Spondyloepiphyseal dysplasia congenita (SEDC) is an autosomal dominant disorder, characterized by disproportionate dwarfism with short spine, short neck associated with variable degrees of coxa vara. Cervical cord compression is the most hazardous skeletal deformity in patients with SEDC which requires special attention and management.Ten patients with the clinical and the radiographic phenotypes of spondyloepiphyseal dysplasia congenita have been recognized and the genotype was compatible with single base substitutions, deletions or duplication of part of the COL2A1 gene (6 patients out of ten have been sequenced). Cervical spine radiographs showed apparent atlantoaxial instability in correlation with odontoid hypoplasia or os-odontoideum.Instability of 8 mm or more and or the presence of symptoms of myelopathy were the main indications for surgery. Posterior cervical fusion from the occiput or C1–3, decompression of C1–2 and application of autorib transfer followed by halo vest immobilization have been applied accordingly.Orthopedic management of children with spondyloepiphyseal dysplasia congenita (SEDC) should begin with the cervical spine to avoid serious neurological deficits and or mortality.
Study Design:Technical note.Objectives:The objective of this study is to check out safety and rationality of standardized and fast tricks to select trajectory of subaxial cervical pedicle screw (SCPS) insertion, based on simple angles to bony landmarks.Materials and Methods:Stage 1 – Computed tomography (CT)-morphometric analysis of C3–C7 vertebrae of ten patients with cervical degenerative diseases. Stage 2 – SCPS insertion in 6 cadavers, according to the developed technique (59 pedicle screws). Stage 3 – SCPS insertion in 6 patients, according to the developed technique (32 pedicle screws).Results:CT-morphometric analysis showed that the average length of C3–C7 pedicle channels was 32 mm, the average angle between a pedicle axis and an axis of contralateral lamina - 180°, the average angle between a pedicle axis and plane of a posterior surface of a lateral mass amounted to 90° and the coordinates of an optimal entry point – 2 mm from a lateral edge and 2 mm from an upper edge of the lateral mass posterior surface. During the cadaveric study, 39 screws had a satisfactory position (66.1%), 7 screws permissible (11.9%), and 13 screws unacceptable (22%). During the clinical study, 26 screws (81.25%) had satisfactory position, 4 (12.5%) had permissible position, and 2 (6.25%) unacceptable position.Conclusion:Developed and clinically approved a method for simplicity SCPS insertion is relatively safe and cheap. No doubt, it requires further investigation, but the results of primary analysis allow us to recommend it to wide practical application.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.