N Nicolaou1, A Ghassemi2, R A Hill2. 1. Maidstone and Tunbridge Wells NHS Trust, Maidstone, Kent UK. 2. Great Ormond Street Hospital for Children NHS Trust, London, UK.
Abstract
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.
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.
Entities:
Keywords:
Management; Pseudarthrosis of the tibia; Treatment
Authors: Jad El-Hoss; Kate Sullivan; Tegan Cheng; Nicole Y C Yu; Justin D Bobyn; Lauren Peacock; Kathy Mikulec; Paul Baldock; Ian E Alexander; Aaron Schindeler; David G Little Journal: J Bone Miner Res Date: 2012-01 Impact factor: 6.741
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