Dysplasia : Abnormal Tissue Development
THE CHONDROOSTEODYSTROPHIESThe chondro-osteodystrophies, or skeletal dysplasias,
are a large group of disorders characterized by abnormal
cartilage and bone growth.
the conditions are presented here in clinical
rather than etiological groups, as follows:
• those with predominantly epiphyseal changes
• those with predominantly physeal and metaphyseal
changes
• those with mainly diaphyseal changes; and
• those with a mixture of abnormalities.
DYSPLASIAS WITH PREDOMINANTLYEPIPHYSEAL CHANGES
MULTIPLE EPIPHYSEAL DYSPLASIADYSPLASIAS WITH PREDOMINANTLYPHYSEAL AND METAPHYSEALCHANGES
HEREDITARY MULTIPLE EXOSTOSIS(DIAPHYSEAL ACLASIS)
DYSPLASIAS WITH PREDOMINANTLYDIAPHYSEAL CHANGES
OSTEOPETROSIS
Marble bones Despite the remarkable density, the bones break easily
CONNECTIVE TISSUEDISORDERS
OSTEOGENESIS IMPERFECTA (BRITTLEBONES)
Osteogenesis imperfecta (OI) is one of the commonest
of the genetic disorders of bone,
Abnormal synthesis and
structural defects of type I collagen result in abnormalities
of the bones, teeth, ligaments, sclerae and
skin. The defining clinical features are (1) osteopenia,
(2) liability to fracture, (3) laxity of ligaments, (4)
blue coloration of the sclerae and (5) dentinogenesis
imperfecta (‘crumbling teeth’)
Osteogenesis imperfecta (a) Moderately severe
(type IV) disease. These deformities can be corrected bymultiple osteotomies and ‘rodding’
LIMB ANOMALIES