Cooley's anemia—Orthodontic and surgical treatment

Abstract

T here are some disease entities which have important orthodontic implications but which are rarely seen in the orthodontic practice. Consequently, little attention is paid to them. Nevertheless, it is important to record whatever experience is accumulated regarding orthodontic diagnosis and treatment for such conditions. Cooley’s anemia (thalassemia, erythroblastic anemia, or Mediterranean anemia) is a blood dyscrasia characterized by a peculiar alteration in skull and long bone structure which produces a Mongoloid facial appearance (Figs. 1 and 2). Other clinical manifestations are icterus (jaundice) and enlargement of the spleen and heart. The disease is congenital and affects almost exclusively persons of Mediterranean extraction, chiefly Greek or Italian. In severe cases the maxilla is grossly enlarged and the malar bones bulge markedly. These bony changes are related to the enlargement of marrow spaces in response to a stimulus for increased production of red cells. Radiographically, these patients exhibit enlarged marrow spaces in the jaws and thinning of the cortical layers (Fig. 3). There is a severe retardation of maturation, which is observable in carpal radiographs, and a widening of the diploic spaces in the skull. As a result of the active marrow hyperplasia, pneumatization of the maxillary sinuses fails to occur at the proper time and there is considerable overgrowth of the maxilla. This causes severe overbite, prominence of the upper incisor teeth, and separation of the orbits.’ The trabeculae of the thickened diploic spaces often become arranged vertically, giving the “hair-on-end” appearance described in the literature. Preventive procedures are now being developed and appear promising. According to one investigator,* the facial disfigurement seen in these patients can be prevented by periodic transfusion therapy, as long as the hemoglobin is kept at an optimal level and therapy is started by the fifteenth month of life. Kaplan and associates3 described dental and oral findings in a study of fifty cases; 64 percent of the patients showed the maxillary deformities previously described, but the DMF rate and gingival inflammation were not unusual. Johnston and Krogman4 studied the patterns of growth of fifty persons with Cooley’s anemia. They concluded that the growth patterns of these children were characterized by a retardation in normal growth