Pseudotumors of the Limbs in Patients with Hemophilia

Introduction: The haemophilic pseudotumour is a truly encapsulated hematoma, which tends to progress and produce different clinical symptoms depending on its anatomic location. Efforts must be made to prevent pseudotumors by ensuring that all patients receive adequate treatment for their muscle and intra-osseous hematomas. The objective of this paper is to show the evolution of the treatment of pseudotumors at our center over 47 years (1967- 2014). Patients and Methods: Between 1967 and 2014 forty-nine patients were treated for sixty four pseudotumors. Forty-six were hemophilia type A (93.8%) and three type B (6.2%). Thirty-nine (79.6%) patients had single pseudotumors and ten (20.4%) had multiples pseudotumors. The average age of the patients was 26 years old. Nine were factor VIII or IX inhibitor patients. Forty-five (70%) pseudotumors were located in bones and nineteen (30%) were soft tissue pseudotumors. Results: The frequency of hemophilic pseudotumors in our center has shown a gradual reduction from 2.6% in 1971 to 0.25% in 2014.Sixty percent of patients treated with radiation therapy evolved favorably. Five amputations were successfully performed. Sixteen resections of the pseudotumor and its pseudocapsule were performed. Four patients died and two patients developed a fistula after pseudotumor resection. Two patients responded to conservative treatment and did not require surgery. Mini-invasive surgery (percutaneous method) was performed on the other thirty-two patients with thirty-eight pseudotumors. Mini-invasive surgery failed in three patients. We did not observe any bleeding or infections due to surgery. Conclusion: We believe that pseudotumors should be treated with minimally invasive technique (suction and refilling) with the proper hemostatic coverage.