Epidemiology of Robin sequence in the United Kingdom and Ireland: findings of a national surveillance study

Wright, Marie; Mehendale, Felicity V.; Jackson, Patricia; Murdoch, Edile; Javadpour, Sheila; Urquhart, Don S.

doi:10.1183/13993003.congress-2020.3509

Cited by 3 publications

(21 citation statements)

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“…This is significantly higher than previous estimates, which may have been miscalculated 1. Importantly, this study also identified a striking geographical variation in prevalence estimates, which were lowest in England and Wales (1/5789), higher in Ireland (1/4635) and highest in Scotland (1/2692) 1. These findings are broadly corroborated by an earlier study by Wright et al (2018), which identified a PRS incidence of 1/2685 in an East of Scotland patient cohort 2.…”

Section: Introductioncontrasting

confidence: 74%

“…PRS is reported to have a prevalence of ~1/8000 to 1/14000 live births 1 2. A recent study by Wright et al ( ADC ), which used dual-source case ascertainment and active surveillance methods, estimated a PRS prevalence of 1:5246 live births 1. This is significantly higher than previous estimates, which may have been miscalculated 1.…”

Section: Introductionmentioning

confidence: 91%

“…Pierre Robin sequence (PRS) is a rare congenital malformation sequence characterised by micrognathia, glossoptosis and upper airway obstruction, often in association with a wide U-shaped cleft palate 1. The aetiology of non-syndromic PRS (nsPRS) remains uncertain; however, non-isolated PRS can present as part of a wider syndromic diagnosis1 (syndromic PRS (sPRS)). PRS is reported to have a prevalence of ~1/8000 to 1/14000 live births 1 2.…”

Section: Introductionmentioning

confidence: 99%

“…The aetiology of non-syndromic PRS (nsPRS) remains uncertain; however, non-isolated PRS can present as part of a wider syndromic diagnosis1 (syndromic PRS (sPRS)). PRS is reported to have a prevalence of ~1/8000 to 1/14000 live births 1 2. A recent study by Wright et al ( ADC ), which used dual-source case ascertainment and active surveillance methods, estimated a PRS prevalence of 1:5246 live births 1.…”

Section: Introductionmentioning

confidence: 99%

“…A recent study by Wright et al ( ADC ), which used dual-source case ascertainment and active surveillance methods, estimated a PRS prevalence of 1:5246 live births 1. This is significantly higher than previous estimates, which may have been miscalculated 1. Importantly, this study also identified a striking geographical variation in prevalence estimates, which were lowest in England and Wales (1/5789), higher in Ireland (1/4635) and highest in Scotland (1/2692) 1.…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Epidemiology of Robin sequence: geographical variation in the UK/Ireland

Atton,

Baralle

2024

Arch Dis Child

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Section: Introductioncontrasting

confidence: 74%

Section: Introductionmentioning

confidence: 91%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Epidemiology of Robin sequence: geographical variation in the UK/Ireland

Atton,

Baralle

2024

Arch Dis Child

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Computerized Surgical Planning for Mandibular Distraction Osteogenesis

Hu,

Aral,

Rancu

et al. 2024

Semin Plast Surg

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Mandibular distraction osteogenesis is a technically challenging procedure due to complex mandibular anatomy, especially in the treatment of Pierre-Robin Sequence due to variable bone thickness in the infant mandible and the presence of tooth buds. Computerized surgical planning (CSP) simplifies the procedure by preoperatively visualizing critical structures, producing cutting guides, and planning distractor placement. This paper describes the process of using CSP to plan mandibular distraction osteogenesis, including discussion of recent advances in the use of custom distractors.

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

Wright,

Knowles,

Cortina-Borja

et al. 2024

Preprint

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Objective There 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 two years of life. Methods Active 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. Results 173 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 isolated RS cases. Conclusions In 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.

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Epidemiology of Robin sequence in the United Kingdom and Ireland: findings of a national surveillance study

Cited by 3 publications

References 0 publications

Epidemiology of Robin sequence: geographical variation in the UK/Ireland

Epidemiology of Robin sequence: geographical variation in the UK/Ireland

Computerized Surgical Planning for Mandibular Distraction Osteogenesis

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

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