British Journal of Radiology 74 (2001),186-188 © 2001 The British Institute of Radiology
Mandibular haemophilic pseudotumour containing a fluid–fluid level
H A Kale, MD1,
K R Rathod, MD1,
S R Prasad, MD1,
C M Madiwale, MD2 and
R J Sheth, MBBS1
1 Departments of Radiology
2 Pathology, King Edward Memorial Hospital, Mumbai, India
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Abstract
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Osseous haemophilic pseudotumours are uncommon. Mandibular haemophilic pseudotumours are rare, with only approximately 12 cases reported in the literature to date. We present imaging findings of a histopathologically proven mandibular haemophilic pseudotumour. A fluid–fluid level, hitherto not associated with this condition, is also described.
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Introduction
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Haemophilia A, transmitted as an X-linked recessive trait, is the most common heritable bleeding disorder [1]. It is characterized by a deficiency in circulating plasma levels of functionally normal clotting factor VIII:c. Haemophilic patients may bleed into various structures, sometimes owing to trivial or imperceptible trauma. Haemarthrosis is the most frequent painful manifestation of haemophilia [1]. Haemophilic pseudotumour (HP) is an extraarticular destruction of bone resulting from pressure necrosis by a haematoma. Other common sites of haemorrhage include subcutaneous tissues, fascia and muscles.
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Case report
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A 17-year-old haemophilic patient (severe deficiency of factor VIII activity <1%) presented with a 3-week history of a painless swelling of the left side of the jaw. There was no history of antecedent trauma to the jaw. Clinical examination demonstrated a non-tender non-inflammatory soft tissue mass overlying the body of the left mandible. Plain radiographs showed a large, well circumscribed, expansile osteolytic lesion in the body of the mandible (Figure 1
). Contrast enhanced CT demonstrated a fluid–fluid level within the large mandibular osteolytic lesion (Figure 2). Histopathological examination of a core biopsy specimen of the lesion showed haemorrhage rimmed by vascular fibrotic connective tissue and haemosiderin-laden macrophages, consistent with a HP (Figure 3). The patient was treated conservatively with factor VIII replacement and immobilization. There was considerable reduction in the size of the HP over the following 6 weeks.
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Figure 1. An osteolytic lesion in the body of the left mandible with an appearance of "floating teeth" on (a) posteroanterior and (b) oblique projections.
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Figure 2. Axial CT through the lesion shows destruction of the body of the left mandible, with fluid–fluid levels.
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Figure 3. Photomicrograph showing haemorrhage rimmed by vascular fibrous connective tissue (large arrow). Foci of haemosiderin deposition (small arrow) and haemosiderin-containing macrophages (double arrowhead) are noted.
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Discussion
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Haemophilia is a heterogeneous genetic disorder resulting from mutation in the factor VIII gene, leading to a reduction in factor VIII activity. Classical haemophilia is an X-linked recessive disorder, manifested only in men. Women are carriers of the disease. Clot formation is abnormal in haemophiliacs. The clot that is formed is friable and easily dislodged, thus contributing to excessive bleeding. Soft tissue haematomas and haemarthrosis are highly characteristic of this disorder.
Skeletal HP was first described by Starker in 1918 [2] in a patient with extensive destruction of the femur. The reported frequency of HP in patients with severe haemophilia is estimated to be 1–2% [3]. The sites of involvement of HP in descending order of frequency are femur, pelvic bones, tibia and small bones of the hand. About one-third of cases involve the femur [3].
Osseous HP may be intraosseous or subperiosteal in location. The pathogenesis of HP is considered to be owing to pressure necrosis secondary to haemorrhage into a closed subperiosteal or intramedullary space [4, 5]. Trauma is the initiating factor in most reported cases [6, 7]. Repeated bleeding into the cystic spaces contributes to growth of HP. Slow progression is the rule, unless complicated by an acute re-bleed. HP is usually asymptomatic, although it can be a source of exsanguinating haemorrhage [4].
The radiographic appearance of HP is variable. Medullary bone destruction may be demonstrated radiographically as well demarcated, small or large, central or eccentric radiolucent lesions through which the trabeculae may criss-cross. The margins are usually, but not always, sclerotic. Cortical involvement and periosteal reaction may be considerable. A large soft tissue mass may be encountered. CT is useful in assessing the extent of osseous involvement, as well as showing an associated soft tissue component and the anatomical relationship to important neurovascular structures. The differential diagnosis of an osteolytic lesion in the mandible includes alveolar abscess, periodontal cyst, dentigerous cyst, nasopalatine duct cyst, globulomaxillary cyst, Stafne's bone cyst, giant cell reparative granuloma, giant cell tumour, ameloblastoma, Langerhans cell histiocytosis, aneurysmal bone cyst, haemangioma, metastases, fibrous dysplasia, bone cyst and brown tumours of the mandible [8]. A specific diagnosis is usually only made on the basis of history or, more commonly, on the basis of histopathology.
Fluid–fluid levels may appear whenever fluids of different densities are contained within a cystic or compartmentalized structure. Fluid–fluid levels have been described in aneurysmal bone cysts, giant cell tumours, simple bone cysts, chondroblastoma and telangiectatic osteosarcoma [9, 10]. The fluid–fluid level associated with the lesion in our patient may represent layering secondary to recurrent bleeding into the cyst.
Conservative therapy consisting of factor VIII replenishment and immobilization is sufficient treatment in most cases of HP. Surgical intervention is restricted to extensive lesions with failed conservative treatment [7, 11, 12].
Received for publication January 25, 2000.
Revision received September 26, 2000.
Accepted for publication October 5, 2000.
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References
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Abstract
Introduction
