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Surgical treatment of the haemophilic pseudotumour: A single centre experience

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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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Acknowledgments

We thank Tanja Altreiter (Medical University of Vienna, Department of Medicine I) and Reinhard Schuh (Medical University of Vienna, Department of Orthopaedics) for proofreading this manuscript.

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Authors and Affiliations

  1. Department of Orthopaedics, Medical University of Vienna, Währinger Gürtel 18–20, 1090, Vienna, Austria

    Joannis Panotopoulos, Philipp T. Funovics, Verena Stockhammer, Johannes Holinka, Reinhard Windhager & Hugo A. Wanivenhaus

  2. Department of Medicine I, Clinical Division of Haematology and Haemostaseology, Medical University of Vienna, Vienna, Austria

    Cihan Ay & Ingrid Pabinger

  3. Department of Orthopaedics, Hospital Wels-Grieskirchen, Vienna, Austria

    Klemens Trieb

  4. Department of Clinical Pathology, Medical University of Vienna, Vienna, Austria

    Susanna Lang

Authors
  1. Joannis Panotopoulos
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  2. Cihan Ay
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  3. Klemens Trieb
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  4. Philipp T. Funovics
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  5. Verena Stockhammer
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  6. Susanna Lang
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  7. Johannes Holinka
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  8. Reinhard Windhager
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  9. Ingrid Pabinger
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  10. Hugo A. Wanivenhaus
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Correspondence to Joannis Panotopoulos.

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Panotopoulos, J., Ay, C., Trieb, K. et al. Surgical treatment of the haemophilic pseudotumour: A single centre experience. International Orthopaedics (SICOT) 36, 2157–2162 (2012). https://doi.org/10.1007/s00264-012-1593-7

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  • DOI: https://doi.org/10.1007/s00264-012-1593-7

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