Endochrmatosis
From WikiLectures
The term enchondromatosis refers to multiple enchondromas (islands of cartilaginous tissue in the metaphyses of tubular bones) that retard growth and deform the bone.
Ollier's disease (Lous Léopold Ollier (1830-1900), French surgeon) - affecting ½ of the body
Maffucci syndrome (Angelo Maffuccini (1847-1903), Italian pathologist) - enchodromatosis + multiple hemangiomas (i.e. hemangiomatosis)
Clinical picture[edit | edit source]
- affected long bones shortened and bent, enlarged metaphyses
- fingers enlarged and deformed in the hands, pseudomadelung's deformity on the forearm (see Madelung's deformity), genu valgum
X-ray image[edit | edit source]
- puffy lucencies, calcified stripes running from the metaphysis to the diaphysis
Therapy[edit | edit source]
- removal of symptomatic enchondromas, spongioplasty
- corrective osteotomy and bone lengthening
- dispensation (risk of malignancy)
Links[edit | edit source]
Related articles[edit | edit source]
- Achondroplasia
- Thanatophoric dwarfism
- Diastrophic dysplasia
- Spondyloepiphyseal dysplasia
- Larsen's syndrome
- Congenital multiple exostoses
- Fibrous bone dysplasia
- Neurofibromatosis
- Dysostosis cleidocranialis
- Apert syndrome
- Osteogenesis imperfecta
- Morbus Albers-Schönberg
- Osteopoikilosis
- Arthrogryposis multiplex congenita
- Marfan syndrome
- Mucopolysaccharidosis
- Hereditary osteoonychodysplasia (nail-patella syndrome)
Used literature[edit | edit source]
- DUNGL, P., et al. Ortopedie. 1. vydání. Praha : Grada Publishing, 2005. ISBN 80-247-0550-8.
- Wikipedia. Ollier Disease [online]. ©2006. Poslední revize 2009, [cit. 2009]. <https://en.wikipedia.org/wiki/Ollier_disease>.
