Challenges of delivery of dental care and dental pathologies in children and young people with osteogenesis imperfecta

Abstract

Background: Osteogenesis imperfecta (OI) is the most common inherited disorder of bone fragility in children, increasing fracture risk 100-fold and can feature dental and facial bone involvement causing additional morbidities. Aim: To assess the utilisation of tertiary dental services by children and young people with OI attending a supra-regional multi-disciplinary OI service and review of the pathology identified and interventions undertaken. Design: Case notes review of the current caseload of children and young people (0–18 years) with OI at a large regional OI specialist centre (n = 92). Primary outcome was whether an initial dental assessment was arranged in a tertiary dental centre and the corresponding attendance. Results: 49% had a tertiary dental assessment arranged, of whom 82% attended (one quarter requiring several appointments) and 18% did not attend (DNA).Those travelling > 100 miles had a DNA rate of 47%. Assessed children had dentinogenesis imperfecta (24%, 50% in Type III OI), radiographs (95%), caries (41%), required extraction under general anaesthesia (38%) and malocclusion (30%). 48% of the total cohort received bisphosphonates. Conclusion: Tertiary dental assessment encountered barriers to uptake of recommended referral in all patients, often due to geographic factors of travel distance, yet when implemented did identify pathology in a large proportion and many resulted in dental intervention. These emphasise the relevance of specialist dental assessment in OI, particularly in the modern context of increased use of bisphosphonates. This is challenging to achieve and several models of delivery of care may need to be considered in this chronic childhood condition.