Pseudotumors of the Limbs in Patients with Hemophilia

Research Article

J Blood Disord. 2015;2(2): 1026.

Pseudotumors of the Limbs in Patients with Hemophilia

Caviglia H1,2*, Landro ME¹, Galatro G1,2, Candela M² and Neme D²

¹Department of Orthopaedic Surgery and Traumatology, Fernández General Hospital, Bs. As.-Argentina

²Haemophilia Foundation, Bs. As.-Argentina

*Corresponding author: Caviglia HA, Department of Orthopaedic Surgery and Traumatology, Fernández General Hospital, Bs. As.-Argentina,

Received: April 16, 2015;Accepted: May 18, 2015; Published: May 25, 2015

Abstract

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.

Keywords: Pseudotumors; Haemophilia; Prophylaxis; Radiation; Amputation; Conventional treatment; Percutaneous treatment

Introduction

The hemophilic pseudotumour is a truly encapsulated hematoma, which tends to progress and produce different clinical symptoms depending on its anatomic location. Therefore, it is a clinical entity rather than a specific pathological lesion [1]. Pseudotumor growth can lead to destruction of soft parts, bone erosion or neurovascular complications due to compression [2]. These lesions were first described by Starker in 1918. He stated that pseudotumors are, in hemophiliacs, clinical entities and not specific pathological injuries [3].

Hemophilic pseudotumours only occur in one-two% of patients with severe hemophilia [4,5]. Nevertheless, when patients do not received adequate treatment the hemorrhage episodes, pseudotumor incidence increases. They can also occur in patients with moderate or mild disease [6]. This paper shows that with proper treatment of bleeding disorders, the incidence of pseudotumors is reduced.

Radiation therapy was the conservative treatment used in the early eighties. This treatment caused a large number of pathological fractures. At that time, conventional surgical treatment was resection of the pseudotumor and its pseudocapsule. Amputation was used when the pseudotumor was very invasive and there was no other option [7].

In 1982, Fernandez-Palazzi et al. developed a new technique for treating early cysts. The procedure consists of locating the cyst by means of an image intensifier, puncturing it with a trocar, and finally, aspiration of the cavity content and filling with fibrin seal [7]. In 1990 the Mariano R. Castex Investigations Institute of Argentinaás National Academy of Medicine decided to treat the largest cysts and pseudotumors with aspiration and then fill the cavity with bone graft in bone pseudotumors and with spongostan® or fibrin seal in soft ones [7].

Also in 1982, Fernandez Valderrama et al., described pseudotumor capsules as defensive fibrous tissue which resists the advance of hematoma [8] Therefore, at our center, the Hemophilia Foundation in Buenos Aires-Argentina, we considered capsule resection unnecessary. In the 1990’s bone pseudotumor cavities were filled with lyophilized bone apatite. Currently we use hydroxyapatite coralline which is easier to work with and to find and also less expensive.

The objective of this paper is to show the evolution of the treatment of pseudotumors at our center over 47 years. We demonstrate that mini-invasive techniques and proper treatment reduce the numbers of pseudotumors in patients with hemophilia.

Patients and Methods

At the Hemophilia Foundation in Buenos Aires, Argentina, forty-nine patients were treated for sixty-four pseudotumors between 1967and 2014. 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 multiple pseudotumors (simultaneous or successive). The average age of the patients was 26 years old (6-75, median 23). Nine were factor VIII or IX inhibitor patients.

There was an average delay of 22 months between the appearance of the pseudotumor and the start of treatment (3-284months, median 14 months).

Forty-five (70%) pseudotumors were located in bones: fourteen (23.4%) in the femur, twelve (18.8%) in the tibia, six (9.4%) in the calcaneus, three (4.7%) in the ulna, three (4.7%) in the phalanx, three (4.7%) in the tarsal-metatarsal, one (1.6%) in the talus, one (1.6%) in the radius, one (1.6 %) in the cuboid, one (1.6%) in the fibula. Nineteen (30%) were soft tissue pseudotumors: ten of these were located in the thigh (15.6%), two (3.1%) in the leg, two (3.1%) in the knee, two (3.1%) in the foot, one (1.6%) in the gastrocnemius muscle and one (1.6%) in the arm Graph 1.