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

Author:

Fleurance Alix12,Leunen Dorothée3,Végas Nancy1245,Soupre Véronique26,Griffon Lucie27,Adnot Pauline125,Malecot Gaelle12,Luscan Romain28,Amiel Jeanne2459,Fauroux Brigitte257ORCID,Abadie Véronique125ORCID

Affiliation:

1. General Paediatrics Unit Necker University Hospital Paris France

2. Reference Centre for Rare Disease–Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion‐déglutition congénitaux» Paris France

3. Pedopsychiatric Unit Necker University Hospital Paris France

4. Malformation and Embryology Lab IMAGINE Institute Paris France

5. Paris Cité University Paris France

6. Maxillo‐Facial and Plastic Surgery Unit Necker University Hospital Paris France

7. Sleep and Non‐Invasive Ventilation Unit Paris France

8. Ear Nose and Throat Unit Paris France

9. Medical Genetics Department Necker University Hospital Paris France

Abstract

AbstractAimWe 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.

Publisher

Wiley

Subject

General Medicine,Pediatrics, Perinatology and Child Health

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