Management of Upper Airway Obstruction in Infants with Robin Sequence in the United Kingdom

Marie Wright, Sheila Javadpour, Patricia Jackson, Edile Murdoch, Felicity Mehendale, Don Urquhart

Research output: Contribution to journalArticlepeer-review

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. Results: 153 infants with confirmed RS (52 were identified. 95 specialist respiratory input was sought in 5476AA); nasopharyngeal airway (NPA) in 56 CPAP 27 endotracheal intubation 11 tracheostomy 1191 days. Tracheostomy was associated with non-isolated RS (p=0.002). 5270 at median 17 days old, which changed practice in 47AA 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.
Original languageEnglish
JournalEuropean Respiratory Journal
Volume52
Issue numbersuppl 62
DOIs
Publication statusPublished - 1 Sep 2018

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