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. 2012 Oct;36(10):2157-62.
doi: 10.1007/s00264-012-1593-7. Epub 2012 Jul 4.

Surgical treatment of the haemophilic pseudotumour: a single centre experience

Joannis Panotopoulos  1 , Cihan Ay, Klemens Trieb, Philipp T Funovics, Verena Stockhammer, Susanna Lang, Johannes Holinka, Reinhard Windhager, Ingrid Pabinger, Hugo A Wanivenhaus
Affiliations

Surgical treatment of the haemophilic pseudotumour: a single centre experience

Joannis Panotopoulos et al. Int Orthop. 2012 Oct.

Abstract

Purpose: Haemophilic pseudotumour was defined by Fernandez de Valderrama and Matthews as a progressive cystic swelling involving muscle, produced by recurrent haemorrhage into muscles adjacent to the bone. The pseudotumour mainly occurs in the long bones and the pelvis. The treatment of the haemophilic pseudotumour poses a challenge, and extensive clinical experience is essential to appropriately address this serious complication in patients with haemophilia. Consequently, the aim of this study is to present our own clinical experience and treatment results of the haemophilic pseudotumour.

Methods: We retrospectively reviewed the records of 87 patients with bleeding disorders treated between 1967 and 2011 for musculoskeletal complications of congenital bleeding disorders. We identified six patients with a haemophilic pseudotumour who were treated at our department.

Results: The mean age at surgery was 45.9 (range, 40-61) years. The iliac bone was affected in three patients (one right, two left), the right tibia (distal diaphysis) in one, the right thigh in two and the right ulna (proximal part) in one patient. One patient had two pseudotumours. The perioperative course was easily controllable with adequate factor VIII substitution. At the latest follow-up after 8.4 (range, 4-24) years, normal healing with no recurrence was observed.

Conclusions: The haemophilic pseudotumour is a rare but severe complication of hereditary bleeding disorders. In the international literature the resection and postoperative course are described as challenging and difficult, requiring detailed preoperative planning. It is advisable to perform such operations in specialised centres with close co-operation between surgeons and haematologists.

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Figures

Fig. 1
Fig. 1
MRI of the right iliac bone with T1-weighted image of a poly-lobulated mass with fibrous capsule (a) and rim enhancement after application of contrast agent (b) (patient 5)
Fig. 2
Fig. 2
MRI of the right thigh with T1-weighted image of a poly-lobulated tumour mass with fibrous capsule and minor contrast agent uptake (patient 3)
Fig. 3
Fig. 3
Factor VIII levels
Fig. 4
Fig. 4
Radiograph with expansive osteolytic tumour mass with minor calcification of the right iliac bone (patient 5)
See this image and copyright information in PMC

References

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