Aims. 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. Methods. 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
Two types of fracture, early and late, have been
reported following limb lengthening in patients with achondroplasia (ACH)
and hypochondroplasia (HCH). We reviewed 25 patients with these conditions who underwent 72
segmental limb lengthening procedures involving the femur and/or
tibia, between 2003 and 2011. Gender, age at surgery, lengthened
segment, body mass index, the shape of the callus, the amount and
percentage of lengthening and the healing index were evaluated to determine
predictive factors for the occurrence of early (within three weeks
after removal of the fixation pins) and late fracture (>
three weeks
after removal of the pins). The Mann‑Whitney U test and Pearson’s
chi-squared test for univariate analysis and stepwise regression
model for multivariate analysis were used to identify the predictive factor
for each fracture. Only one patient (two tibiae) was excluded from
the analysis due to excessively slow formation of the regenerate,
which required supplementary measures. A total of 24 patients with
70 limbs were included in the study. There were 11 early fractures in eight patients. The shape of
the callus (lateral or central callus) was the only statistical
variable related to the occurrence of early fracture in univariate
and multivariate analyses. Late fracture was observed in six limbs
and the mean time between removal of the fixation pins and fracture
was 18.3 weeks (3.3 to 38.4). Lengthening of the tibia, larger healing
index, and lateral or central callus were related to the occurrence
of a late fracture in univariate analysis. A multivariate analysis
demonstrated that the shape of the callus was the strongest predictor
for late fracture (odds ratio: 19.3, 95% confidence interval: 2.91
to 128). Lateral or central callus had a significantly larger risk
of fracture than fusiform, cylindrical, or concave callus. Radiological monitoring of the shape of the callus during distraction
is important to prevent early and late fracture of lengthened limbs
in patients with ACH or HCH. In patients with thin callus formation,
some measures to stimulate bone formation should be considered as
early as possible. Cite this article:
Congenital pseudarthrosis of the tibia (CPT)
is a rare but well recognised condition. Obtaining union of the pseudarthrosis
in these children is often difficult and may require several surgical
procedures. The treatment has changed significantly since the review
by Hardinge in 1972, but controversies continue as to the best form
of surgical treatment. This paper reviews these controversies. Cite this article:
Congenital pseudarthrosis of the tibia is an uncommon manifestation of neurofibromatosis type 1 (NF1), but one that remains difficult to treat due to anabolic deficiency and catabolic excess. Bone grafting and more recently recombinant human bone morphogenetic proteins (rhBMPs) have been identified as pro-anabolic stimuli with the potential to improve the outcome after surgery. As an additional pharmaceutical intervention, we describe the combined use of rhBMP-2 and the
