The Journal of Bone and Joint Surgery (American) 83:1643-1649 (2001)
© 2001 The Journal of Bone and Joint Surgery, Inc.
Dominant Endosteal Hyperostosis
Skeletal Characteristics and Review of the Literature
Rodney K. Beals, MD,
Sean W. McLoughlin, PhD,
Ronald L. Teed, MD and
Clark McDonald, MD
Investigation performed at the Department of Orthopaedics and
Rehabilitation, Oregon Health Sciences University, Portland, Oregon
Rodney K. Beals, MD
Sean W. McLoughlin, PhD
Ronald L. Teed, MD
Clark McDonald, MD
Department of Orthopaedics and Rehabilitation, Oregon Health
Sciences University, 3181 S.W. Sam Jackson Park Road, L477, Portland,
OR 97201-3098
No benefits in any form have been received or will be received from
a commercial party related directly or indirectly to the subject
of this article. No funds were received in support of this study.
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Introduction
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A family with dominant endosteal hyperostosis was described by
the senior one of us (R.K.B.) in 19761.
The propositus was a sixty-four-year-old man who had a cemented
total hip replacement for the treatment of arthritis that had developed
after a traumatic hip dislocation without a fracture. The finding
of widespread increased density of the skeleton led to the evaluation
of multiple family members. The pedigree demonstrated four generations
with affected members. Eight members were examined clinically and
radiographically. The affected individuals among the remainder were
identified on the basis of family reports as the facial features
of individuals with the disorder are quite distinctive. These features
are not present until early adulthood so it was not possible to
determine with certainty the affected members in the most recent
generation. Evaluation of the first three generations indicated
that eleven of the twenty-five offspring of affected parents were
affected, and the . . . [Full Text of this Article]
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