Radiotherapy of Hemophilia Pseudotumors

Abstract

The different radiologic features of hemophiliac joint and bone changes are well known and described in the literature (1, 5). The most common manifestation of hemophilia is crippling joint deformity. The classic and typical joint and bone changes are usually seen only in authentic hemophilia or hemophilia A and in Christmas disease or hemophilia B. In cases of hemophilia C there are only minimal and slight changes in the bones and joints. In extensive hemophiliac arthropathies, the pediatrician and the orthopedic surgeon usually deal with the therapeutic problems. A hemophiliac patient has been followed for eleven years at the University of Chicago Hospitals. The first episode of bleeding was at the age of thirteen months, and at that time the diagnosis of hemophilia A was made. The patient's family moved to Illinois in 1955 when he was nine years old, and he has been followed by us ever since. In the past twelve years he has had 32 admissions to our hospital with different complications of hemophilia such as: (a) chronic anemia; (b) gastrointestinal bleeding; (c) genitourinary bleeding; (d) soft-tissue hematomas; (e) hemarthroses with joint deformities; and (f) hemophiliac pseudotumors. Since 1955 this patient has had numerous episodes of joint involvement with radiographic changes. In addition he has had the unusual x-ray findings of hemophiliac pseudotumors. Hemophiliac pseudotumors, the most severe and extensive destructive lesions, are due to subperiosteal hemorrhage (5). In these cases large areas of bone are completely destroyed. The term hemophiliac pseudotumor is used because subperiosteal hemorrhages are seen, usually with soft-tissue swelling. The hemophiliac pseudotumors can be quite similar to malignant bone tumors, but they should be differentiated from benign lesions as well as from aneurysmal bone cysts. At eleven years of age our patient was admitted because of severe bleeding of the gums. The dental roentgenogram revealed only a slight shift mesially and forward of the second lower left molar. The bleeding stopped, and the patient was discharged but was readmitted several weeks later for transfusions because of severe anemia. On physical examination a fixed nontender mass was palpable at the angle of the left mandible (Fig. 1). Because of hemophilia, biopsy was contraindicated, and the boy received a course of x-ray therapy. Eight hundred rads were delivered to the jaw in 4 treatments in six days. The effect of radiation therapy is seen in Figure 2. At the age of fifteen years a firm, nontender swelling of the left hand was noticed by the patient (Fig. 3). He received 1,000 rads to the fifth metacarpal in 5 treatments in a period of five days. Figure 4 shows radiographs of the hand after therapy. The patient is now twenty-one years old and has no restriction of movement of the left hand. There has been no recurrence of the multiple pseudotumors.