%0 Journal Article
%T Ischiopubic and odontoid synchondrosis in a boy with progressive pseudorheumatoid chondrodysplasia
%A Ali Al Kaissi
%A Farid Ben Chehida
%A Maher Ben Ghachem
%A Franz Grill
%A Klaus Klaushofer
%J Pediatric Rheumatology
%D 2007
%I BioMed Central
%R 10.1186/1546-0096-5-19
%X Detailed clinical and radiological examinations were undertaken with emphasis on the usefulness of 3D-CT scanning.There was synchondrosis between the odontoid and the body of the axis and the cephalad part of the odontoid was detached. Bilateral ischiopubic ossification defects and ischiopubic and odontoid synchondroses were additional abnormalities. 3D-CT scan showed an orthotopic type of os odontoideum associated with an occult axial fracture.Children who are younger than seven years of age are predisposed to develop odontoid fracture. The latter occur because of the presence of physiological odontoid synchondrosis, but fractures can result from trivial injuries as well as from high-energy trauma. The persistence of an infantile odontoid, with a large pre-adulthood head in children with skeletal dysplasias, is a major risk factor for sudden death or significant morbidity. Comprehensive orthopaedic management must follow early identification of these malformations.Osteochondrodysplasias are a large heterogeneous group of genetic skeletal dysplasias. Skeletal dysplasias are diagnosed and classified by their clinical phenotype, radiographic features, and their genetic pattern of inheritance [1]Spranger et al. first described progressive pseudorheumatoid chondrodyspalsia (PPRC) as a progressive connective tissue disease, which combined the radiological features of Scheuermann's disease, with radiographic features of juvenile rheumatoid arthritis [1-3]. The disorder is classified as an autosomal, recessively inherited chondrodyspalsia, with absence of inflammatory parameters. Differentiating progressive pseudorheumatoid chondrodyspalsia (PPRC) from Juvenile rheumatoid arthritis (JRA) is important for Rheumatologists, radiologists and clinicians to further assess and estimate the prognosis and treatment of this generalised bone-cartilage dysplasia syndrome.In addition our patient had a divided odontoid process, in which the cephalad part was detached from its base, and
%U http://www.ped-rheum.com/content/5/1/19