A Huge Subcutaneous Hematoma in an Adult with Kasabach-Merritt Syndrome

Abstract

BACKGROUND Kasabach-Merritt syndrome is a potentially fatal disease that consists of hemangioma(s) with thrombocytopenia, microangiopathic hemolytic anemia, and coagulopathy. Extensive hemangiomatosis is rare. We present the radiological features and treatment strategy of a young adult suffering from Kasabach-Merritt syndrome with widespread hemangiomas and an infected huge hematoma in the right thigh. CASE REPORT A 33-year-old Taiwanese male presented with a painful 20-cm mass over his right thigh and gross hematuria for 2 days. Hemangiomatosis was bioptically proven in infancy and the patient was under regular follow-up. Physical examination revealed normal heart rate, respiratory rate, and body temperature. Multiple palpable lumps with brown and purple areas of skin over the neck, trunk, and right thigh were noted. Laboratory examinations revealed thrombocytopenia anemia and elevated fibrin degradation products. There were no signs of sepsis. Blood transfusion and steroid therapy were executed. Computed tomography showed a huge complicated subcutaneous hematoma in the right thigh. Drainage of the huge hematoma was performed and antibiotics were prescribed. After the local infection in the right thigh and the bleeding tendency were controlled, the patient was discharged in a stable condition two weeks later. CONCLUSIONS A huge infected hematoma and widespread hemangiomas are extremely rare complications of Kasabach-Merritt syndrome. There are no known treatment guidelines currently available. Our patient was successfully treated with steroids, drainage, and antibiotics.

Figure 1.

Chest x-ray shows soft-tissue-density mass lesions (arrows) at the right axilla, right neck, and pleural involvement in addition to infiltration of the chest wall.

Figure 2.

Contrast-enhanced computed tomography demonstrates lobulated masses (arrows) involving the right neck, right axilla, pleural involvement in addition to infiltration of the chest wall, right paraspinal region, left psoas muscle, peritoneal cavity, and right thigh.

Figure 3.

Contrast-enhanced computed tomography of the abdomen shows delayed and progressive centripetal filling in the enhancement pattern of the lobulated mass (arrows) at the right longissimus thoracis muscle and right spinalis muscle. (A) Early arterial phase; (B) late arterial phase; (C) delayed phase.

Figure 4.

(A) Contrast-enhanced computed tomography shows a cystic mass lesion (arrows) with peripheral enhancement and inner septation at right thigh. (B) A 3D volume rendering reconstruction shows associated bony erosion of the greater trochanter of the right femur (arrow) due to the mass rather than to osteomyelitis with bony destruction.