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AN UNUSUAL BONE DYSPLASIA: PARASTREMMATIC DWARFISM

LEONARD O. LANGER 1, DIETRICH PETERSEN 2, and JURGEN SPRANGER 3

1 Clinical Associate Professor, Department of Radiology, University of Minnesota Medical School, Minneapolis, Minnesota.
2 Orthopedic Department, Annastift, Hannover, Germany.
3 Department of Pediatrics, University of Kiel, Germany.

The term parastremmatic dwarfism is suggested for a bone dysplasia characterized by severe dwarfism, kyphoscoliosis, distortion and bowing of the extremities and contractures of the large joints.

Roentgenographically the disease is characterized by a combination of decreased bone density, bowing of the long bones, platyspondyly and striking irregularities of endochondral ossification with areas of calcific stippling and streaking in radiolucent epiphyses, metaphyses and apophyses.

The disease is inherited as a dominant trait and must be differentiated from other bone dysplasias with similar structural bone changes or kyphoscoliosis, such as the Jansen type of metaphyseal dysostosis, Morquio’s disease, diastrophic dwarfism and metatropic dwarfism.


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