hereditary multiple exostoses (diaphyseal aclasis)

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Introduction

Hereditary disorder characterized by exostoses arising from bony cortex deforming the metaphyseal region of some long bones.

Pathology

Genetics

Clinical manifestations

  • bilateral involvement, but not symmetric
  • ulna many be shortened, resulting in ulnar deviation of the hand
  • valgus deformities of ankle are common
  • mechanical interference in a joint may be only symptom
  • dwarfism may occur

Radiology

exostoses

More general terms

Additional terms

References

  1. DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 623
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 2274

Database