Hereditary multiple exostosis

Hereditary multiple exostosis is an autosomal dominantly inherited disorder that occurs in childhood and early adolescence. It arises from endochondrally formed bones and is associated with some shortening and angular deformities of the bones. Approximately 10% to 20% of the patients have malignant degeneration of the lesions. Treatment consists of local resection for symptomatic lesions and radical surgery for chondrosarcomatous degeneration.