Clinical problems in multiple osteochondroma |
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Authors: | Ham S J John de Lange Jan van der Zwan Arnard L Schaap Gerard R van der Woude Henk-Jan Heeg Minne |
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Affiliation: | Onze Lieve Vrouwe Gasthuis, afd. Orthopedie, Amsterdam, the Netherlands. s.j.ham@olvg.nl |
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Abstract: | Multiple osteochondroma, also known as hereditary multiple exostoses, is a relatively rare genetic disorder characterized by the presence of multiple osteochondromas. The disease is frequently painful, with restriction of the activities of daily living, problems with carrying out an occupation and performance at school. In addition, characteristic skeletal deformities and postural abnormalities of the joints very frequently occur in patients with this disorder. Malignant transformation of osteochondroma to chondrosarcoma occurs in 1-5% of the patients with multiple osteochondroma. Treatment of patients with multiple osteochondromas must be tuned to the problems experienced by the patient. Symptomatic osteochondromas are often an indication for excision; knowledge of the natural progression of the abnormality is important in this. Periodical screening is essential: in children to prevent or correct deformity and postural abnormalities and in adults to detect and treat malignant transformation of osteochondroma at an early stage. |
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