Introduction

The use of vascularised fibula grafts is an accepted method for reconstructing the distal femur following resection of malignant childhood tumours. Limitations relate to the mismatch of the cross-sectional area of the transplanted fibula graft and thel ocal bone, instability of the construct and union difficulties. We present midterm results of a unique staged technique—an immediate defect reconstruction using a double-barrel vascularised fibula graft set in in A-frame configuration and a subsequent intramedullary femoral lengthening.

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.

Introduction: The management of deformities of the lower extremity in children with spina bifida is challenging. Surgery is fraught with high complication and recurrence rates. The Ilizarov technique has shown to be a successful tool in the management of complex lower limb deformity. There are few published series in the literature dealing specifically with Ilizarov correction of complex lower limb deformities in spina bifida. In this paper we present our experience of the Ilizarov technique for complex deformities in children with spina bifida.

Methods: From 1989 to 2006, 33 patients with spina bifida underwent 48 corrections of their lower extremity deformity using the Ilizarov technique at one of three tertiary care centres (Royal Children’s Hospital, Melbourne, Australia, Musgrave Park Hospital, Belfast, Northern Ireland and Birmingham Children’s Hospital, Birmingham, UK). Notes were reviewed retrospectively. Patient demographics, indications for surgery, deformity analysis and the extent of surgery were recorded. Complications of the surgery and outcomes were noted.

Results: Group I comprised of 12 fixed knee flexion deformities and one unstable neuropathic joint. Group II comprised 15 external tibial rotational deformities. Group III comprised 20 complex foot deformities. The mean age of the patients was 12.1 years (5.2–20.6 years). Prior to their treatment using the Ilizarov technique patients had undergone a mean of 1.6 previous surgeries (range: 0–5) on the affected limb. The mean duration of treatment in the frame was 9.3 weeks (range: 2–26 weeks). The mean follow-up is 4.0 years (range 0.3–9.0 years). Thirteen problems occurred in the 48 procedures (27.1%). Five obstacles occurred in the 48 procedures (10.4%). Thirteen complications occurred in the 48 procedures (27.1%). Further surgical procedures are either planned, or have been performed, in 8 of the 33 patients (24.2%). Following treatment, all feet were recorded to be plantigrade. All were comfortable in their respective orthoses where appropriate.

Conclusion: Through the pitfalls in treating the complex lower limb deformity in spina bifida, the Ilizarov technique offers a refreshing approach. It offers distinct advantages throughout the treatment period. It modifies both the intraoperative and postoperative environment to address the morbidity of the significant complications associated with the traditional approach to the correction of the limb deformity in the child with spina bifida.

Significance: This is a large series of complex limb reconstruction cases in spina bifida showing excellent results, an acceptable complication rate and recommendations for further use of this technique.

Periacetabular osteotomy (PAO) is a well established method to treat hip dysplasia in the adult. There are, however, a number of complications associated with this procedure as well as a time related deterioration in the grade of osteoarthritis that can influence the long term result. It is essential that patients are fully informed as to the effectiveness of PAO, the likelihood of complications and their influence on the subjective outcome prior to giving consent for surgery. Generic outcome measures offer the opportunity to determine treatment efficacy and the influence on the outcome by complications.

60 PAOs on 50 patients were investigated retrospectively after a mean follow up of 7.4 years. The patients’ self reported assessment of health and function was evaluated by the SF-36 and the WOMAC questionnaires at last follow-up. 40 healthy persons served as a control group.

The centre-edge angle improved from a mean of 8.7° to 31.5°. The weight bearing surface improved from a mean lateral opening of 8.7° to 4.2°. The degree of osteoarthritis improved in one case, remained unchanged in 20 and deteriorated in 17. There was a tendency of higher CE-angles towards a higher rate of deterioration, indicating that overcorrection may increase osteo-arthritic degeneration. 13 of the 60 interventions had no complications. Minor complications occurred in 25 (41%) interventions and in 22 (37%) at least one major complication occurred. SF-36 summary measure was 76.4 for PAO patients and 90.3 for the control group. Mean WOMAC score was 25.1. The severity of ectopic bone formation, incidence of postoperative peroneal nerve dysfunction and delayed wound closure did not influence the subjective result. Patients with major complications had a similar subjective outcome as patients with minor or no complications, but persistent dysesthesia due to lateral femoral cutaneous nerve dysfunction led to a worse subjective function as assessed with the WOMAC score.