Quality of Life & Phonatory and Morphological Outcomes in Cognitively Unimpaired Adolescents With Pierre Robin Sequence – A Cross-sectional Study in a 72-patient Series

Abstract

We assessed the phonatory and morphological outcome of 72 cognitively unimpaired adolescents with Pierre Robin Sequence (PRS), studied their generic (Kidscreen-52), oral (COHIP-SF19) and vocal (VHI-9i) qualities of life (QoL), and sought to identify determinants of these outcomes. Two-thirds of our adolescents retained low or moderate phonation difficulties but risk factors for them were not identified. For 14%, esthetic results were considered disharmonious with no link to neonatal retrognathia severity. Only two-stage surgery appeared to have a role in improving esthetic results. The generic QoL of these adolescents was slightly lower than that of control patients, especially in dimensions concerning physical well-being, relationships and autonomy. Patients with non-isolated PRS had lower results than those with isolated PRS. Phonatory and morphological sequelae had no impact on generic QoL. Only non-isolated PRS and a low oral QoL had an impact on generic QoL. The oral QoL of these adolescents was comparable to that of control patients and significantly better than that of children with craniofacial malformations. The oral QoL of the adolescents with non-isolated PRS, was significantly worse than that of control patients and close to that of children with craniofacial malformations. The vocal QoL of our subjects was better than that of patients with other voice pathologies and better when phonation was good.