Management of upper airway obstruction in infants with Robin sequence in the United Kingdom

Abstract

Introduction: Robin sequence (RS) is a congenital disorder resulting in upper airway obstruction (UAO) which can be late-onset or subclinical, leading to risk of under-recognition and associated long-term morbidity. There is currently no consensus about best-practice management of UAO in RS. We aimed to describe current management of RS-related UAO in the United Kingdom and Ireland (UK/I), as a prelude to developing a national clinical guideline. Methods: A surveillance study was conducted throughout UK/I (Jan 2016 - Jan 2017) using a monthly reporting card distributed to 3500 paediatricians/neonatologists and regional cleft teams. For each reported case of RS, a questionnaire was provided to collect detailed clinical data (85% response rate). Results: 153 infants with confirmed RS (52% isolated RS) were identified. 95% were admitted to a neonatal or paediatric unit; specialist respiratory input was sought in 54% cases. 87% had signs of UAO and 66% required an airway adjunct (AA); nasopharyngeal airway (NPA) in 56%, CPAP 27%, endotracheal intubation 11%, tracheostomy 11%. 29% were discharged with NPA after median 21 days. Tracheostomy was associated with non-isolated RS (p=0.002). 52% underwent sleep study (70% oximetry) at median 17 days old, which changed practice in 47% cases (AA required, 32%; AA not required, 15%). Conclusion: UK/I centres favour non-surgical UAO management, mainly with NPA. Surgery is reserved for treatment failure, mainly in non-isolated RS. Our findings differ from those reported by North American and European centres, reinforcing the need for a consensus guideline. Data collection is underway to compare 1-year clinical outcomes between different UAO management approaches.