Mandibular distraction osteogenesis in children with Pierre Robin sequence: impact on health-related quality of life.

Background: Upper airway obstruction in children with Pierre Robin sequence (PRS) may be severe enough to require surgical intervention. Although many studies have demonstrated the effectiveness of mandibular distraction osteogenesis (MDO) in relieving airway obstruction, no study has reported health-related quality of life (QOL) outcomes. The objective of the present study was to conduct the first health-related QOL assessment post-MDO.

Methods: The Glasgow Children's Benefit Inventory (GCBI) questionnaire was distributed in a retrospective manner to the caregivers of all children who had undergone MDO at the authors' institutions between January 2007 and December 2010. Patients who had other major surgical procedures were excluded.

Results: The response rate was 84% (21 of 25 questionnaires completed). The total mean GCBI score was +54 (SD, 19.5), which demonstrated a subjective overall benefit in health-related QOL post-MDO. All of the domains within the GCBI also scored in the positive range, indicating domain specific improvements in health-related QOL. There were no significant differences in the GCBI scores between syndromic-PRS patients and isolated PRS patients; similar results were observed between tracheostomy patients and no tracheostomy patients.

Summary: In treating children with PRS and severe upper airway obstruction, MDO resulted in a subjective overall benefit in health-related QOL in our study population.