The aim of the study was to review the effectiveness of rigid IM nailing in stabilisation and deformity correction of lower limb long bones in adolescents with metabolic bone disease which to our knowledge has not been studied before. Medical records and radiographs were retrospectively reviewed looking at indications, deformity correction, number of osteotomies-if needed, bone healing, time to healing and incidence of complications. Between Aug 2010 and Mar 2015 fifteen patients (24 segments) had rigid IM nailing. Ten patients had Osteogenesis Imperfecta, four with McCune Albright syndrome and one with hypophosphatemic rickets. 22 femora and two tibiae were IM nailed. The mean age of the patients was 13.1 (9.6–16.75 years). Eleven out 24 segments were previously rodded. Eight segments were for acute fractures. 13 bones had significant deformities requiring corrective osteotomies. One patient had previous fracture non union. All patients were allowed to partial weight bear immediately postoperatively and were fully mobile six weeks following surgery. Mean follow up was 24 months (3–51 months) post-operatively. All deformities were corrected. All fractures and osteotomies radiologically united. Mean radiological union time was 5.5 months (6 weeks – 11 months). Patients with acute fractures had mean radiological union time of 4 months. Patients who had osteotomies had a mean radiological union time of 7.1 months. The patient with previous non union had BMP at the same time and radiologically healed in 10 months. Two patients had persistent bisphosphonate osteotomy lines but were asymptomatic. One patient had removal of a prominent distal locking screw and one had persistent Trendelenburg gait. Rigid intramedullary nailing is effective in stabilisation and deformity correction of long bones in adolescent patients with brittle bone disease. The technique has a low complication rate. We recommend the use of this technique in paediatric limb reconstruction in managing metabolic bone conditions

Purpose of the study. We report on the clinical, radiographic and functional outcomes after salvage arthrodesis for complex ankle and hindfoot problems the Portsmouth experience with the Ilizarov ring fixator. Methods and results. We report on 10 patients who underwent ankle and hindfoot (tibio-calcaneal) arthrodeses using an Ilizarov ring fixator between 2006 and 2010. The indications included failed fusion after primary arthrodesis, sepsis complicating internal fixation of fractures, talar avascular necrosis and failed total ankle arthroplasty (TAR). All patients had undergone multiple previous surgeries which had failed. There were 7 males and 3 females in this group. Average age of the patients was 60 (47 years–77 years) Mean follow up was 32 months (6–56 months) BMP 2 (Inductos) was used in three cases. The procedure was combined with a proximal corticotomy and lengthening in 2 patients who had undergone a talectomy and tibio-calcaneal fusion. There were no major complications apart from minor pin site infections requiring oral antibiotics. There were no deep infections, thromo-embolic issues, CRPS, or functional problems on account of limb shortening. Patients were assessed clinically, radiologically and using functional outcome scores EQ50 and AOFAS. Solid arthrodesis was achieved in all but one patient who was subsequently revised with a hindfoot nail. All patients were satisfied with their overall improvement in pain and function. Conclusion. We conclude that this is an effective salvage technique for complex ankle and hindfoot problems in patients with impaired healing potential, insufficient bone stock and progressive deformity

Aim. To study the results of using recombinant human bone morphogenic protein-2 (rhBMP-2) to achieve bone union in children with complex orthopaedic problems including skeletal dysplasia, congenital limb deficiencies and complex trauma. Materials & Methods. Between November 2006 to April 2010, rhBMP-2 was used to achieve bone healing in a total of 19 children. The indications were delayed union or non-union in 16 long bones where previous surgeries had failed and 4 posterior spinal fusions. All children had underlying complex orthopaedic problems predisposing them to delayed bone healing. The average age at the time of surgery was 13 years and 8 months (Range: From 4 years to 19 years and 4 months). There were 7 boys and 12 girls. Results. Average follow-up period was 9.6 months (Range: From 3 months to 2 years and 2 months). Out of the 16 long bone non-unions treated with rhBMP-2, 13 united without further interventions but 3 required further intervention. The average time for clinical union was 10 weeks (Range: from 6 weeks to 18 weeks). The average time for radiological union was 15 weeks (Range: from 7 weeks to 27 weeks). Two children who underwent instrumented posterior spinal fusion and two children who underwent uninstrumented spinal fusion achieved radiologically evident spinal fusion at an average of 16 weeks. No local or systemic complications attributable to BMP were noted in any of the children. Conclusions. rhBMP-2 can be used successfully to achieve bone healing in long bone nonunion and delayed union and posterior spinal fusion in children with complex orthopaedic problems including skeletal dysplasia, congenital limb deficiencies and complex trauma. Its use in children has shown no unwanted effects in the short term. Significance. rhBMP-2 has been approved by Food and Drug Administration (FDA) as a bone graft substitute in adults. Its use in adults has been widely reported but there are only a few case reports of its use in children. This study shows that rhBMP-2 can be safely

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.

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.

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.