We review the results of a modified quadricepsplasty in five children who developed stiffness of the knee after femoral lengthening for congenital short femur using an Ilizarov external fixator which spanned the knee. All had a full range of movement of the knee before lengthening was undertaken. Unifocal lengthening was carried out in the distal metaphysiodiaphyseal region of the distal femur with a mean gain of 6.5 cm. The mean percentage lengthening was 24%. At the end of one year after removal of the Ilizarov frame and despite intensive physiotherapy all patients had stiffness. Physiotherapy was continued after the quadricepsplasty and, at the latest follow-up (mean 27 months), the mean active flexion was 102° (80 to 130). The gain in movement ranged from 50° to 100°. One patient had a superficial wound infection which settled after a course of oral antibiotics. None developed an increased extension lag after surgery and all were very satisfied with the results. Quadricepsplasty is a useful procedure for stiffness of the knee after femoral lengthening which has not responded to physiotherapy

The traditional techniques involving an oblique tunnel or triangular wedge resection to approach a central or mixed-type physeal bar are hindered by poor visualisation of the bar. This may be overcome by a complete transverse osteotomy at the metaphysis near the growth plate or a direct vertical approach to the bar. Ilizarov external fixation using small wires allows firm fixation of the short physis-bearing fragment, and can also correct an associated angular deformity and permit limb lengthening.

We accurately approached and successfully excised ten central- or mixed-type bars; six in the distal femur, two in the proximal tibia and two in the distal tibia, without damaging the uninvolved physis, and corrected the associated angular deformity and leg-length discrepancy. Callus formation was slightly delayed because of periosteal elevation and stretching during resection of the bar. The resultant resection of the bar was satisfactory in seven patients and fair in three as assessed using a by a modified Williamson–Staheli classification.

Cite this article: Bone Joint J 2015;97-B:1726–31.

Aims

The aim of this study was to report the clinical, functional and radiological outcomes of children and adolescents with tibial fractures treated using the Ilizarov method.

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.

Between 1990 and 2001, 24 children aged between 15 months and 11 years presented with late orthopaedic sequelae after meningococcal septicaemia. The median time to presentation was 32 months (12 to 119) after the acute phase of the disease. The reasons for referral included angular deformity, limb-length discrepancy, joint contracture and problems with prosthetic fitting. Angular deformity with or without limb-length discrepancy was the most common presentation. Partial growth arrest was the cause of the angular deformity. Multiple growth-plate involvement occurred in 14 children. The lower limbs were affected much more often than the upper. Twenty-three children underwent operations for realignment of the mechanical axis and limb-length equalisation. In 15 patients with angular deformity around the knee the deformity recurred. As a result we recommend performing a realignment procedure with epiphysiodesis of the remaining growth plate when correcting angular deformities.

The use of recombinant human bone morphogenetic protein-2 (rhBMP-2) for the treatment of congenital pseudarthrosis of the tibia has been investigated in only one previous study, with promising results. The aim of this study was to determine whether rhBMP-2 might improve the outcome of this disorder. We reviewed the medical records of five patients with a mean age of 7.4 years (2.3 to 21) with congenital pseudarthrosis of the tibia who had been treated with rhBMP-2 and intramedullary rodding. Ilizarov external fixation was also used in four of these patients. Radiological union of the pseudarthrosis was evident in all of them at a mean of 3.5 months (3.2 to 4) post-operatively. The Ilizarov device was removed after a mean of 4.2 months (3.0 to 5.3). These results indicate that treatment of congenital pseudarthrosis of the tibia using rhBMP-2 in combination with intramedullary stabilisation and Ilizarov external fixation may improve the initial rate of union and reduce the time to union.

Further studies with more patients and longer follow-up are necessary to determine whether this surgial procedure may significantly enhance the outcome of congenital pseudarthrosis of the tibia, considering the refracture rate (two of five patients) in this small case series.

The Ilizarov method for leg lengthening was used for cosmetic reasons in 54 patients with constitutional short stature. A mean lengthening of 7 cm with a low rate of complications produced an excellent or good outcome in all the patients, including improvement in psychological disturbances related to short stature. Those who undergo the procedure must be highly motivated, fully informed and understand the technique and possible complications. We suggest that the Ilizarov method for cosmetic limb lengthening is a technique without major complications. However, it requires careful follow-up, and the involvement of orthopaedic surgeons who are familiar with use of the circular frame and are experienced in limb lengthening and correction of deformity for pathological conditions.

Congenital unilateral anterolateral tibial bowing in combination with a bifid ipsilateral great toe is a very rare deformity which resembles the anterolateral tibial bowing that occurs in association with congenital pseudarthrosis of the tibia. However, spontaneous resolution of the deformity without operative treatment and with a continuously straight fibula has been described in all previously reported cases. We report three additional cases and discuss the options for treatment. We suggest that this is a specific entity within the field of anterolateral bowing of the tibia and conclude that it has a much better prognosis than congenital pseudarthrosis of the tibia, although conservative treatment alone may not be sufficient.

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 (Nf1^+/−) mice and control mice. Fractures were open and featured periosteal stripping. All mice received 10 μg rhBMP-2 delivered in a carboxymethylcellulose carrier around the fracture as an anabolic stimulus. Bisphosphonate-treated mice also received five doses of 0.02 mg/kg zoledronic acid given by intraperitoneal injection.

When only rhBMP but no zoledronic acid was used to promote repair, 75% of fractures in Nf1^+/− mice remained ununited at three weeks compared with 7% of controls (p < 0.001). Systemic post-operative administration of zoledronic acid halved the rate of ununited fractures to 37.5% (p < 0.07).

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.

We report our experience of the use of callus distraction with a monolateral fixator for the treatment of acquired radial club-hand deformity after osteomyelitis. Between 1994 and 2004, 13 patients with a mean age of eight years (4 to 15) were treated by callus distraction with a monolateral fixator after a preliminary period of at least four weeks in a corrective short-arm cast.

All patients achieved bony union and were satisfied with the functional and cosmetic outcome. There were no major complications, but three patients required cancellous bone grafting at the docking site for delayed union. Local treatment and oral antibiotics were required for pin-site infection in six patients. There were no deep infection or recurrence of osteomyelitis.