Clinically the disease is attended with the gradual and painless development during childhood or adolescence of a number of tumours or irregular projections of bone, at the ends of the long bones, the vertebral border of the scapula, and the cristal border of the ilium. They exhibit a rough symmetry; they rarely attain any size; and they usually cease growing when the skeleton attains maturity—the conversion of cartilage into bone being then completed. While they originate from the ossifying junctions of the long bones, they tend, as the shaft increases in length, to project from the surface of the bone at some distance from the ossifying junction and to “point” away from it. They may cause symptoms by “locking” the adjacent joint or by pressing upon nerve-trunks or blood vessels.
In a considerable proportion of cases, the disturbance of growth is further manifested by dwarfing of the long bones; these are not only deficient in length but are sometimes also curved and misshapen, which accounts for the condition being occasionally confused with the disturbances of growth resulting from rickets. In about one-third of the recorded cases there is a dislocation of the head of the radius on one or on both sides, a result of unequal growth between the bones of the forearm.
Fig. 140.—Multiple Cartilaginous Exostoses in a man æt. 27. The scapular tumour projecting above the right clavicle has taken on active growth and pressed injuriously on the cords of the brachial plexus.
In early adult life, one of the tumours, instead of undergoing ossification, may take on active growth and exhibit the features of a chondro-sarcoma, pressing injuriously upon adjacent structures ([Fig. 140]) and giving rise later to metastases in the lungs.
The X-ray appearances of the bones affected are of a striking character; apart from the outgrowths of bone or “tumours” there is evident a widespread alteration in the internal architecture of the bones, which suggests analogies with other disturbances of ossification such as achondroplasia and osteomyelitis fibrosa. The condition is one that runs in families, sometimes through several generations; we have more than once seen a father and son together in the hospital waiting-room.
As regards treatment, there is no indication for surgical interference except when one or other tumour is a source of disability as by pressing upon a nerve-trunk or by locking a joint, in which case it is easily removed by chiselling through its neck.
