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Prolonged bisphosphonates treatment and telescoping implants in OI – Is there synergy?

Background: Administration of Bisphoshonates (BPs) in children with osteogenesis imperfecta (OI) is in almost global use since classic article by Glorieux and co-workers was published in 1998. The third generation of intra-medullary (IM) telescoping nails according to Fassier- Duval (FD) is in use since 2000. Those two novel treatment modalities with more sophisticated rehabilitation programme has significantly changed outcome in OI patients The goal: In the course of time paediatricians and orthopaedic surgeons realised that there are some long-term side-effects of use of BPs and complications of application of FD nails in genetically changed long bones. Those BPs side- effects and surgical complications are well described in relevant medical literature. Aim of this study is to present specific, not described, group of side-effects of prolonged use of BPs in children with severe OI. Material and methods: Out of 31 surgically treated segments in 13 patients with severe types (Type III and Type IV) of OI, we present unique series of seven patients with 11 segments who were surgically treated with FD nails. Inclusion criteria: 1. Patients with more than 12 cyclic infusions of BPs ; 2. Long bone segment deformation of 60 or more degrees. 3. Segment that had not previous surgery. Surgery was performed in a way that is described in the literature. Due to extremely distorted geometry of long bone and sclerosis, even obliteration of IM canal some specific surgical approach was developed. Results: All segments were straight after surgical excision of trapezoid bone part at the apex of deformity. In addition, soft tissues release was utilized, when necessary, to avoid over pressure at the ends of bone fragments. IM canal widening was performed with patience and gradual drilling to avoid breakage of sclerotic or obliterated bone. In that way, central position of IM implants was secured in the most segments. In two segments correction of position was necessary, at the beginning of this series. Conclusion: The side-effect of prolonged use of BPs in severe deformed segment are obliterated IM canal and rib- shape diaphysis of the long bone produce additional challenge for the surgical treatment. We are against prolonged use of BPs with no surgical correction, because significant intra and extra long bone deformity with potential complications during surgical correction.

bisphosphonates, surgical treatment with telescoping implants, osteogenesis imperfecta

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