THE SPECTRUM OF COSTOVERTEBRAL DYSOSTOSIS

Costovertebral dysostosis is a rare developmental defect encompassing combined segmental anomalies of the spine and ribs. Classification in the literature is confusing. These rare developmental defects have bee n described as dysplasia, dysostosis or Jarcho-Levin Syndrome (Type I or II). We report on five Saudi cases diagnosed on their initial radio graphs. Age ranged from a newborn to 45 years. Three had predominantly skeletal (spondylocostal) anomalies with normal life expectancy. Two had skeletal (spondylothoracic) anomalies with severe associations, Th e severity of costovertebral dysostosis is determined by the type of a ssociated anomalies. Prognosis of costovertebral dysostosis is not rel ated to the mode of transmission. Sporadic cases are common. Although consanguinity is common in the Middle East, there have been only few r eports on Yemenite consanguinous families. No previous reports from Sa udi Arabia. Costovertebral dysostosis is possibly under diagnosed. The purpose of this paper is to report the wide spectrum of radiologic fi ndings in costovertebral dysostosis and to discuss the variety of its clinical presentations.