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

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

Aim. To determine the preferable treatment for congenital pseudarthrosis of the tibia, we retrospectively reviewed 19 patients (20 limbs) treated consecutively over a 22 year period (1988–2007). Fifteen were followed up to maturity. The patients were assessed for union, leg length discrepancy (LLD), ankle valgus, range of ankle movement and distal tibial physeal injury. Results. The median age at surgery was 3 years. At surgery nineteen of the tibiae had a dysplastic constriction with a fracture (Crawford II-C or Boyd II) lesion. To obtain union in the 20 tibiae, 29 procedures were done. Nine failed primarily and required a second procedure to obtain union. Older patients (≥ 5 years) had a significantly higher success rate. Excision, intramedullary rodding and bone graft (IMR) was done in 14 tibiae: 10 (71.4%) were successful. Six of 10 primary operations and all 4 secondary operations after a previous failed procedure were successful. Ipsilateral vascularized fibula transfer (IVFT) was successful in 5 tibiae (3 primary and 2 secondary). Ilizarov with bone transport only, failed in two patients. Ilizarov with excision, intramedullary rodding and bone graft with lengthening was successful in 2 of 5 cases (40%); two sustained fractures at the proximal lengthening site. A median leg length discrepancy (LLD) of 3 cms occurred post surgery which was treated with contralateral epiphysiodesis. At maturity 3 patients had a LLD of ≥ 2cms. Six limbs had ankle valgus and were treated with stapling and tibio-fibular syndesmosis. Decreased range of movement of the ankle (< 50%) occurred in 7 patients. Distal tibial physeal injury occurred in 4 patients and was associated with repeated rodding. Conclusion. We concluded that surgery should be delayed as long as possible. If there is adequate tibial purchase for the rod distally, IMR is the best option. If purchase is inadequate, Ilizarov with rodding will avoid ankle stiffness. Epiphysiodesis is preferable to lengthening because of the risk of fracture above the rod. IVFT is a good option as a secondary procedure. NO DISCLOSURES

It is generally accepted that children treated for congenital pseudarthrosis of the tibia (CPT) should be followed-up until skeletal maturity, before drawing conclusions about the efficacy of treatment. We undertook this study in order to evaluate the long-term results of treatment of CPT by excision of the pseudarthrosis, intramedullary rodding and onlay cortical bone grafting. Among a total of 46 children with CPT treated by a single surgeon during a 20-year period, 38 had been treated by this technique and 11 of these children have reached skeletal maturity. These eleven cases (nine boys and two girls) formed the basis for this study. The mean age at presentation was 3.1 years (range 0.4–7 years); the mean age at index surgery was 3.2 years (range 0.7–7 years). The mean age at follow-up was 18.4 years (range 16–21.6 years) with a mean interval between surgery and final follow-up of 15.2 years (range 12.8–17.4 years). In all 11 children bone graft was harvested from the contralateral tibial diaphysis. Rods passed from the heel were used in nine children and in two Sheffield telescopic rods were passed from the ankle into the tibia. The fibula was divided in three children to ensure that the tibial fragments were in good contact before placing the graft astride them; the fibula was not touched in the remaining eight instances. To ensure that the intramedullary rod supported the pseudarthrosis site till skeletal maturity, revision rodding was performed as needed when the tip of the rod receded into the distal third. A thermoplastic clamshell orthosis was used till skeletal maturity. At final follow-up the union at the pseudarthrosis site was deemed to be ‘sound’ only if two independent observers concurred that there was definite bony continuity of the cortices on both the anteroposterior and lateral radiographs. Deformities of the tibia and ankle and ranges of motion of the knee, ankle and subtalar joints were noted. The limb lengths were measured with scanograms. The morbidity at the bone graft donor site was recorded. The function of the ankle was assessed by applying the American Orthopaedic Foot and Ankle Society (AOFAS) Ankle-Hind foot Score. Primary union of the tibial pseudarthrosis was achieved in nine of 11 cases with a mean time to union of 6.1 months. Secondary union was achieved in the remaining two cases following further intervention. At final follow-up sound union of the tibial pseudarthrosis was noted in all eleven patients but persistent pseudarthrosis of the fibula was present in 10 of 11 cases. The lateral malleolus was proximally situated in six cases. Ten of eleven children underwent a total of 21 secondary operations on an average of 2.6 years (range 0.5–5.1 years) after initial union was achieved. Six re-fractures were encountered in five patients at a mean of 6.1 years after index surgery. All the re-fractures united following the single episode of intervention. The overall mean shortening at final follow-up was 2.6 cm. At final follow-up, five patients had ankle valgus greater than 10 degrees. All the 11 patients walked without pain. Only two patients had significant motion at the ankle. Despite the ankle stiffness in the remaining children the AOFAS ankle-hindfoot scores ranged between 70 and 98 (mean 83.3). Our long-term results are comparable to the results of other studies in terms of the rate of union, the re-fracture rate, limb length discrepancy, residual deformity and the frequency of surgery

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.