Aims. Congenital pseudarthrosis of the tibia (CPT) has traditionally been a difficult condition to treat, with high complication rates, including nonunion, refractures, malalignment, and leg length discrepancy. Surgical approaches to treatment of CPT include intramedullary rodding, external fixation, combined intramedullary rodding and external fixation, vascularized fibular graft, and most recently cross-union. The current study aims to compare the outcomes and complication rates of cross-union versus other surgical approaches as an index surgery for the management of CPT. Our hypothesis was that a good index surgery for CPT achieves union and minimizes complications such as refractures and limb length discrepancy. Methods. A multicentre study was conducted involving two institutions in Singapore and China. All patients with CPT who were surgically managed between January 2009 and December 2021 were included. The patients were divided based on their index surgery. Group 1 included patients who underwent excision of hamartoma, cross-union of the tibia and fibula, autogenic iliac bone grafting, and internal fixation for their index surgery. Group 2 included patients who underwent all other surgical procedures for their index surgery, including excision of hamartoma, intramedullary rodding, and/or external fixation, without cross-union of the tibia and fibula. Comparisons of the rates of union, refracture, limb length discrepancy, reoperations, and other complications were performed between the two groups. Results. A total of 36 patients were included in the study. Group 1 comprised 13 patients, while Group 2 comprised 23 patients. The mean age at index surgery was four years (1 to 13). The mean duration of follow-up was 4.85 years (1.75 to 14). All patients in Group 1 achieved bony union at a mean of three months (1.5 to 4), but ten of 23 patients in Group 2 had nonunion of the pseudarthrosis (p = 0.006). None of the patients in Group 1 had a refracture, while seven of 13 patients who achieved bony union in Group 2 suffered a refracture (p = 0.005). None of the patients in Group 1 had a limb length discrepancy of more than 2 cm, while ten of 23 patients in Group 2 have a limb length discrepancy of more than 2 cm (p = 0.006). In Group 1, four of 13 patients had a complication, while 16 of 23 patients in Group 2 had a complication (p = 0.004). Excluding removal of implants, four of 13 patients in Group 1 had to undergo additional surgery, while 18 of 23 patients in Group 2 had to undergo additional surgery following the index surgery (p = 0.011). Conclusion. A good index surgery of excision of hamartoma, cross-union of the tibia and fibula, autogenic iliac bone grafting, and internal fixation for CPT achieves union and minimizes complications such as refractures, limb length discrepancy, and need for additional surgeries

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

Forty cases of congenital pseudarthrosis of the tibia were reviewed. The results were assessed so as to emphasise function rather than simply the presence or absence of union. At the time of review, 14 patients had undergone amputation; eight others had non-union or tenuous union. No surgical procedure except the Farmer operation (a composite skin and bone pedicle graft from the other leg) showed any clear superiority. Among the factors associated with a poor result were considerable shortening, older children, and rapid resorption of the bone graft. It is felt that congenital pseudarthrosis of the tibia is a biological problem and not merely a mechanical one; consequently biological approaches to its treatment are needed

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

We describe 11 patients with congenital pseudarthrosis of the tibia treated by a free vascularised fibular graft (FVFG) and followed up from 10 to 64 months (mean 38). Bony union was achieved in nine of the 11 cases: two failures required amputation. The mean time for union in the successful cases was five months. Nine of the 11 patients had had an average of four surgical procedures before the FVFG, so the graft was a salvage procedure for which the only alternative was amputation. FVFG is recommended as a primary procedure for the treatment of congenital pseudarthrosis of the tibia if there is a large tibial defect (over 3 cm) or shortening of more than 5 cm. The primary use of this operation is not advised for cases in which standard orthopaedic procedures are expected to succeed. For a small defect with a favourable prognosis (Boyd and Sage 1958), we recommend conventional bone grafting, intramedullary nailing and electrical stimulation

Congenital pseudarthrosis of the tibia remains one of the most difficult orthopaedic problems. We describe early excision and the use of the Masquelet technique to reconstruct the bone defect in a child aged 14 months. Consolidation sufficient for complete weight-bearing was achieved by seven weeks. After two and a half years, the child was asymptomatic with a fully reconstructed tibia and no leg-length discrepancy

Five patients with Boyd type II congenital pseudarthrosis of the tibia underwent excision of the pseudarthrosis and double onlay bone grafting. Stability was maintained by extending intramedullary rods. Clinical union was achieved in all cases at a mean of 8.6 months (range six to 11). The rods extended by 15.7% (range 2% to 31.4%) as growth occurred. One rod was removed because of infection and a vascularised free fibular graft was subsequently performed. The extending rods provided stability while union occurred and did not require revision as the legs grew. The rods can be removed easily and have not jeopardized further surgical options

Five cases of congenital pseudarthrosis of the tibia successfully treated by a free vascularised fibular graft are described. Follow-up ranged from 5 to 34 months with a mean of 17.5 months. The technique, which includes radical excision of abnormal bone and soft tissue around the pseudarthrosis, also permits primary bone lengthening, and correction of deformity. The early results indicate that satisfactory bony union is achieved in a relatively short period of time

Five children with congenital pseudarthrosis of the tibia treated by free vascularised fibular grafts were followed up until skeletal maturity. The ipsilateral fibula was used in four cases, the contralateral fibula in one. All our cases achieved bone union, but leg length discrepancy, atrophy of the foot and ankle stiffness were frequent complications, due perhaps to the many previous operations. Vascularised fibular grafting might achieve better results if it were done as the primary procedure

We describe a 13-year-old boy with atrophic tibial pseudarthrosis associated with neurofibromatosis who had undergone nine unsuccessful operations. Eventually, union was obtained by the use of bone morphogenetic protein 7 in conjunction with intramedullary stabilisation and autologous bone graft.

A treatment regime using electrical stimulation in association with a variety of surgical procedures has improved the prognosis in congenital pseudarthrosis of the tibia--one of the most challenging of all orthopaedic disorders. The technique consists of correction of the tibial deformity, intramedullary fixation and cancellous bone grafting, augmented by electrical stimulation using an implanted bone-growth stimulator. Experience with 27 pseudarthroses in 25 patients is presented; of those, 20 have joined. The cases have been reviewed and the causes of failure analysed. These results offer encouragement to the orthopaedic surgeon treating this difficult condition.

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 (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

Aims

As an alternative to external fixators, intramedullary lengthening nails (ILNs) can be employed for distraction osteogenesis. While previous studies have demonstrated that typical complications of external devices, such as soft-tissue tethering, and pin site infection can be avoided with ILNs, there is a lack of studies that exclusively investigated tibial distraction osteogenesis with motorized ILNs inserted via an antegrade approach.

The case histories of two brothers with congenital defects of the tibia and neurofibromatosis are presented. This report of congenital pseudarthrosis of the tibia in siblings is believed to be the first in the English literature

The results and complications of 104 vascularised fibular grafts in 102 patients are presented. Bony union was ultimately achieved in 97 patients, with primary union in 84 (84%). The mean time to union was 15.5 weeks (8 to 40). In 13 patients, primary union was achieved at one end of the fibula and secondary union at the other end. In these patients, the mean time to union was 31.1 weeks (24 to 40). Five patients failed to achieve union, with a resultant pseudarthrosis (3 patients) or amputation (2 patients). There were various complications. Immediate thrombosis occurred in 14 cases. In two of 23 patients with osteomyelitis, infection recurred at two and six months after surgery, respectively. Both patients had active osteomyelitis less than one month before the operation. Bony infection occurred in a patient with a synovial sarcoma of the forearm one year after surgery. In 15 patients, 19 fractures of the fibular graft occurred after bony union, all except one within one year after union. In patients in whom an external fixator had been used, fracture occurred soon after its removal. Union was difficult to achieve in cases of congenital pseudarthrosis of the tibia. Appropriate alignment of the fibular graft is an important factor in preventing stress fracture. The vascularised fibula should be protected during the first year after union. Postoperative complications at the donor site included transient palsy of the superficial peroneal nerve in three patients, contracture of flexor hallucis longus in two and valgus deformity of the ankle in three. Vascularised fibular grafts are useful in the reconstruction of massive bony defects. We believe that meticulous preoperative planning, including choosing which vessels to select in the recipient and the type of fixation devices to use, and care in the introduction of the vascularised fibula, can improve the results and prevent complications

Aims

To clarify the effectiveness of the induced membrane technique (IMT) using beta-tricalcium phosphate (β-TCP) for reconstruction of segmental bone defects by evaluating clinical and radiological outcomes, and the effect of defect size and operated site on surgical outcomes.

Aims

Guided growth using eight-plates is commonly used for correction of angular limb deformities in growing children. The principle is of tethering at the physeal periphery while enabling growth in the rest of the physis. The method is also applied for epiphysiodesis to correct limb-length discrepancy (LLD). Concerns have been raised regarding the potential of this method to create an epiphyseal deformity. However, this has not been investigated. The purpose of this study was to detect and quantify the occurrence of deformities in the proximal tibial epiphysis following treatment with eight-plates.

Pathological fractures in children can occur as a result of a variety of conditions, ranging from metabolic diseases and infection to tumours. Fractures through benign and malignant bone tumours should be recognised and managed appropriately by the treating orthopaedic surgeon. The most common benign bone tumours that cause pathological fractures in children are unicameral bone cysts, aneurysmal bone cysts, non-ossifying fibromas and fibrous dysplasia. Although pathological fractures through a primary bone malignancy are rare, these should be recognised quickly in order to achieve better outcomes. A thorough history, physical examination and review of plain radiographs are crucial to determine the cause and guide treatment. In most benign cases the fracture will heal and the lesion can be addressed at the time of the fracture, or after the fracture is healed. A step-wise and multidisciplinary approach is necessary in caring for paediatric patients with malignancies. Pathological fractures do not have to be treated by amputation; these fractures can heal and limb salvage can be performed when indicated.