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 bisphosphonate zoledronic acid in a mouse model of NF1-deficient fracture repair. Fractures were generated in the distal tibiae of neurofibromatosis type 1-deficient ( When only rhBMP but no zoledronic acid was used to promote repair, 75% of fractures in These data support the concept that preventing bone loss in combination with anabolic stimulation may improve the outcome following surgical treatment for children with congenital pseudarthoris of the tibia and NF1.
Two protocols for the operative technique and care of the pin-site with external fixation were compared prospectively. There was a total of 120 patients with 46 in group A and 74 in group B. Infection was defined as an episode of pain or inflammation at a pin site, accompanied by a discharge which was either positive on bacterial culture or responded to a course of antibiotics. Patients in group B had a lower proportion of infected pin sites (p = 0.003) and the time to the first episode of infection was longer (p <
0.001). The risk of pin-site infection is lower if attention is paid to avoiding thermal injury and local formation of haematoma during surgery and if after-care includes the use of an alcoholic antiseptic and occlusive pressure dressings.
We present two children with massive defects of the tibia and an associated active infection who were treated by medial transport of the fibula using the Ilizarov device. The first child had chronic discharging osteomyelitis which affected the whole tibial shaft. The second had sustained bilateral grade-IIIB open tibial fractures in a motor-car accident. The first child was followed up for three years and the second for two years. Both achieved solid union between the proximal and distal stumps of the tibia and the fibula, with hypertrophy of the fibula. The first child had a normal range of movement at the knee, ankle and foot but there was shortening of 1.5 cm. The second had persistent anterior angulation at the proximal tibiofibular junction and the ankle was stiff in equinus.
Compartment pressures have not previously been studied in healthy children. We compared the pressures in the four lower leg compartments of healthy children with those of healthy adults. We included patients aged between two months and six years, and measured the pressures in 80 compartments of 20 healthy children using simple needle manometry. Measurements were repeated in a control group of 20 healthy adults. The mean compartment pressure in the lower leg in children was significantly higher than in adults (p <
0.001). On average, pressures in the four compartments varied between 13.3 mmHg and 16.6 mmHg in the children and between 5.2 mmHg and 9.7 mmHg in the adults. The latter is in accordance with those recorded in the literature. The mean arterial pressure did not relate to age or to pressure in the compartment. The findings of this study that the normal compartment pressure of the lower leg in healthy children is significantly higher than that in adults may be of considerable significance in clinical decision-making in children of this age.
