Congenital pseudarthrosis of the tibia: the results of an evolving protocol of management.

PURPOSE: This retrospective cohort study assesses the outcomes of a protocol of management, based on the recommendations of the European Paediatric Orthopaedic Society (EPOS) multi-centre study, for the management of congenital pseudarthrosis of the tibia. METHODS: Utilising an incremental protocol of bracing, intramedullary rods and circular frame fixation with or without bone morphogenetic protein-2 (BMP-2), 11 patients had reached skeletal maturity or had follow up of 5 years from radiological union of the pseudarthrosis. Demographic data, deformity parameters before and after treatment, and functional outcome scores were recorded. RESULTS: Ten of the 11 patients successfully healed and two sustained a refracture. All deformity parameters improved and a mean leg length discrepancy of 2.5 cm (range 0-7.5 cm) existed at the time of the last follow up. Some pseudarthroses healed with deformity correction and rod insertion alone. Six of the 11 patients had a confirmed diagnosis of neurofibromatosis and nine had sustained a fracture before 4 years of age. Refracture was associated with malalignment after healing. CONCLUSION: This method of treatment provides a successful stepwise protocol for the management of this complex disorder, avoiding the use of aggressive limb reconstruction techniques at a young age in some cases. Level of evidenceCase series Level IV.