Babies With Pierre Robin Sequence: Neuropsychomotor Development

Souza, Caroline Duchatsch Ribeiro de; Padovani, Leticia Faccim; Ferreira-Donati, Grace Cristina; Moraes, Márcia Cristina Almendros Fernandes; Corrêa, Camila de Castro; Maximino, Luciana Paula

doi:10.1016/j.pediatrneurol.2023.01.010

Cited by 4 publications

(3 citation statements)

References 14 publications

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“…The previous study by the Necker team showed that psychomotor and cognitive development improved over time, with a DQ‐global of 90.5 at 15 months of age according to the Brunet Lézine scale and 100.9 at 3 years and 109.1 at 6 years according to the Kaufman Assessment Battery for Children 15 . This improvement over time was also evoked in the recent Brazil study, showing that infants with RS who were <12 months of age were at risk of developmental delay 24 . Several hypotheses can explain this improvement over time.…”

Section: Discussionmentioning

confidence: 77%

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Developmental outcome of children with Robin sequence treated with the current Paris protocol

Fleurance,

Leunen,

Végas

et al. 2023

Acta Paediatrica

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AimWe aimed to investigate the developmental outcome of children with Robin sequence (RS) for whom continuous positive airway pressure was the main strategy to release upper airway obstruction.MethodsWe included children with isolated RS or RS associated with Stickler syndrome who were aged 15 months to 6 years. We used the French version of the Child Development Inventory and calculated the developmental quotient (DQ) for eight different domains and the global DQ (DQ‐global). We searched for determinants of risk of delay.ResultsOf the 87 children, for 71%, the developmental evolution was within the norm (DQ‐global ≥86 or ≥−1 SD), 29% were at high risk of delay (DQ‐global <86 or <−1 SD), and only 3% were at very high risk of delay (DQ‐global <70 or <−2 SD). The DQs for expressive language and language comprehension were lower in our study population than the general population, but an improvement was noticed with the children's growth.ConclusionRisk of a developmental delay was not greater for children with the most severe respiratory phenotype than the others. Children whose mothers had low education levels were more at risk than the others.

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Section: Discussionmentioning

confidence: 77%

“…15 This improvement over time was also evoked in the recent Brazil study, showing that infants with RS who were <12 months of age were at risk of developmental delay. 24 Several hypotheses can explain this improvement over time. Otitis and hypernasality predominate in younger children.…”

Section: Discussionmentioning

confidence: 99%

Developmental outcome of children with Robin sequence treated with the current Paris protocol

Fleurance,

Leunen,

Végas

et al. 2023

Acta Paediatrica

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“…There are conflicting reports about whether children with isolated RS are at risk of NDD. Several studies have reported neurocognitive impairment, which is typically mild and demonstrates improvement over time [34][35][36] . However, case-control studies involving Denver II developmental screening and intelligence quotient testing have reported similar results between children with RS and age-matched healthy controls 37,38 .…”

Section: Neurodevelopmental Delaymentioning

confidence: 99%

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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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

Pediatric Pulmonology

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ObjectiveThere 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.

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Babies With Pierre Robin Sequence: Neuropsychomotor Development

Cited by 4 publications

References 14 publications

Developmental outcome of children with Robin sequence treated with the current Paris protocol

Developmental outcome of children with Robin sequence treated with the current Paris protocol

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

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

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