Infection was reported in 15 patients (24.2%): 2 early infections (healed with surgical debridment), 1 femoral stem septic loosening (treated with early revision with cemented stem); in 8 cases removal of the infected APC was required followed by implant of a new prosthetic device after cement spacer; two infections did not healed and patient underwent amputation; in two cases a good functional result was achieved removing the infected graft and covering the proximal tibia with cement and no other surgery was required. Non union of the graft was observed in 8 patients (12.9%): in 4 patients autologous bone grafting was necessary to heal the osteotomy line. In other 3 cases non union was associated with graft fracture. In one case non union was associated with tibial stem loosening and revision of the whole implant was done. Polyethylene wear was assessed in 5 patients (8%) and revision of the polyethylene components was always required. Nine patellar tendon rupture (14.5%) were assessed and repaired was performed in seven cases. The functional outcome of 42 patients with more than two years of follow up was excellent in 25 cases, good in 13, fair in 2 and poor in 2.
Since high levels of urokinase-type plasminogen activation system have been associated with cancer metastasis, purpose of this study was to investigate its expression in patients with giant cell tumor and the relationship with outcome.
Key points for successful fixation are absolute rotational stability and satisfactory circumferential bone-bone contact at the time of surgery. Postoperative regimen consisted of hip, followed by progressive bracing and toe-touch weight-bearing for 6 weeks, weight-bearing.
According to MSTS, results were satisfactory in 90% of the patients, with average score 91% (75%–96%).
Multiple Hereditary Exostoses is a rare skeletal chondrodysplasia characterized by the presence of a variable number of osteochondromas, usually mostly affecting the long bones but possibly located anywhere. Appearance and growth of exostoses is parallel to the patient’s growth, essentially ending when skeletal maturity is reached. Its clinical expression is well known and may vary from asymptomatic to severe deformities and is rarely complicated by trasformation to secondary chondrosarcoma (0.5–2%). Research in the field of genetics has lead to identification of 2 responsible genes, EXT1 and EXT2, located respectively on chromosome 8 and 11, both coding for transmembrane glycoproteins involved in the synthesis of heparan-sulfate chains. A third rare abnormality (EXT3) has been located on chromosome 19 but the responsible gene has not been identified yet. Seems logical to investigate the genetic basis of the disease and the correlation with clinical aspects, either severity of the deformities and consequent functional impairment and potential for chondrosarcoma. At the authors’ Institution a total of 550 patients with Multiple Hereditary Exostoses are presently filed. Genetic screening by DHPLC (Denaturing High Performance Liquid Chromatography) and clinicoradiographic orthopedic evaluation has been carried out on 200 patients. So far, 45 mutations have been identified (35 in EXT1 and 10 in EXT2) in 167 patients, 20 of which presented with negative family history and are therefore considered “de-novo” mutations. Comparison of the clinical data and prospective long term follow-up will possibly clarify different prognosis and risk of secondary chondrosarcoma for different genotypes.
Bone reconstruction in pediatric oncology always has to face two major problems: the frequent unavailability of small prosthetic tools and the difficulty in finding bone allografts of adequate size. Aim of this work is to present the research lines in this field, currently active in our institution to improve the planning and the results of reconstructive tumor surgery in children. Starting from patient’s CT data sets, subject-specific 3D models of bone segments can be created and compared with the similar models obtained by the CT analysis of massive allografts stored in the Rizzoli Bone Bank. In the same time the computer modelling technologies allow the development of three-dimensional environment, where the surgeon can navigate and exploit both artificial (prostheses, metallic plates and screws) or biological tools (bone allografts or autografts). The presented method has been utilized with success in 10 children (mean age 8, range 4–13) that underwent a skeletal reconstruction of the limbs in the last year (proximal humerus 1, diaphyseal humerus 1, total humerus 1, distal radius 1, proximal femur 3, diaphyseal femur 1, proximal tibia 1, diaphyseal tibia
The relationship with adamantinoma remains unclear, follow-up is suggested.
Infection occurred in 7 patients (10%) from 1 to 144 months (median 12 mo): in 6 patients prosthesis removal was needed to achieve healing. Mechanical complications were present in 19 patients (27%): 15 (21%) had prosthetic head instability (5 surgically treated), 2 breakage of the prosthetic stem and 2 prosthetic disassembly.