Enchondromatosen der Hand

Abstract

In the introduction the authors report on the suspected theories of origin of the enchondromas: some believe that they are the result of disintegration of islands of cartilage from the epiphyseal growth cartilage, while in the opinion of others they are brought about by disturbances of blood supply in the metaphyseal vessels during enchondral growth. No evidence of heredity has yet been established. Surgical treatment and late results are described taking the course of disease of a patient who is now 33 years old as an example. This patient had grotesque tumors on both hands; the tumors not only disfigured the limbs but also constituted a functional handicap. Surgery was indicated for both esthetic and functional reasons. For large tumors, with destruction of the substantia corticalis or regions close to the joint, cross-section or segmental resection is given as the therapy of choice, and if possible segmental resection is to be preferred, filling the defect with loosely emplaced autologous spongiosa. After this procedure the hand does not have to be immobilized for as long as after a cross-section. In this patient, after a total of 16 procedures on both hands, there have been no recurrences in the ten years of the follow-up period. The same applies to two other patients with multiple enchondromas (which were admittedly less severe) who were followed up between 3 and just under 8 years after surgery. It is stressed that growing tumors which disturb the function or appearance of the hand should be surgically treated in childhood, but that by no means every enchondroma seen in enchondromatosis requires such therapy.