Image Quiz
Cortical Erosion of the Distal Parts of the Radius and Ulna in a Sixteen-Year-Old Boy with Hemophilia1
A sixteen-year-old boy with hemophilia presented to the Khartoum Teaching Hospital with an open wound on the volar surface of the distal part of the left forearm (Fig. 1).
Fig. 1 |
Fig. 1 Photograph made at presentation, demonstrating the ruptured cyst and contractures of the fingers.
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He had had painful swelling of the forearm, following heavy lifting, one month earlier. The swollen area spontaneously opened and drained for ten days prior to presentation. The fingers were contracted in an intrinsic-minus position. Active finger motion was limited, and only a few degrees of painful passive extension could be achieved. Radial and ulnar pulses were not felt because of the extensive swelling, but capillary filling was satisfactory. There was decreased sensation in the median nerve distribution. Radiographs of the left forearm showed soft-tissue swelling with subperiosteal new-bone formation, cortical erosion, and lytic lesions in the distal third of the radius and ulna (Fig. 2).
Fig. 2 |
Fig. 2 Preoperative anteroposterior radiograph (left image) of the distal part of the forearm, showing soft-tissue swelling with cortical erosion of the distal parts of the radius and ulna. Preoperative lateral radiograph (right image) showing erosion of both the radius and the ulna.
For larger view, click on image |
The medical history revealed a major delay in the diagnosis of the hemophilia. The patient had bled abnormally at circumcision in infancy and following minor injuries throughout childhood. At ten years of age, he had swelling in the right forearm with an osseous erosion, which was diagnosed radiographically as an osteosarcoma. A below-the-elbow amputation was performed and was followed by extensive postoperative bleeding. Painful swelling of the right elbow one year later was thought to be a recurrence, and a higher amputation was recommended. The patient's family refused, a course of radiation therapy was administered, and the pain and swelling subsequently subsided. When the patient was twelve years of age, trauma to the scalp resulted in soft-tissue swelling and spontaneous rupture in the right elbow, necessitating blood transfusions. It was at that time that investigations for bleeding disorders were first carried out and the diagnosis of factor-VIII deficiency was made. There was also a family history of bleeding disorders: of the patient's twelve siblings, three (all male) also had factor-VIII deficiency.
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