Oral health-related quality of life in children and adolescents with osteogenesis imperfecta: cross-sectional study

BACKGROUND: Osteogenesis imperfecta (OI) affects dental and craniofacial development and may therefore impair Oral Health-Related Quality of Life (OHRQoL). However, little is known about OHRQoL in children and adolescents with OI. The aim of this study was to explore the influence of OI severity on oral health-related quality of life in children and adolescents. METHODS: Children and adolescents aged 8–14 years were recruited in the context of a multicenter longitudinal study (Brittle Bone Disease Consortium) that enrolls individuals with OI in 10 centers across North America. OHRQoL was assessed using the Child Perceptions Questionnaire (CPQ) versions for 8 to 10-year-olds (CPQ(8–10)) and for 11 to 14-year-olds (CPQ(11–14)). RESULTS: A total of 138 children and adolescents (62% girls) diagnosed with OI types I, III, IV, V and VI (n = 65, 30, 37, 4 and 2, respectively) participated in the study. CPQ(8–10) scores were similar between OI types in children aged 8 to 10 years. In the 11 to 14-year-old group, CPQ(11–14)-scores were significantly higher (i.e. worse) for OI types III (24.7 [SD 12.5]) and IV (23.1 [SD 14.8]) than for OI type I (16.5 [SD 12.8]) (P < 0.05). The difference between OI types was due to the association between OI types and the functional limitations domain, as OI types III and IV were associated with significantly higher grade of functional limitations compared to OI type I. CONCLUSION: The severity of OI impacts OHRQoL in adolescents aged 11 to 14 years, but not in children age 8 to 10 years. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s13023-018-0935-y) contains supplementary material, which is available to authorized users.