Surgical treatment of scoliosis in a rare disease: arthrogryposis

<p>Abstract</p> <p>Background</p> <p>The reported incidence of scoliosis in arthrogryposis varies from 30% to 67% and, in most cases, the curves progress rapidly and become stiff from early age.</p> <p>The authors report six cases of scoliosis in arthrogryposis to assess the role of surgical treatment.</p> <p>Methods</p> <p>Six cases (3 males, 3 females; mean age at surgery 13.2 years) with arthrogryposis multiplex congenita associated with the characteristic amyoplasia were reviewed: they were operated on for scoliosis at the authors' Spine Surgery Department between 1987 and 2008.</p> <p>Surgery was performed using the Harrington-Luque instrumentation (2 cases), the Luque system (1), a hybrid segmental technique with hooks and screws (1) and spinal anchoring with pedicle screws (2).</p> <p>Results</p> <p>The patients were clinically and radiologically reviewed at a mean follow-up of 4.2 years, ± 2.7 (range, 1 to 9 years). Three minor postoperative complications were encountered; a long-term pulmonary complication was seen in one case after reintervention and was successfully resolved after 10 days. Surgery was successful in the other 5 cases, where solid arthrodesis was achieved and no significant curve progression was observed at follow-up.</p> <p>Conclusions</p> <p>The experience acquired with the present case series leads the authors to assert that prompt action should be taken when treating such aggressive forms of scoliosis. In case of mild spinal deformities in arthrogryposis, brace treatment should be attempted, the evolution of the curves being unpredictable; however, when the curve exceeds 40° and presents with marked hyperkyphosis, hyperlordosis or pelvic obliquity, surgery should not be delayed.</p

Paediatric Orthopaedic Surgery with 3D Printing: Improvements and Cost Reduction

This paper presents a a novel alghorithm of diagnosis and treatment of rigid flatfoot due to tarsal coalition. It introduces a workflow based on 3D printed models, that ensures more efficiency, not only by reducing costs and time, but also by improving procedures in the preoperative clinical phase. Since this paper concerns the development of a new methodology that integrates both engineering and medical fields, it highlights symmetry. An economic comparison is made between the traditional method and the innovative one; the results demonstrate a reduction in costs with the latter. The current, traditional method faces critical issues in diagnosing the pathologies of a limb (such as the foot) and taking decisions for further treatment of the same limb. The proposed alternative methodology thus uses new technologies that are part of the traditional workflow, only replacing the most obsolete ones. In fact, it is increasingly becoming necessary to introduce new technologies in orthopedics, as in other areas of medicine, to offer improved healthcare services for patients. Similar clinical treatments can be performed using the aforementioned technologies, offering greater effectiveness, more simplicity of approach, shorter times, and lower costs. An important technology that fits into this proposed methodology is 3D printing

CT Conversion Workflow for Intraoperative Usage of Bony Models: From DICOM Data to 3D Printed Models

This paper presents the application of a low-cost 3D printing technology in pre-operative planning and intra-operative decision-making. Starting from Computed Tomography (CT) scans, we were able to reconstruct a 3D model of the area of interest with a very simple and rapid workflow, using open-source software and an entry level 3D printer. The use of High Temperature Poly-Lactic Acid (HTPLA) by ProtoPasta allowed fabricating sterilizable models, which could be used within the surgical field. We believe that our method is an appealing alternative to high-end commercial products, being superior for cost and speed of production. It could be advantageous especially for small and less affluent hospitals that could produce customized sterilizable tools with little investment and high versatility

Neurofibromatosis type I and multiple myeloma coexistence: A possible link?

The association between Neurofibromatosis type I (NF1) and multiple myeloma (MM), a plasma cell, dyscrasia is very rare. Here we put to the attention of the scientific community two new cases. The first one is a patient with active MM whereas the second with smoldering MM. Both patients present typical features of NF1 but skeletal alterations were present only in the second case including dysplasia, marked scoliosis and osteoporosis. MM osteolytic lesions were absent in both patients. In addition to the clinical diagnosis of NF1, a molecular testing for NF1 gene mutations has been performed finding that patient one was heterozygous for the c.6855C>A (Tyr2285Ter) mutation, while patient two was heterozygous for the c.7838dupC (Lys2614GlufsTer20) mutation. The two mutations were diagnosed both in genomic DNA from peripheral blood and from MM cells. The potential link between NF1 mutation and the increased risk of MM is discussed in the report