Ollier's Disease (Multiple Enchondromatosis)

Skip to end of metadata
Go to start of metadata

Background

  • Inborn error of osseous development with respect to endochondral ossification
  • Lesions generally involve metaphysis and diaphysis
  • Often a predominant unilateral distribution
  • No hereditary or familial tendency

Clinical

  • Presentation: Knobby swellings of the digits or gross discrepancy of length and axial alignment of extremities
  • Frequently diagnosed in childhood and adolescence.
  • M:F = 1:1
  • Lesion affecting growth plate causes angular and rotational deformities and foreshortening of limbs

Imaging

  • Plain films demonstrate features of multiple enchondromatosis
  • Often expansion of overlying cortex
  • Lesions innocuous in infancy and early childhood; become more clinically worrisome with skeletal growth, causing deformity and growth disturbance
  • Development of destructive bony changes and soft tissue mass with pain suggest malignant transformation
  • Malignant changes may present simultaneously in several lesions with differing levels of activity

Pathology

Histologically, lesions similar to solitary enchondroma, but may be more cellular

Complications

  • Risk of malignant degeneration to chondrosarcoma difficult to state with confidence; rare disease with no good longitudinal studies of patients at risk
    • However, risk of developing malignancy may be as high as 25% over the patient's lifetime
  • The most common malignant transformation is to low- or medium-grade chondrosarcoma
Unknown macro: {corr}
Unknown macro: {springerlink}
Unknown macro: {panel}

Internet resources validated by OrthopaedicWebLinks.com

Unknown macro: {cache}
Unknown macro: {report-on}
Error formatting macro: rss: java.lang.IllegalArgumentException: Invalid uri 'http://www.orthopaedicweblinks.com/cgi-bin/owl/search.cgi?query=%content:title > url encode%&xml_feed=1': Invalid query
Unknown macro: {panel-related}
Unknown macro: {rate}
Cite this page
Unknown macro: {div}

. *

Unknown macro: {page-info}

*. _

Unknown macro: {builder-spacetitle}

. _In: OrthopaedicsOne - The Orthopaedic Knowledge Network. Created

Unknown macro: {page-info}

. Last modified

Unknown macro: {page-info}

ver.

Unknown macro: {page-info}

. Retrieved

Unknown macro: {report-info}

, from

Unknown macro: {page-info}

.

Unknown macro: {builder-hide}
Page contributions
Unknown macro: {div}

The following individuals have contributed to this page:

Unknown macro: {contributors-summary}