TY - JOUR T1 - Utility of sleep studies in Pierre Robin sequence: A 10-year study JF - European Respiratory Journal JO - Eur Respir J VL - 44 IS - Suppl 58 SP - P4926 AU - Marie Wright AU - Felicity Mehendale AU - Don Urquhart Y1 - 2014/09/01 UR - http://erj.ersjournals.com/content/44/Suppl_58/P4926.abstract N2 - AimChildren with Pierre Robin Sequence (PRS) are at risk of early upper airway obstruction (UAO) and have increased prevalence of childhood obstructive sleep apnoea (OSA). Polysomnography is gold standard for OSA diagnosis and is used to objectively evaluate treatment modalities. This study defines its utility in PRS.MethodsWe retrospective reviewed case-notes and sleep study (SS) reports of 84 children with PRS (born 2004-2013). Sleep studies were performed as limited-channel cardiorespiratory studies with measurement of airflow (allowing confident detection of apnoea) and effort (allowing delineation of central versus obstructive events) in addition to SpO2. Scoring was in accordance with AASM standards.ResultsManagement of UAO included prone positioning (36%), nasopharyngeal airway (51%), tongue-lip adhesion (4%) and tracheostomy (4%).60% children (n=50) underwent SS (1-8 studies/child, mean 2.1), of whom 82% had abnormal studies. Syndromic PRS was highly predictive of abnormal SS (100% vs 76% in isolated PRS).48% underwent SS aged <1 year (median 9d), of whom 98% had an abnormal study; 80% showed OSA and 18% central apnoeas alone. 23% underwent SS aged 1-6 years and 47% had an abnormal study (64% OSA, 36% central apnoeas).SS results guided the following interventions: NPA insertion/removal (n=16), caffeine therapy (n=2), home oxygen (n=2), overnight CPAP (n=3), adenotonsillectomy (n=2).ConclusionsSS play an important role in PRS management, guiding decisions about airway support and timing of surgery, identifying OSA in older children, and demonstrating central apnoeas alone in a subset of patients. All of the above suggest that sleep diagnostics be considered an essential component of cleft services. ER -