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Multiple hereditary osteochondromas (Diaphyseal achalasia)

By Dr.Pavan Kumar In MSK Posted October 15, 2018

Patient History : Not specified
Gender:Not specified
Age:

Multiple hereditary osteochondromas (Diaphyseal achalasia).

Location

  • Any bone with enchondral ossification can be involved
  • Metaphysis of long bones: 70% , knee almost always involved. 
Imaging Findings

Radiography

  • Cartilage covered bony projection (exostosis) on external surface of bone.
    • Calcification of hyaline cartilage cap.
  • Pedunculated type: Slender pedicle directed away from joint.
    • Lesion grows at right angles to long axis of host bone.
  • Sessile type: Broad-based attachment to cortex
  • Undertubulation of long bones (Erlenmeyer flask deformity)
  • Malignant degeneration
    • Development of thick, bulky, cartilaginous cap (thickness > 1 cm by CT, > 2 cm by MRI).
    • Dispersed calcifications within cartilaginous cap.
    • Development of soft tissue mass.

CT Findings

  • To demonstrate continuity of cortical and medullary portions of lesion with host bone.
  • To demonstrate thickness of cartilaginous cap.
 

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