Synovial Chondromatosis, The Alfred I. duPont Institute

Classification of Dysplasias

What is the meaning? The word “Diastrophic” is derived from Greek meaning crooked or distorted. Lamy and Maroteaux, who coined the term in 1960, differentiated it from other forms of short-stature by its characteristic features including severe growth restriction, involvement of the spine, clubfeet and thumb deformity. Variations of the dysplasia include taller, less severely afflicted patients, who nevertheless have features resembling classic diastrophic dysplasia. Following an international consensus conference, the term diastrophic dwarfism is no longer used.

Radiology teaching site from UCLA

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.

Pediatrics: Skeletal Dysplasia 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. UCSF Department of Orthopaedic Surgery

Diastrophic Dysplasia

Multiple Epiphyseal Dysplasia

SPONDYLO-EPI-METAPHYSEAL DYSPLASIA WITH JOINT LAXITY

Heterotopic Bone Formation, The Alfred I. duPont Institute

Multiple Epiphyseal Dysplasia, The Alfred I. duPont Institute

DIASTROPHIC DYSPLASIA

Polyostotic Fibrous Dysplasia, The Alfred I. duPont Institute

Robert Mellors, 1999 Cornell University. Notes and illustrations

Cedars Sinai Website.