by some practitioners. Few PRS children require a tracheostomy.
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