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BISPHOSPHONATE THERAPY IN OSTEOGENESIS IMPERFECTA -- IS IT DANGEROUS?



Abstract

Children with osteogenesis imperfecta(OI) have multiple long bone fractures with subsequent deformities. The mainstay of treatment is correction with multiple osteotomies and intramedullary fixation. The Shefffield intramedullary telescoping rod system has been successful in the treament of long bone fractures and deformities (Wilkinson et al ,JBJS-B,1998) Bisphosphonates (Pamidronate -1- 1.5mg/kg/day)have been used as adjuvant therapy in the treatment of OI since the last five years. The perceived benefits include reduction in fracture frequency, improvement in bone density and a general feeling of well being.

We present our experience of five cases of OI who developed infections around thier Sheffield telescoping rods while on Pamidronate therapy. There was only one case of sepsis over a ten year period(over eighty patients)in a previously reported series from our centre.

The time interval between the start of Pamidronate therapy and the diagnosis of infection varied between 12–36 months ie. between 4–12 cycles of Pamidronate (parenteral administration over a three day period at three month intervals). All patients had their intramedullary rods in situ from anywhere between 2–7 years. The infections were low grade with a 2–3 month period of dull ache prior to actual presentation. Intrestigly though all patients had multiple rods in situ, only one of their femoral rods was affected and they did not have any other infective focus at the time of diagnosis. Three patients presented with thigh abcesses while the other two presented with ipsilateral knee pain and effusion. All had raised inflammatory markers, radiological signs of sepsis with Staph Aureus the commonest infecting organism.

Those cases presenting with abcesses were treated by drainage and rod removal, however only antibiotics were sufficient in the rest. The relationship between Pamidronate therapy and these infections is not absolutely clear and has not been reported previously. The possible links are discussed and a high degree of suspicion is recommended for those cases of OI on bisphosphonate

Local Host: British Society for Children’s Orthopaedic Surgery. Conference Theme: Congenital Deficiencies of the Lower Limb. These abstracts were prepared by A.Catterall.