Addressing surgical challenges in patients with severe form of osteogenesis imperfecta and with prolonged bisphosphonate treatment: intramedullary sclerosis and technical solutions.

PURPOSE: Osteogenesis imperfecta (OI) is commonly managed with bisphosphonates (BPs) which are associated with significant side effects. This study aimed to investigate intramedullary sclerosis as a potential side effect of prolonged BP use in paediatric patients.

METHODS

Thirteen children with OI underwent surgery at our hospital. We assessed 21 non-operated bone segments from 13 paediatric patients using a tailored protocol to measure sclerosis severity and its impact on surgical outcomes.

RESULTS

Among 21 non-operated segments, 17 exhibited intramedullary sclerosis. Pamidronate cycles inversely correlated with the percentage of the sclerosing area and varus deformity, plateauing after 15 cycles. Angulation in the sagittal plane decreased progressively.

CONCLUSIONS

Our study introduces intramedullary sclerosis as a possible novel BP-related side-effect in pediatric OI patients. Our findings suggest a critical review of prolonged BP therapy in OI patients, advocating for a surgical intervention threshold after fifteen BP cycles to mitigate complications and optimize treatment outcomes.