Orthopedics - Oleksa A.P. 2006

Pathophysiology of Joints
Malformations and Congenital Anomalies of the Skeleton
Multiple Osteocartilaginous Exostoses (Osteochondrodysplasia)

The disorder also exhibits an Autosomal dominant inheritance pattern, meaning it belongs to the group of skeletal dysplasias and is characterized by multiple osteochondromas (osteocartilaginous exostoses) arising from the juxta-epiphyseal region of long bones (Shapiro F., Simon S., Glimcher M.J., 1979). These exostoses develop in bones that contain hyaline Cartilage (Fig. 52). The growth of such bones is driven by The activity of the epiphyseal growth cartilage. Malignant transformation of the soft-tissue cap covering the osteochondroma is a potential risk. As the osteochondroma grows, it can cause both longitudinal and angular bone deformations, accompanied by corresponding displacements of tendons, nerves, and Blood Vessels, which also presents a cosmetic defect. In such cases, paired bones (such as the radius and ulna, or the Tibia and Fibula) exhibit asymmetric growth, leading to pathological Changes in the ankle and wrist joints. Surgical intervention remains the only effective method to correct these deformities (Snnearly W.N., Peterson H.A., 1989).

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Fig. 52. Osteochondromas (Crawford and David, 1990).