Airway management in infants with Robin sequence in the United Kingdom and Ireland: A prospective population‐based study

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Autori principali: Marie Wright, Rachel L. Knowles, Mario Cortina‐Borja, Sheila Javadpour, Felicity V. Mehendale, Don S. Urquhart
Natura: Artículo Open Access
Pubblicazione: Wiley 2024
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author Marie Wright
Rachel L. Knowles
Mario Cortina‐Borja
Sheila Javadpour
Felicity V. Mehendale
Don S. Urquhart
author_facet Marie Wright
Rachel L. Knowles
Mario Cortina‐Borja
Sheila Javadpour
Felicity V. Mehendale
Don S. Urquhart
Marie Wright
Rachel L. Knowles
Mario Cortina‐Borja
Sheila Javadpour
Felicity V. Mehendale
Don S. Urquhart
collection Wiley Open Access
contents Airway management in infants with Robin sequence in the United Kingdom and Ireland: A prospective population‐based study Marie Wright Rachel L. Knowles Mario Cortina‐Borja Sheila Javadpour Felicity V. Mehendale Don S. Urquhart Pediatric Pulmonology AbstractObjectiveThere 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.MethodsActive 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.Results173 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.ConclusionsIn 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. 10.1002/ppul.27140 http://creativecommons.org/licenses/by/4.0/
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spellingShingle Airway management in infants with Robin sequence in the United Kingdom and Ireland: A prospective population‐based study
Marie Wright
Rachel L. Knowles
Mario Cortina‐Borja
Sheila Javadpour
Felicity V. Mehendale
Don S. Urquhart
Pediatric Pulmonology
Airway management in infants with Robin sequence in the United Kingdom and Ireland: A prospective population‐based study Marie Wright Rachel L. Knowles Mario Cortina‐Borja Sheila Javadpour Felicity V. Mehendale Don S. Urquhart Pediatric Pulmonology AbstractObjectiveThere 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.MethodsActive 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.Results173 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.ConclusionsIn 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. 10.1002/ppul.27140 http://creativecommons.org/licenses/by/4.0/
title Airway management in infants with Robin sequence in the United Kingdom and Ireland: A prospective population‐based study
topic Pediatric Pulmonology
url https://onlinelibrary.wiley.com/doi/10.1002/ppul.27140