title: Surgical treatment of the haemophilic pseudotumour: a single centre experience.
abstract: OBJECTIVE
  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.
authors: Department of Orthopaedics, Medical University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria. joannis.panotopoulos@meduniwien.ac.at
authors: Joannis
authors: J
authors: Panotopoulos
authors: Cihan
authors: C
authors: Ay
authors: Klemens
authors: K
authors: Trieb
authors: Philipp T
authors: PT
authors: Funovics
authors: Verena
authors: V
authors: Stockhammer
authors: Susanna
authors: S
authors: Lang
authors: Johannes
authors: J
authors: Holinka
authors: Reinhard
authors: R
authors: Windhager
authors: Ingrid
authors: I
authors: Pabinger
authors: Hugo A
authors: HA
authors: Wanivenhaus
mesh_terms: D000328:Adult; D006104:Granuloma, Plasma Cell / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D006467:Hemophilia A / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D006470:Hemorrhage / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology; D009135:Muscular Diseases / Q000473:pathology; D012189:Retrospective Studies; D016896:Treatment Outcome