Osteofibrous dysplasia (OFD) is a rare benign lesion predominantly affecting the tibia in children. Its potential link to adamantinoma has influenced management. This international case series reviews the presentation of OFD and management approaches to improve our understanding of OFD. A retrospective review at three paediatric tertiary centres identified 101 cases of tibial OFD in 99 patients. The clinical records, radiological images, and histology were analyzed.Aims
Methods
The Fassier Duval (FD) rod is a third-generation telescopic implant for children with osteogenesis imperfecta (OI). Threaded fixation enables proximal insertion without opening the knee or ankle joint. We have reviewed our combined two-centre experience with this implant. In total, 34 children with a mean age of five years (1 to 14) with severe OI have undergone rodding of 72 lower limb long bones (27 tibial, 45 femoral) for recurrent fractures with progressive deformity despite optimized bone health and bisphosphonate therapy. Data were collected prospectively, with 1.5 to 11 years follow-up.Aims
Methods
In 1937 Blount described a series of 28 patients with ‘Tibia vara’. Since then, a number of deformities in the tibia and the femur have been described in association with this condition. We analysed 14 children with Blount’s disease who were entered into a cross-sectional study. Their mean age was 10 (2 to 18). They underwent a clinical assessment of the rotational profile of their legs and a CT assessment of the angle of anteversion of their hips (femoral version). We compared our results to previously published controls. A statistically significant increase in femoral anteversion was noted in the affected legs, with on average the femurs in patients with Blount’s disease being 26° more anteverted than those in previously published controls. We believe this to be a previously unrecognised component of Blount’s disease, and that the marked intoeing seen in the disease may be partly caused by internal femoral version, in addition to the well-recognised internal tibial version.
We investigated patterns of refracture and their risk factors in patients with congenital pseudarthrosis of the tibia after Ilizarov osteosynthesis. We studied 43 cases in 23 patients. Temporal and spatial patterns of refracture and refracture-free survival were analysed in each case. The refracture-free rate of cumulative survival was 47% at five years and did not change thereafter. Refracture occurred at the previous pseudarthrosis in 16 of 19 cases of refracture. The risk of refracture was significantly higher when osteosynthesis was performed below the age of four years, when the tibial cross-sectional area was narrow, and when associated with persistent fibular pseudarthrosis. Refracture occurs frequently after successful osteosynthesis in these patients. Delaying osteosynthesis, maximising the tibial cross-sectional area and stabilising the fibula may reduce the risk of refracture.
