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Journal of Bone and Joint Surgery, 1973;55:461-474.
© 1973 by The Journal of Bone and Joint Surgery, Inc


Progressive Diaphyseal Dysplasia

REVIEW OF THE LITERATURE AND REPORT OF SEVEN CASES IN ONE FAMILY

JAMES D. HUNDLEY M.D.1 and FRANK C. WILSON M.D.1

1 From the Division of Orthopaedic Surgery, University of North Carolina School of Medicine, Chapel Hill

Seven cases of progressive diaphyseal dysplasia in three generations of one family were observed and data presented. The affected grandfather had six children, all but one of whom are described as documented cases, the other being probably affected. One of the grandchildren is documented as a definite case and three of thirteen other grandchildren probably were affected. The characteristic features of the dysplasia are the roentgenographic appearance of bilaterally symmetrical diaphyseal cortical thickening and autosomal dominant inheritance.


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