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The haemophilic pseudotumour
1Haemophilia Unit, La Paz Orthopaedic and Traumatology Hospital, Madrid, Spain.
International Orthopaedics
|January 1, 1995
Summary
Pseudotumours are rare but serious complications of severe haemophilia, causing bone and soft tissue damage. Surgical removal is preferred for proximal pseudotumours, while distal ones may respond to conservative management.
Area of Science:
- Orthopaedic Surgery
- Haematology
- Radiology
Background:
- Severe haemophilia commonly involves intra-articular and intramuscular bleeding.
- Pseudotumours are rare, progressive cystic masses resulting from recurrent bleeding, often with bone involvement.
Purpose of the Study:
- To outline the orthopaedic management of pseudotumours in severe haemophilia.
- To differentiate treatment strategies for proximal versus distal pseudotumours.
Main Methods:
- Diagnostic imaging including ultrasonography, CT scan, MRI, and vascular injection studies.
- Preoperative biopsy is contraindicated.
- Surgical removal for proximal pseudotumours; conservative management (factor replacement, immobilization) for distal pseudotumours.
Main Results:
- Proximal pseudotumours can cause significant soft tissue destruction, bone erosion, and neurovascular compromise if untreated.
- Surgical removal of proximal pseudotumours has a 20% mortality rate.
- Distal pseudotumours may respond to conservative treatments like factor replacement and immobilization.
Conclusions:
- Pseudotumours require prompt diagnosis and appropriate management based on location.
- Surgical intervention is the primary treatment for proximal pseudotumours in haemophilia centres.
- Conservative management is indicated for distal pseudotumours and in specific cases of proximal pseudotumours (e.g., high-titre inhibitors).