The Haemophilic Pseudotumour or Haemophilic Subperiosteal Haematoma

Valderrama, J. A. Fernandez de; Matthews, J. M.

doi:10.1302/0301-620x.47b2.256

Cited by 67 publications

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“…A distinctive type of pesudotumour was described by Fernandez de Valderrama and Matthews, which affects the muscle and has no effect on the adjacent bone [1].…”

Section: Introductionmentioning

confidence: 99%

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Surgical treatment of the haemophilic pseudotumour: A single centre experience

Panotopoulos

Trieb

et al. 2012

International Orthopaedics (SICOT)

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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 cooperation between surgeons and haematologists.

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“…A distinctive type of pesudotumour was described by Fernandez de Valderrama and Matthews, which affects the muscle and has no effect on the adjacent bone [1].…”

Section: Introductionmentioning

confidence: 99%

“…The haemophilic pseudotumour was defined by Fernandez de Valderrama and Matthews as a progressive cystic swelling involving muscle, produced by recurrent haemorrhage and accompanied by radiographic evidence of bone involvement [1][2][3]. Anatomically, it is an encapsulated haematoma with calcification and ossification [2].…”

Section: Introductionmentioning

confidence: 99%

Surgical treatment of the haemophilic pseudotumour: A single centre experience

Panotopoulos

Trieb

et al. 2012

International Orthopaedics (SICOT)

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“…Persistent, encapsulated haematomas, frequently referred to as `haemophilic pseudocysts' or pseudotumours, which occur when cysts develop in close proximity to bone and cause destruction of cortex, periosteal elevation and new bone formation simulating osteosarcoma [3,6,8], are uncommon complications of severe congenital bleeding disorders. An estimated incidence of 1–2% among patients with severe haemophilia A and B is cited by Gunning [9], and was confirmed in a recent survey of Spanish haemophiliacs [14].…”

Section: Discussionmentioning

confidence: 99%

“…Development of a pseudotumour or pseudocyst, presumably derived from a persistent, slowly expanding, encapsulated soft‐tissue haematoma, is a well‐recognized but rare complication of severe and moderate haemophilia A and B [3,6,8,14,19], but has only been reported in one patient with vWD [17]. This report describes the complicated clinical course and successful management of a recurrent, massive, infected retroperitoneal pseudotumour in a 41‐year‐old patient with type 3 vWD.…”

Section: Introductionmentioning

confidence: 99%

Massive retroperitoneal pseudotumour in a patient with type 3 von Willebrand disease

et al. 2002

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Formation of destructive haemorrhagic pseudocysts or pseudotumours thought to arise from unresolved, encapsulated haematomas is a well-recognized, rare complication of severe haemophilia A or B, and has been reported in a single patient with von Willebrand disease (vWD). We report a 41-year-old patient with type 3 vWD who underwent incomplete resection of a large retroperitoneal pseudocyst in 1995 and presented with a recurrent, extensive right abdominal and flank mass and signs and symptoms of large bowel obstruction. He required emergency partial colectomy for bowel ischaemia and removal of his right kidney, which was hydronephrotic due to prolonged ureteral obstruction by the pseudocyst. Following repeat partial resection of the pseudotumour, he developed persistent bleeding into the operative site despite aggressive administration of von Willebrand factor (vWF)-rich factor VIII concentrates, resulting in retroperitoneal haematomas and abscesses, which resolved after 13 months of percutaneous drainage, extended supplementation of vWF and antibiotic therapy.

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“…In 1965 Fernandez Valderrama and Matthews described the haemophilic pseudotumour as a progressive cystic swelling involving muscle, produced by recurrent haemorrhage and accompanied by radiographic evidence of bone involvement [6]. They reported that the condition should be differentiated from simple blood cysts which occur in the fascial envelopes of muscles without radiographic changes.…”

Section: Introductionmentioning

confidence: 99%

The haemophilic pseudotumour

Merchan

1995

International Orthopaedics

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The orthopaedic management of severe haemophilia is mainly concerned with intra-articular and intramuscular bleeding. Pseudotumour is a serious, but very rare, complication; it is a progressive cystic swelling involving muscle, produced by recurrent bleeding and accompanied by radiographic evidence of bone involvement. Ultrasonography, CT scan, MRI and vascular injection studies should be undertaken, but preoperative biopsy is contraindicated. Most pseudotumours are seen in adults and occur near the large bones of the proximal skeleton. However, a number develop distal to the wrist and ankle in younger patients before skeletal maturity. If untreated, proximal pseudotumours will destroy soft tissues, erode bone and produce vascular or neurological lesions. Surgical removal is the treatment of choice when it can be carried out in major haemophilia centres, and has a mortality rate of 20%. Regression, but not a true cure, may occur with long term replacement therapy and immobilisation; this conservative treatment is not recommended except in patients with high-titre inhibitors in whom operation is not a possibility. In these cases, percutaneous evacuation and filling with a fibrin seal or cancellous bone, depending on the size of the cavity, should be carried out. Distal pseudotumours should be treated primarily with long term factor replacement and cast immobilisation because they may respond to many modalities of conservative management. The presence of one or more progressively enlarging masses in the limbs or pelvis of a haemophiliac should raise the suspicion of a pseudotumour, although chondrosarcoma and liposarcoma have occurred in such patients.

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The Haemophilic Pseudotumour or Haemophilic Subperiosteal Haematoma

Abstract: Knochenusur durch em hamophiles subperiostales H#{228}matom. Mitteilungen aiis den Grenzgebieten der Medizin und Chirurgie, 31, 381.TRUETA,

Cited by 67 publications

References 0 publications

Surgical treatment of the haemophilic pseudotumour: A single centre experience

Surgical treatment of the haemophilic pseudotumour: A single centre experience

Massive retroperitoneal pseudotumour in a patient with type 3 von Willebrand disease

The haemophilic pseudotumour

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