Enchondromatosis

Enchondromas of the distal part of the radius..

By the term enchondromatosis we mean the multiple occurrence of enchondromas (islands of cartilage tissue in the metaphyses of tubular bones), which slow down growth and deform the bone.

  • Ollier's disease' (Lous Léopold Ollier (1830-1900), French surgeon) – half body affected
  • Maffucci syndromem (Angelo Maffuccini (1847-1903), Italian pathologist) – enchodromatosis + multiple hemangiomas (i.e. hemangiomatosis)

Clinical picture

  • affected long bones shortened and bent, extended metaphyses
  • when the hands are affected, the fingers are extended and deformed, on the forearm pseudomadelung's deformity (see Madelungs deformity), genu valgum

X-ray image

  • cloudy clearing, calcified strips going from the metaphysis to the diaphysis

Therapy

  • removal of symptomatic enchondromas, spongioplasty
  • corrective osteotomy and bone prolongation
  • dispensary (risk of malignancy)


Refernces

  • DUNGL, P.. Ortopedie. 1. edition. Praha : Grada Publishing, 2005. ISBN 80-247-0550-8.