We have treated seven children with relapsed infantile Blount’s disease by elevation of the hemiplateau using the Ilizarov frame. Three boys and four girls with a mean age of 10.5 years were reviewed at a mean of 29 months after surgery. All had improved considerably and were pleased with the results. The improvements in radiological measurements were statistically significant (p < 0.001). Three-dimensional CT reconstruction was useful for planning surgery. There were no major complications. The advantages of this technique are that in addition to elevation of the hemiplateau, rotational deformities and limb-length discrepancies may be addressed

Aims. We wished to examine the effectiveness of tibial lengthening using a two ring Ilizarov frame in skeletally immature patients. This is a potentially biomechanically unstable construct which risks the loss of axial control. Patients and Methods. We retrospectively reviewed a consecutive series of 24 boys and 26 girls, with a mean age of 8.6 years (4 to 14), who underwent 52 tibial lengthening procedures with a mean follow-up of 4.3 years (4.0 to 16.9). Tibial alignment was measured before and after treatment using joint orientation lines from the knee and a calculation of the oblique plane axis. Results. The mean percentage length gain was 20% (13 to 31) with a mean bone-healing index (BHI) of 34 days per centimetre (20 to 54). Age at surgery and location of the osteotomy site showed a strong relationship as the location of the osteotomy migrated more proximally with age. However, no significant correlation was found between BHI and the position of the osteotomy. The coronal and sagittal joint orientation of the lengthened tibias were within the normal range in 67% and 92% of cases, respectively. The oblique plane angulation calculation revealed a statistically significant change pre- to post-operatively in both magnitude (p < 0.05) and direction (p < 0.01), but without clinical consequence. The majority of complications were minor (n = 40 in 27 patients; 1.48 complications per segment lengthened) with no residual disability reported. Conclusion. Two-ring tibial lengthening is effective in maintaining segmental alignment, efficient in callous production and relatively comfortable for the patient with few significant complications. Cite this article: Bone Joint J 2016;98-B:1276–82

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

We performed limb lengthening and correction of deformity of nine long bones of the lower limb in six children (mean age, 14.7 years) with osteogenesis imperfecta (OI). All had femoral lengthening and three also had ipsilateral tibial lengthening. Angular deformities were corrected simultaneously. Five limb segments were treated using a monolateral external fixator and four with the Ilizarov frame. In three children, lengthening was done over previously inserted femoral intramedullary rods. The mean lengthening achieved was 6.26 cm (mean healing index, 33.25 days/cm). Significant complications included one deep infection, one fracture of the femur and one anterior angulation deformity of the tibia. The abnormal bone of OI tolerated the external fixators throughout the period of lengthening without any episodes of migration of wires or pins through the soft bone. The regenerate bone formed within the time which is normally expected in limb-lengthening procedures performed for other conditions. We conclude that despite the abnormal bone characteristics, distraction osteogenesis to correct limb-length discrepancy and angular deformity can be performed safely in children with OI

Aims

The aim of this retrospective cohort study was to assess and investigate the safety and efficacy of using a distal tibial osteotomy compared to proximal osteotomy for limb lengthening in children.

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 report the effect of introducing a dedicated Ponseti service on the five-year treatment outcomes of children with idiopathic clubfoot.

Between 2002 and 2004, 100 feet (66 children; 50 boys and 16 girls) were treated in a general paediatric orthopaedic clinic. Of these, 96 feet (96%) responded to initial casting, 85 requiring a tenotomy of the tendo-Achillis. Recurrent deformity occurred in 38 feet and was successfully treated in 22 by repeat casting and/or tenotomy and/or transfer of the tendon of tibialis anterior, The remaining 16 required an extensive surgical release.

Between 2005 and 2006, 72 feet (53 children; 33 boys and 20 girls) were treated in a dedicated multidisciplinary Ponseti clinic. All responded to initial casting: 60 feet (83.3%) required a tenotomy of the tendo-Achillis. Recurrent deformity developed in 14, 11 of which were successfully treated by repeat casting and/or tenotomy and/or transfer of the tendon of tibialis anterior. The other three required an extensive surgical release.

Statistical analysis showed that children treated in the dedicated Ponseti clinic had a lower rate of recurrence (p = 0.068) and a lower rate of surgical release (p = 0.01) than those treated in the general clinic.

This study shows that a dedicated Ponseti clinic, run by a well-trained multidisciplinary team, can improve the outcome of idiopathic clubfoot deformity.

Cite this article: Bone Joint J 2014;96-B:1424–6

Osteofibrous dysplasia is an unusual developmental condition of childhood, which almost exclusively affects the tibia. It is thought to follow a slowly progressive course and to stabilise after skeletal maturity. The possible link with adamantinoma is controversial and some authors believe that they are part of one histological process.

We retrospectively reviewed 16 patients who were diagnosed as having osteofibrous dysplasia initially or on the final histological examination. Their management was diverse, depending on the severity of symptoms and the extent of the lesion. Definitive (extraperiosteal) surgery was localised ‘shark-bite’ excision for small lesions in five patients. Extensive lesions were treated by segmental excision and fibular autograft in six patients, external fixation and bone transport in four and proximal tibial replacement in one. One patient who had a fibular autograft required further excision and bone transport for recurrence. Six initially underwent curettage and all had recurrence. There were no recurrences after localised extraperiosteal excision or bone transport. There were three confirmed cases of adamantinoma.

The relevant literature is reviewed. We recommend extraperiosteal excision in all cases of osteofibrous dysplasia, with segmental excision and reconstruction in more extensive lesions.

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.

We report the outcome of 28 patients with spina bifida who between 1989 and 2006 underwent 43 lower extremity deformity corrections using the Ilizarov technique. The indications were a flexion deformity of the knee in 13 limbs, tibial rotational deformity in 11 and foot deformity in 19. The mean age at operation was 12.3 years (5.2 to 20.6). Patients had a mean of 1.6 previous operations (0 to 5) on the affected limb. The mean duration of treatment with a frame was 9.4 weeks (3 to 26) and the mean follow-up was 4.4 years (1 to 9). There were 12 problems (27.9%), five obstacles (11.6%) and 13 complications (30.2%) in the 43 procedures. Further operations were needed in seven patients. Three knees had significant recurrence of deformity. Two tibiae required further surgery for recurrence. All feet were plantigrade and braceable.

We conclude that the Ilizarov technique offers a refreshing approach to the complex lower-limb deformity in spina bifida.

We describe three cases of infantile tibia vara resulting from an atraumatic slip of the proximal tibial epiphysis upon the metaphysis. There appears to be an association between this condition and severe obesity. Radiologically, the condition is characterised by a dome-shaped metaphysis, an open growth plate and disruption of the continuity between the lateral borders of the epiphysis and metaphysis, with inferomedial translation of the proximal tibial epiphysis. All patients were treated by realignment of the proximal tibia by distraction osteogenesis with an external circulator fixator, and it is suggested that this is the optimal method for correction of this complex deformity. There are differences in the radiological features and management between conventional infantile Blount’s disease and this ‘slipped upper tibial epiphysis’ variant.

We undertook a retrospective analysis of 306 procedures on 233 patients, with a mean age of 12 years (1 to 21), in order to evaluate the use of somatosensory evoked potential (SSEP) monitoring for the early detection of nerve compromise during external fixation procedures for limb lengthening and correction of deformity. Significant SSEP changes were identified during 58 procedures (19%). In 32 instances (10.5%) the changes were transient, and resolved once the surgical cause had been removed. The remaining 26 (8.5%) were analysed in two groups, depending on whether or not corrective action had been performed in response to critical changes in the SSEP recordings. In 16 cases in which no corrective action was taken, 13 (81.2%, 4.2% overall) developed a post-operative neurological deficit, six of which were permanent and seven temporary, persisting for five to 18 months. In the ten procedures in which corrective action was taken, four patients (40%, 1.3% overall) had a temporary (one to eight months) post-operative neuropathy and six had no deficit.

After appropriate intervention in response to SSEP changes, the incidence and severity of neurological deficits were significantly reduced, with no cases of permanent neuropathy. SSEP monitoring showed 100% sensitivity and 91% specificity for the detection of nerve injury during external fixation. It is an excellent diagnostic technique for identifying nerve lesions when they are still highly reversible.

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.

Septicaemia resulting from meningococcal infection is a devastating illness affecting children. Those who survive can develop late orthopaedic sequelae from growth plate arrests, with resultant complex deformities. Our aim in this study was to review the case histories of a series of patients with late orthopaedic sequelae, all treated by the senior author (CFB). We also describe a treatment strategy to address the multiple deformities that may occur in these patients.

Between 1997 and 2009, ten patients (seven girls and three boys) were treated for late orthopaedic sequelae following meningococcal septicaemia. All had involvement of the lower limbs, and one also had involvement of the upper limbs. Each patient had a median of three operations (one to nine). Methods of treatment included a combination of angular deformity correction, limb lengthening and epiphysiodesis. All patients were skeletally mature at the final follow-up. One patient with bilateral below-knee amputations had satisfactory correction of her right amputation stump deformity, and has complete ablation of both her proximal tibial growth plates. In eight patients length discrepancy in the lower limb was corrected to within 1 cm, with normalisation of the mechanical axis of the lower limb.

Meningococcal septicaemia can lead to late orthopaedic sequelae due to growth plate arrests. Central growth plate arrests lead to limb-length discrepancy and the need for lengthening procedures, and peripheral growth plate arrests lead to angular deformities requiring corrective osteotomies and ablation of the damaged physis. In addition, limb amputations may be necessary and there may be altered growth of the stump requiring further surgery. Long-term follow-up of these patients is essential to recognise and treat any recurrence of deformity.

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.

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.