Enchondromatosis



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)

Related articles

 * 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
 * Mukopolysacharidosis
 * Hereditary osteonychodysplasia (nail-patella syndrome)