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

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

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

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

Introduction. Patient-reported outcome measures (PROMs) has gained momentum in the orthopaedic literature. The GOAL-LD (Gait Outcomes Assessment List for Limb Deformity) incorporates the framework of The WHO International Classification of Functioning, Disability and Health and correlates highly with the Paediatric Outcomes Data Collection Instrument (PODCI) when applied in a limb reconstruction setting. The Royal Children's Hospital Melbourne, Australia, supported by The University of Melbourne is designing and implementing a Limb Reconstruction Registry, prospectively enrolling patients with a number of key conditions and provide a mechanism for capturing surgical data and PROMs at regular follow-up intervals. LimbDREAM (The Limb Reconstruction Registry of Experience, Aspiration and Measures) has begun recruiting participants, and this paper serves as a narrative review of our design and implementation process. Materials & Methods. After provision of a research grant, audit was conducted to examine local case mix and estimate recruitment based on conditions selected. Literature review was focussed on (i) registries in the paediatric limb reconstruction domain and (ii) use of PROMs across conditions selected. A high-level process-flow chart was constructed in order to inform governance and staffing requirements in addition to REDCap Database structure. Local as well as scalable deployment was considered. Alpha and beta testing was performed by principal and associate investigators prior to participant recruitment. Results. Audit identified 6 condition streams for inclusion: Congenital limb deficiencies, congenital pseudarthrosis of the tibia, bone dysplasias, bone tumours, amputation, and complex deformity of other etiology. Literature review identified 20 PROMs used across these conditions, with limited validation studies and significant implementation heterogeneity. REDCap database design took longer than anticipated (planned 10 months, actual 18 months) before being recruitment ready, due to the complexity of ensuring data collection logic would work across highly diverse patient journeys. Initial recruitment across all streams has been promising. Email and text message reminders have proved helpful in increasing survey return rates. Conclusions. Prospective collection of PROMs as well as surgical data via a standardized dataset will provide the basis for future condition-specific research, with the ability to support nested trials. Implementation requires forethought with regard to time frames and staffing for non-automated steps as well as data integrity review. The next phase of the LimbDREAM project will be to integrate use of the Registry into our weekly Limb Reconstruction Meeting to ensure that operative plans and data are captured close to the point of care

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

Introduction. This is a report of the outcome of management of congenital pseudoarthrosis of the tibia (CPT) at skeletal maturity. Materials and Methods. Retrospective study. Inclusion criteria:. CPT Crawford IV. Skeletally maturity. Availability of radiographs and medical records. Outcome: union rate, healing time, residual deformities, ablation and refracture. Results. 23 patients who reached maturity were analysed. Time to union was 7.6 months. Union rate 70%. External fixation group: 7 patients, age 6.1 years, all united, 1 needed Bone graft. Average union time 8.2 months, no residual mal-alignment, no amputations, 2/7 needed corrective osteotomies and residual LLD in 2/7 < 1 cm. Numbers of surgery was 3. Vascularized fibular graft was done in 3 cases; all had failed previous attempts. Union time was 7 months. 2/3 united, 1 had amputation due to extensive disease. All patients had residual mal-alignment. Rodding group included 13 patients, age 3.2 years, union rate 61%, union time 8.3 months. Average LLD 1 cm with 1 patient LLD > 2 cm. Residual knee mal-alignment in 2/13, 4 had procurvatum and 55% of patients had ankle valgus. Fibula pseudoarthrosis. Refracture was reported in 53% of the rodding group. The causes of refracture were mal-alignment in 3, traumatic in 2 and idiopathic in 2. Total surgeries no 5. 2 patients had amputations after an unsuccessful 1. st. attempt. Our current trend of treatment was applied on 8 patients. They are not skeletally mature yet. the treatment combined excision of hamartoma, tibial rodding, wrapped periosteal graft with/out neutralization frame, and fibular fixation. Average age 3.8 years, union time was 10 months, Union rate 80%, no residual deformities. Conclusions. Our study shows the evolution of the treatment of CPT with increasing union rate, fewer residual deformities, and numbers of surgeries with more recent techniques

Aims: The purpose of this study is to to assess the surgical results, complications, and long-term results of vascularized fibula in the treatment of congenital pseudarthrosis of the tibia. Methods: Seven patients who had congenital pseudarthrosis of the tibia were treated consecutively at our clinic between 1992 and 2000 with free vascularized fibular graft. There were four females and three males. The mean age at the time of operation averaged 6.5 years (range 1–12 years). Four left tibias and 3 right tibias were involved. Stability was maintained with internal fixation in four patients, external fixation in two patients and intramedullary pin in one patient. Results: The average follow-up was 2.6 years (range 6 months to 8 years). In five patients, both ends of the graft healed primarily within 2.7 months (range 1.5 to 3 months), and hypertrophy of the fibular graft occurred rapidly with a well-formed medullary canal. In one patient the distal junction did not unite and although required three subsequent operations still not healed. Stress fracture occurred in one patient underwent four additional operations before union achieved. Conclusions: Despiting the continuing problems and the relatively high complication rate, the ultimate results with free vascularized fibula transplant are generally good specially as compared with published series in whom conventional grafting techniques had failed. However, even achieving union of pseudarthrosis is not enough for the resolution of this disease and is only half of the problem; the other half is to maintaining

This paper reviews the outcome of 13 children with congenital pseudarthrosis of the tibia after intramedullary rodding and autogenous bone grafting. The oldest patient was aged nine years at the time of surgery. The ages of the others ranged from 12 to 24 months. The oldest patient at follow-up was 18 years. All 13 had bone defect and angulation. Ten children had clinical features of neurofibromatosis. Ten had pseudarthrosis involving the distal third of the tibia, two the middle third and one the proximal third. Autogenous iliac crest chips were used following excision of fibrous tissue and dense and atrophic bone. Rodding was done across the ankle joint in 10 patients. Postoperatively an above-knee cast was applied for 6 to 12 months, after which an above-knee brace was used to protect the rodding. At follow-up, which ranged from 10 months to 16 years after surgery, all patients were fully weight-bearing and ambulant. Three patients were lost to follow-up after 2 to 4 years. Complications included refracture and rod breakage (two), rod migration (three), and growth retardation with shortening of up to 5 cm. Ten patients had ankle and subtalar joint stiffness and two had valgus deformities of the ankle. Three patients underwent repeat rodding and bone grafting. Radiological union was observed to be progressing in all patients. Intramedullary rodding of the tibia for congenital pseudarthrosis of the tibia is a simple procedure and can be repeated. It avoids prolonged hospital stay and permits early weight-bearing. Careful supervision is necessary, and until there are signs of good bony union, external support is mandatory

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 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