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Fibrous dysplasia

Fibrous dysplasia is a disease that causes growths or lesions in one or more bones of the human body. These lesions are tumor-like growths that consist of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. Especially when involving the skull or facial bones, the lesions can cause externally visible deformities. The skull is often, but not necessarily, affected, and any other bone(s) can be involved. Many patients have lesions localized in only one bone (70-80%), but others have them in many bones. In many cases, people suffering from fibrous dysplasia also have endocrine diseases and skin pigmentation; the three together constitute McCune-Albright syndrome. more...

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Fibrous dysplasia is very rare, not much is known about it, and there is no known cure. However, it is known that it is caused by a genetic mutation that occurs sometime during fetal development, and is not hereditary.

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Intracerebral abscess secondary to frontal mucocele with underlying fibrous dysplasia
From Ear, Nose & Throat Journal, 4/1/04 by Enrique Palacios

Intracranial meningitis and intracerebral abscesses secondary to frontal sinus diseases, such as sinusitis and mucoceles, are well known? Frontal sinus mucoceles are usually the result of an inflammatory obstruction of the ostium. (2) However, other causes of frontal sinus obstruction can be identified, such as a frontal sinus osteoma, a post-traumatic sinus lesion, and occasionally obstruction by a fibro-osseous lesion, such as a fibrous dysplasia. (2,3)

In the case illustrated here, an intracranial complication from a frontal sinus mucocele with cerebral abscess was identified in a 45-year-old man who had complained of headaches, obtundation, and fever. The obstruction of the frontal sinus proved to be a localized fibrous dysplasia. Fibrous dysplasia represents a benign idiopathic bone disorder in which cancellous bone is replaced by abnormal fibrous tissue. (3) A frontal sinus mucocele is a rare complication of craniofacial fibrous dysplasia, as was demonstrated in this case by computed tomography (CT) (figure 1) and magnetic resonance imaging (MRI) (figure 2).

[FIGURES 1-2 OMITTED]

References

(1.) Harnsberger HR. Sinonasal imaging: Imaging issues in sinusitis. In: Harnsberger HR. Handbook of Head and Neck Imaging. 2nd ed. St. Louis: Mosby, 1995:339-95.

(2.) Som PM, Brandwein MS. Inflammatory diseases. In: Som PM, Curtin HD, eds. Head and Neck Imaging. 4th ed. St, Louis: Mosby, 2003:193-259.

(3.) Atasoy C, Ustuner E, Erden I, Akyar S. Frontal sinus mucocele: A rare complication of craniofacial fibrous dysplasia. Clin Imaging 2001;25:388-91.

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COPYRIGHT 2004 Gale Group

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