Background Pierre Robin sequence (PRS) is a heterogeneous condition involving retro(micro)gnathia, glossoptosis and upper airway obstruction, very often with posterior cleft palate. Patients with PRS, either isolated or associated with Stickler syndrome have good intellectual prognosis. Nevertheless, the quality of life in adolescence and the phonatory and morphological outcomes are rarely analysed. We assessed the phonatory and morphological outcomes of 72 cognitively unimpaired adolescents with PRS, studied their oral (COHIP-SF19), vocal (VHI-9i) and generic quality of life (QoL; KIDSCREEN-52), and searched for determinants of these outcomes. Results Two-thirds of our adolescents retained low or moderate phonation difficulties, but risk factors were not identified. For 14%, morphological results were considered disharmonious, with no link to neonatal retrognathia severity. Only one vs two-stage surgery seemed to affect final aesthetic results. The oral QoL of these adolescents was comparable to that of control patients and was significantly better than that of children with other craniofacial malformations (COHIP-SF19 = 17.5, 15.4 and 25.7, respectively). The oral QoL of the adolescents with non-isolated PRS was significantly worse (COHIP-SF19 = 24.2) than that of control patients and close to that of children with other craniofacial malformations. The vocal QoL of the adolescents (mean [SD] VHI-9i = 7.5 [5.4]) was better than that of patients with other voice pathologies and better when phonation was good. The generic QoL of the adolescents was satisfactory but slightly lower than that of controls, especially in dimensions concerning physical well-being, relationships and autonomy. QoL results were lower for adolescents with non-isolated than isolated PRS. Only non-isolated PRS and low oral QoL affected generic QoL. Conclusion Morphological or phonatory impairments remain non-rare in adolescents with PRS but do not seem to be directly responsible for altered QoL. These adolescents, especially those with non-isolated PRS, show self-confidence and social-relation fragility. We must focus on long-term functional and psychological results for PRS patients and improve therapy protocols and follow-up, notably those affecting the oral aspects of the disease.
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.
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