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Developmental outcome of children with Robin sequence treated with the current Paris protocol.
Fleurance, Alix; Leunen, Dorothée; Végas, Nancy; Soupre, Véronique; Griffon, Lucie; Adnot, Pauline; Malecot, Gaelle; Luscan, Romain; Amiel, Jeanne; Fauroux, Brigitte; Abadie, Véronique.
Affiliation
  • Fleurance A; General Paediatrics Unit, Necker University Hospital, Paris, France.
  • Leunen D; Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux¼, Paris, France.
  • Végas N; Pedopsychiatric Unit, Necker University Hospital, Paris, France.
  • Soupre V; General Paediatrics Unit, Necker University Hospital, Paris, France.
  • Griffon L; Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux¼, Paris, France.
  • Adnot P; Malformation and Embryology Lab, IMAGINE Institute, Paris, France.
  • Malecot G; Paris Cité University, Paris, France.
  • Luscan R; Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux¼, Paris, France.
  • Amiel J; Maxillo-Facial and Plastic Surgery Unit, Necker University Hospital, Paris, France.
  • Fauroux B; Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux¼, Paris, France.
  • Abadie V; Sleep and Non-Invasive Ventilation Unit, Paris, France.
Acta Paediatr ; 112(12): 2601-2610, 2023 12.
Article in En | MEDLINE | ID: mdl-37786287
ABSTRACT

AIM:

We 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.

METHODS:

We 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.

RESULTS:

Of 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.

CONCLUSION:

Risk 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.
Subject(s)
Key words

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Pierre Robin Syndrome / Hearing Loss, Sensorineural Limits: Child / Female / Humans / Infant Country/Region as subject: Europa Language: En Journal: Acta Paediatr Year: 2023 Document type: Article

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Pierre Robin Syndrome / Hearing Loss, Sensorineural Limits: Child / Female / Humans / Infant Country/Region as subject: Europa Language: En Journal: Acta Paediatr Year: 2023 Document type: Article