Enchondromatosis
From WikiLectures
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[edit | edit source]
- 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[edit | edit source]
- cloudy clearing, calcified strips going from the metaphysis to the diaphysis
Therapy[edit | edit source]
- removal of symptomatic enchondromas, spongioplasty
- corrective osteotomy and bone prolongation
- dispensary (risk of malignancy)
Links[edit | edit source]
Refernces[edit | edit source]
- DUNGL, P.. Ortopedie. 1. edition. Praha : Grada Publishing, 2005. ISBN 80-247-0550-8.
- Wikipedia. Wikipedia, The Free Encyclopedia : Ollier Disease [online]. ©2006. The last revision 2009, [cit. 2009]. <https://en.wikipedia.org/wiki/Ollier_disease>.
Related articles[edit | edit source]
- Achondroplasia
- Thanatophoric dwarfism
- Diastrophic dysplasia
- Spondyloepiphyseal dysplasia
- Larsen syndrome
- Congenital multiple exostoses
- Fibrous bone dysplasia
- Neurofibromatosis
- Dysostosis cleidocranialis
- Apert syndrome
- Osteogenesis imperfecta
- Morbus Albers-Schönberg
- Osteopoikilosis
- Arthrogryposis multiplex congenita
- Marfan syndrome
- Mucopolysacharidosis
- Hereditary osteonychodysplasia (nail-patella syndrome)
