Pediatrics Skeletal Dysplasia

Description: GENERAL PRINCIPLES OF SPINAL DEFORMITIES IN THE SKELETAL DYSPLASIAS What is skeletal dysplasia? The term skeletal dysplasia refers to a "bad formation" of the bones and joints. All the bones of the skeleton are affected: this differs from a skeletal dysostosis, in which a group of bones are affected while the remainder of the skeleton is normal. Patients with skeletal dysplasia are usually, though not always, of small stature. The small stature is disproportionate: the different parts of the skeleton are affected to differing degrees. For example, the trunk may be smaller than the limbs, and within the limbs, the bones of the forearm may be more deformed than the bones of the hands, which may be relatively spared. Skeletal dysplasia is a general term that includes many distinct syndromes. The most common non-lethal form is achondroplasia, while the most common potentially lethal form is osteogenesis imperfecta. These and the other principal types that characteristically affect the spine will be discussed below.