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Developmental outcome of children with Robin sequence: How does the question arise?
A Fleurance1, C Poets2, C Chalouhi1
1Department of General Paediatrics, Reference Centre for Rare Diseases "Pierre Robin Sequences and Congenital Sucking-swallowing Troubles", Necker University Hospital, Paris, France.
Abstract:
Robin sequence (RS) is a heterogeneous congenital condition characterized by retrognathia, glossoptosis, upper airway obstruction, and very often, posterior U-shape cleft palate. Half the children with RS have an underlying syndrome, either identified (syndromic RS) or not (RS+). Long-term intellectual developmental outcome first depends on the underlying diagnosis and is often poor in syndromic cases. On the contrary, the rare studies that analysed the long-term developmental outcome of children with isolated RS who received effective treatment of their respiratory and feeding difficulties early in life, showed intellectual and academic results close to or within the normal range. Speech outcome in RS is often delayed with phonation disorders. Speech difficulties depend on intellectual level, hearing and velar function after palate repair. It affects most children with RS and deserves active monitoring and care.
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