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 bisphosphonate therapy. Data were collected prospectively, with 1.5 to 11 years follow-up. Results. A total of 24 patients (33%) required exchange of implants (14 femora and ten tibiae) including 11 rods bending with refracture. Four (5%) required reoperation with implant retention. Loss of proximal fixation in the femur and distal fixation in the tibia were common. Four patients developed coxa vara requiring surgical correction. In total, 13 patients experienced further fractures without rod bending; eight required implant revision. There was one deep infection. The five-year survival rate, with rod revision as the endpoint, was 63% (95% confidence interval (CI) 44% to 77%) for femoral rods, with a mean age at implantation of 4.8 years (1.3 to 14.8), and 64% (95% CI 36% to 82%) for tibial rods, with a mean age at implantation of 5.2 years (2.0 to 13.8). Conclusion. FD rods are easier to implant but do not improve on the revision rates reported for second generation T-piece rods. Proximal femoral fixation is problematic in younger children with a partially ossified greater trochanter. Distal tibial fixation typically fails after two years. Future generation implants should address proximal femoral and distal tibial fixation to avoid the majority of complications in this series. Cite this article: Bone Joint J 2020;102-B(8):1048–1055

The Sheffield Expanding Intramedullary Rod System was developed after experiencing problems with existing rod systems in the management of osteogenesis imperfecta. Between 1986 and 1996 we treated 74 bones in the lower limb in 28 children at a median follow-up of 5.25 years. We have reviewed 24 children with a total of 60 rods. Before surgery, all children had had multiple fractures of the lower limb. At review eight patients had experienced no further fractures, but three had suffered five or more subsequently. Before initial stabilisation, 15 children had never walked, and only three (13%) used walking as their main means of mobility. After surgery, half of those who showed motor arrest were able to walk (p = 0.016). The number of patients able to walk, with or without aids, increased to 17 (p = 0.0001). We have experienced no evidence of epiphyseal damage after the procedure, and complication rates requiring rod exchange have been low (7%)

Aims. The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management. Patients and Methods. A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation. Results. Mean preoperative major curves were 76° (34° to 115°) in the skeletal dysplasia group and 75° (51° to 113°) in the idiopathic group (p = 0.55), which were corrected at final follow-up to 49° (13° to 113°) and 46° (12° to 112°; p = 0.68), respectively. T1-S1 height increased by a mean of 36 mm (0 to 105) in the skeletal dysplasia group and 38 mm (7 to 104) in the idiopathic group at the index surgery (p = 0.40), and by 21 mm (1 to 68) and 46 mm (7 to 157), respectively, during the distraction period (p = 0.0085). The skeletal dysplasia group had significantly worse scores in the physical function, daily living, financial impact, and parent satisfaction preoperatively, as well as on financial impact and child satisfaction at final follow-up, than the idiopathic group (all p < 0.05). The domains of the 24-Item Early-Onset Scoliosis Questionnaire (EOSQ24) remained at the same level from preoperative to final follow-up in the skeletal dysplasia group (all p > 0.10). Conclusion. Children with skeletal dysplasia gained significantly less spinal growth during growth-friendly management of their EOS and their health-related quality of life was significantly lower both preoperatively and at final follow-up than in children with idiopathic EOS. Cite this article: Bone Joint J 2019;101-B:1563–1569

Aims

Given the possible radiation damage and inaccuracy of radiological investigations, particularly in children, ultrasound and superb microvascular imaging (SMI) may offer alternative methods of evaluating new bone formation when limb lengthening is undertaken in paediatric patients. The aim of this study was to assess the use of ultrasound combined with SMI in monitoring new bone formation during limb lengthening in children.

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

We report a postal survey of 59 families of children with osteogenesis imperfecta. From the 51 replies we collected data on developmental milestones and walking ability and related them to the Sillence and the Shapiro classifications of osteogenesis imperfecta. Twenty-four of the patients had been treated by intramedullary rodding. Both classifications helped to predict eventual walking ability. We found that independent sitting by the age of ten months was a predictor for the use of walking as the main means of mobility with 76% attaining this. Of the patients who did not achieve sitting by ten months, walking became the main means of mobility in only 18%. The developmental pattern of mobility was similar in the rodded and non-rodded patients

Aims

The objective of this study was to evaluate the clinical and radiological outcomes of patients younger than six months of age with developmental dysplasia of the hip (DDH) managed by either a Pavlik harness or Tübingen hip flexion splint.

We have reviewed the results of the Sofield-Millar operation on 58 long bones in ten patients. If more than three osteotomies were undertaken the time to union of the bone was significantly prolonged (p< 0.001) with significant thinning of the bone (p< 0.02). We have used a modified technique in order to minimise surgical trauma and devascularisation of the bone. The rod is introduced under the control of an image-intensifier. Small surgical exposures are made only at the sites of corrective wedge osteotomies. The number of osteotomies is kept to the minimum

Aims

The treatment of tibial aplasia is controversial. Amputation represents the gold standard with good functional results, but is frequently refused by the families. In these patients, treatment with reconstructive limb salvage can be considered. Due to the complexity of the deformity, this remains challenging and should be staged. The present study evaluated the role of femoro-pedal distraction using a circular external fixator in reconstructive treatment of tibial aplasia. The purpose of femoro-pedal distraction is to realign the limb and achieve soft tissue lengthening to allow subsequent reconstructive surgery.

We have used the Ilizarov technique for severe flexion deformity of the knee in 11 patients (13 knees) between 1986 and 1994 and have followed them up for an average of 4.1 years. The age of the patients at operation ranged from 1.7 to 18.8 years. The femoral and tibial components were connected by two anterior hinges, medial and lateral, and two posterior distraction rods. The deformity was corrected to a femorotibial lateral shaft angle of less than 20°. A permanent orthosis was applied after removal of the fixator. Fractures occurred in four patients and paralysis of the common peroneal nerve in another. There was a recurrence of the deformity in four patients. At the last review all patients were able to walk on their operated leg with or without an orthosis. We have found the Ilizarov method to be helpful in correcting severe fixed flexion deformity of the knee, with relatively few complications, but the basic principles of the method must be carefully followed

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

We aimed to evaluate the health-related quality of life (HRQoL) in children with supracondylar humeral fractures (SCHFs), who were treated following the recommendations of the Paediatric Comprehensive AO Classification, and to assess if HRQoL was associated with AO fracture classification, or fixation with a lateral external fixator compared with closed reduction and percutaneous pinning (CRPP).

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.

Aims

Although atlantoaxial rotatory fixation (AARF) is a common cause of torticollis in children, the diagnosis may be delayed. The condition is characterised by a lack of rotation at the atlantoaxial joint which becomes fixed in a rotated and subluxed position. The management of children with a delayed presentation of this condition is controversial. This is a retrospective study of a group of such children.

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.

Our aim was to review the efficacy of the wound vacuum-assisted closure (VAC) system in the treatment of deep infection after extensive instrumentation and fusion for spinal deformity in children and adolescents.

A total of 14 patients with early deep spinal infection were treated using this technique. Of these, 12 had neuromuscular or syndromic problems. Clinical and laboratory data were reviewed. The mean follow-up was 44 months (24 to 72). All wounds healed. Two patients required plastic surgery to speed up the process. In no patient was the hardware removed and there was no loss of correction or recurrent infection.

We believe that the wound VAC system is a useful tool in the armamentarium of the spinal surgeon dealing with patients susceptible to wound infections, especially those with neuromuscular diseases. It allows for the retention of the instrumentation and the maintenance of spinal correction. It is reliable and easy to use.

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.

We present a retrospective study of 27 patients treated by callus distraction using a unilateral external fixator of our own design for nonunion with bone loss and shortening of the femur caused by suppurative osteomyelitis. The unilateral external fixator was used either alone or in combination with an intramedullary nail. The mean age of the patients was 13.6 years (8 to 18). The fixator was used alone in 13 patients and with an intramedullary nail in 14. The bone results at a mean follow-up of 88 months (37 to 144) were excellent in 16 patients and good in 11.

The functional results were excellent in 18 patients and good in nine. However, four patients still had draining sinuses at the latest follow-up. A residual deformity greater than 7° was present in seven femora, but this did not adversely affect function or require further treatment.

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: Bone Joint J 2013;95-B:1027–34.

We reviewed the long-term radiological outcome, complications and revision operations in 19 children with quadriplegic cerebral palsy and hip dysplasia who underwent combined peri-iliac osteotomy and femoral varus derotation osteotomy. They had a mean age of 7.5 years (1.6 to 10.9) and comprised 22 hip dislocations and subluxations. We also studied the outcome for the contralateral hip. At a mean follow-up of 11.7 years (10 to 15.1) the Melbourne cerebral palsy (CP) hip classification was grade 2 in 16 hips, grade 3 in five, and grade 5 in one. There were five complications seen in four hips (21%, four patients), including one dislocation, one subluxation, one coxa vara with adduction deformity, one subtrochanteric fracture and one infection. A recurrent soft-tissue contracture occurred in five hips and ten required revision surgery.

In pre-adolescent children with quadriplegic cerebral palsy good long-term outcomes can be achieved after reconstruction of the hip; regular follow-up is required.

Cite this article: Bone Joint J 2013;95-B:259–65.