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

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%)

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.

The aim of this study was to determine the effectiveness of intra-operative tranexamic acid in children with osteogenesis imperfecta, who have been shown to have increased bleeding tendency, in deformity correction surgery. We retrospectively reviewed all cases of lower limb intramedullary rodding in patients with osteogenesis imperfecta treated in our unit from 2000–2013 in whom a pre and post- operative full blood count was available (n=69). Case notes were reviewed and patients were grouped according to the use of tranexamic acid. Peri-operative change in haemoglobin (Hb), haematocrit (HCT) and requirement for blood transfusion was assessed. Of 69 operations performed, 62.3% were in female patients, 78.3% were femoral, and 43.5% were bilateral cases (19 femur, 11 tibia). In the non-tranexamic acid group there was a mean drop in Hb of 28.9 g/L (range 0–62), mean HCT drop of 8.8 (range 2.2–19.4) and 3 patients required red cell transfusion. In the tranexamic acid group there was a mean Hb drop of 22.5 g/L (range 1–49), mean HCT drop of 7.35 (range −0.8–16.7) and one patient required red cell transfusion. There was a significant decrease in Hb drop (p=0.0287) in the tranexamic acid group. Tranexamic acid seems to decrease the drop in haemoglobin during lower limb intramedullary rodding in patients with osteogenesis imperfecta, with little associated risk. Protocols should be established for future use a further review undertaken

The aim was to assess the long-term impact of humeral and forearm rodding on functional ability, grip strength, joint range of motion and angular deformity in children with osteogenesis imperfecta. A retrospective chart review was conducted on 57 children with osteogenesis imperfecta who underwent humeral rodding or forearm rodding at our institution between 1996 and 2013. Functional ability was assessed using the self-care and mobility domains of the Pediatric Evaluation and Disability Inventory (PEDI). Grip strength was measured using a dynamometer and joint range of motion with a goniometer. Deformity was measured on radiographs of the humerus or forearm. Outcomes were assessed pre-operatively and every year post-operatively. Differences between pre-operative and 1-year post-operative outcomes were compared using paired T-tests. In 44 patients with a minimum of 2 years follow-up, outcome measures at 1-year post-surgery were compared to those at the latest clinic visit (mean follow-up = 8.0 years). Humeral and forearm rodding resulted in a significant improvement in PEDI self-care score (mean change =5.75, p=0.028 for the humerus, mean change = 6.77, p=0.0017 for the forearm) and mobility score (mean change =3.59, p=0.008 for the humerus, mean change =7.21, p=0.020 for the forearm) at 1 year post-surgery. Grip strength improved following forearm rodding (mean change = +6.13N, p=0.015) but not humeral rodding. Joint range of movement improved following humeral rodding but not forearm rodding. There was a significant improvement in radiographic angular deformity of the forearm and humerus following surgery (p<0.0001). Over 80% of improvements were maintained in the long-term. Humeral and forearm rodding in children with osteogenesis imperfecta leads to long-term improvement in functional ability and angular deformity

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

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.

Background. Unicameral bone cysts (UBCs) are difficult to treat and have a high recurrence rate. Their pathogenesis is unknown making targeted therapies difficult. Attributed causes include venous and interstitial fluid obstruction, oxygen free radicals, lysosomal enzymes, prostaglandins and genetic factors. Skeletal stem cells (SSCs) are osteoblast precursors critical to bone formation and cyst fluid may influence their growth, however the association between SSCs and cyst fluid has never been investigated. Aim. To investigate the effect of UBC fluid on SSC growth. Methods. Fluid was aspirated from a UBC in the proximal femur of a nine year old boy and centrifuged to isolate the acellular supernatant. SSCs were harvested from bone marrow of a haematologically normal adult and cultured with graded concentrations of cyst fluid in culture media (0,10,25,50%). Cell growth was assessed by alkaline phosphatase staining, and cytokine levels in the fluid were measured. Results. High levels of cytokines known to be chemo-attractive for cells of the of macrophage-monocyte lineage were found, including Macrophage Chemotactic Protein-1 (1853pg/ml), Monokine Induced by γ-interferon (656pg/ml), Macrophage Inflammatory Protein (MIP)-1α (401pg/ml) and MIP-1β (34pg/ml) suggesting a role of osteoclasts in UBC pathogenesis. Furthermore, SSC growth in vitro was reduced in cyst fluid in a concentration dependent manner. Conclusion. This is the first time altered SSC and osteoprogenitor function has been associated with the fluid of a UBC. A negative effect on osteogenesis was demonstrated, the precise mechanisms of which are under investigation, and macrophage-monocyte chemokines suggest high osteoclast activity. This study has indicated a role of the cyst fluid in limiting osteogenesis and bone turnover, which may explain the high failure rate for current interventions. More patients are needed to validate these findings

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

Aim. The use of intraoperative cell salvage as a tool for reducing allogenic transfusion has been demonstrated in pelvic osteotomies. The aims of this audit were to identify any problems or complications with cell salvage, reduction in allogenic transfusion and identify procedures that would benefit. Methods. The use of cell salvage and allogenic transfusions were prospectively recorded over a 27-month period for all those who had major non spinal surgery looking at whether cell-salvage reduced allogenic transfusions and where cell salvage was used it was matched to procedure, diagnosis and age with cases where it was not used over the same time period. Results. Cell salvage was used in 61 cases. For these, average blood loss was 624mls and re-transfused volume 176mls (range=0-888mls). There were no complications. 4 problems occurred, 2 where suction became desterilised and 2 with insufficient sample to process. Of those that were matched, 3/55 cases required allogenic transfusion versus 11/55 that did not have cell salvage (p=0.03). Sub group analysis according to procedure did not reach significance. Excluding those with osteogenesis imperfecta, no isolated femoral osteotomy required allogenic transfusion (total number=48). Conclusion. Overall its use has reduced the number of children receiving allogenic blood and negates the need to cross match preoperatively. Group and save sample is probably sufficient for most major paediatric orthopaedic surgery with cell salvage. The specific indications for cell salvage have not been identified by this study, though useful in OI

The aim of this retrospective study was to review the outcome of patients treated with Fassier-Duval (FD) rods and highlight some of the complications found during treatment. Between April 2006 and August 2010 we inserted 24 FD rods in 13 patients. 17 rods for osteogenesis imperfecta (OI), 2 for fractures and deformity associated with cerebral palsy, 1 for fracture associated with muscular dystrophy, 1 for fibrous dysplasia and 3 for centralisation of single bone forearms. In the upper limb one patient required revision for proximal migration of the male component and another patient is waiting for revision for the same problem. In the lower limb, a tibial nail was revised because of proximal migration of the male component. A femoral nail was adjusted because of loss of the proximal fixation. One of the OI patients fell, fractured the femur and bent a femoral nail. This awaits revision at a later date. A second OI patient fell on 2 separate occasions bending both a tibial and a femoral nail respectively. These were both revised to trigen intramedullary nails. In all the other cases there were no complications. In summary the Fassier Duval system provides a versatile way of providing intramedullary stabilisation for growing bones through a single entry point. However in our experience we have a 33% complication rate most notably bending of the rods. We advocate careful patient selection and using as high a diameter nail as is feasible

Aim. Since 2008 we have been using third generation Fassier Duval (FD) telescopic rods for children with complex and severe osteogenesis imperfecta (sOI). We present our experience with the technique. Method. 8 children with sOI have undergone rodding of 16 lower limb long bones; 6 tibial, 10 femoral. 3 were revision procedures, exchanging previous Sheffield rods that had been implanted with the female portion only. Indications were recurrent fracture and/or progressive deformity. Age range and follow up are 2–12 and 0.4–4.75 years respectively. All but one of the procedures were performed by a single surgeon. Results. There have been 3 re-fractures at 3–5 years with bending of the implant at the rod-tip interface and one re-fracture around a well-positioned implant without bending, all requiring revision. Some female femoral rods have migrated distally (3/10) leading in one case to re-fracture above the implant, which was also revised. All rods have initially telescoped well but we have experienced high pull out rates of the threads in the distal tibia (3/6) at an average 18 months post implantation (range 14–20 months). None has yet required revision for this. Proximal migration of the femoral female component has not occurred except in one revision case, which responded well to interference wire fixation. We have one case of coxa vara that developed in a child whose femur was rodded at 2 years of age. Conclusion. The FD implants have functioned well and enabled us to avoid opening the ankle joint for tibial roddings. Nonetheless the re-fracture rates remain high in this group of severely affected children and are comparable to older generation implants. Proximal femoral and distal tibial anchoring points remain common sites of failure though rarely require early re-operation

Aim. A retrospective study was undertaken to evaluate and compare the results of telescoping (group I) versus non-telescoping rods (group II) in the treatment of osteogenesis imperfecta. Materials and Methods. Thirty-three lower limb segments in ten patients were studied (14 segments in group I and 19 segments in group II). The surgical techniques of Sofield and Miller (1959) and Lang-Stevenson and Sharrard (1984) for rod insertion were used. All cases were assessed clinically regarding mobility status, growth and limb-length, refracture, and infection. They were also assessed radiologically regarding rod migration, bone outgrowing the rod, incorrect T-piece placement, breakage and bending of rods. Results. The average duration of follow-up was 86.2 months (range : 6 to 8 years). Mobility status and bone growth were better in group I than in group II patients. The overall implant related complication rate was 28.6% in group I in comparison to 68.4% in group II. Rod migration was twice more common in group II than in group I. Bone outgrowing the rod and breakage of rods with fracture was seen in group II only. The three-year survival rate for telescoping rods was 92.9% in contrast to 68.4% for non-telescoping rods. The reoperation rate was 7.2% in group I and 31.6% in group II. Conclusions. From this comparative study it was clear that the results were significantly better after Sheffield rods with regard to mobility status, longevity of the rod, and the frequency of complications requiring reoperations. Also most of the complications were related to the technique of rod insertion and the type of rod

Aim. Paediatric fractures are common but those occurring in non-ambulant children are associated with higher rates of Non Accidental Injury (NAI). There is little published on the mechanisms of injury associated with accidental fracture in young children. This study explores the aetiology of long bone fractures in non-ambulant children. Methods. This retrospective multicentre study looked at children aged ≤18 months presenting to three hospitals over 3 years (2009 to 2011). Information was gathered on age, gender, fracture type, injury mechanism, final diagnosis, treatment and details of screening for NAI. Results. 147 children were identified who were ≤18 months old (mean 12 months). There were 32 femoral, 37 tibial, 43 forearm, 17 humeral, 16 clavicular and 3 fibular fractures. We identified 6 confirmed cases of NAI and 7 pathological fractures (osteopenia of prematurity or osteogenesis imperfecta). 5/64 children aged ≤12 months old had NAI compared with 1/83 in those aged >12 months. All 7 pathological fractures occurred in the ≤12 months group. NAI or pathological fracture was more likely in ≤12 months group compared to those >12 months (p=0.0002) Of the 12 children with no clear mechanism of injury, 5 had NAI and 3 had pathological fractures. In 39/147 children NAI was considered in the documentation and 29 had a paediatric review. Falls from beds and change mats were more common in ≤12 months group, as well as transverse femoral fractures; caused when those carrying the child slipped downstairs and applied a sudden bending force to the held leg. In those >12 months falls from chairs, down steps, in playgrounds or on trampolines were more common. 12/147 fractures were caused directly by other children (6 in each group). Conclusion. Our study identified causes of accidental long-bone fracture in non-ambulant children. In cases where there is no clear mechanism of injury, NAI must be carefully excluded

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.

Cubitus varus is the most frequent complication following the treatment of supracondylar humeral fractures in children. We investigated data from publications reporting on the surgical management of cubitus varus found in electronic searches of Ovid/MEDLINE and Cochrane Library databases. In 894 children from 40 included studies, the mean age at initial injury was 5.7 years (3 to 8.6) and 9.8 years (4 to 15.7) at the time of secondary correction. The four osteotomy techniques were classified as lateral closing wedge, dome, complex (multiplanar) and distraction osteogenesis. A mean angular correction of 27.6º (18.5° to 37.0°) was achieved across all classes of osteotomy. The meta-analytical summary estimate for overall rate of good to excellent results was 87.8% (95% CI 84.4 to 91.2). No technique was shown to significantly affect the surgical outcome, and the risk of complications across all osteotomy classes was 14.5% (95% CI 10.6 to 18.5). Nerve palsies occurred in 2.53% of cases (95% CI 1.4 to 3.6), although 78.4% were transient. No one technique was found to be statistically safer or more effective than any other.

Cite this article: Bone Joint J 2014;96-B:691–700.

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 analysed the operative technique, morbidity and functional outcome of osteotomy and plate fixation for malunited fractures of the forearm sustained in childhood.

A total of 20 consecutive patients underwent corrective osteotomy of 21 malunited fractures at a mean age of 12 years (4 to 25). The mean time between the injury and the osteotomy was 30 months (2 to 140).

After removal of the plate, one patient suffered transient dysaesthesia of the superficial radial nerve. The mean gain in the range of movement was 85° (20° to 140°). The interval between injury and osteotomy, and the age at osteotomy significantly influenced the functional outcome (p = 0.011 and p = 0.004, respectively).

Malunited fractures of the forearm sustained in childhood can be adequately treated by osteotomy and plate fixation with excellent functional results and minimal complications. In the case of established malunion it is advisable to perform corrective osteotomy without delay.

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.