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Radiology, Vol 175, 761-766, Copyright © 1990 by Radiological Society of North America

ARTICLES

Spondylometaphyseal dysplasia, corner fracture type: a heritable condition associated with coxa vara

LO Langer Jr, PW Brill, MB Ozonoff, RM Pauli, WG Wilson, BA Alford, H Pavlov and DG Drake
Department of Radiology, University of Minnesota Hospital and Clinic, Minneapolis.

The authors present the radiographic features of a previously incompletely delineated bone dysplasia, which they call spondylometaphyseal dysplasia, corner fracture type. This is a dominant heritable condition associated with short stature and developmental coxa vara. The progressive hip deformity usually causes significant disability requiring surgical correction. Developmental coxa vara, simulated corner fractures of long tubular bones, and vertebral body abnormalities result in a diagnostic constellation. Knowledge of these distinctive radiologic features allows accurate diagnosis, which in turn should lead to appropriate genetic counseling and possibly to earlier, more efficacious surgical treatment of the coxa vara.




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