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The cause of fibrous dysplasia of the bone is obscure. We reviewed the available literature concerning temporal bone involvement and analyzed temporal bone involvement and analyzed the most common aspects of this fascinating disease, adding three cases of our own. A clinical staging is proposed: stage 1, latent; stage 2, symptomatic; and stage 3, complication. Clinical evolution and follow-up are described and treatment is suggested.
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Monostotic fibrous dysplasia of the occipital bone is an unusual finding. A case is presented and the differential diagnosis is discussed.
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Three cases of fibrous dysplasia of bone, affecting the long bones of the leg are described. This condition is a clearly defined radiological and clinical entity. The tibial lesion is reminiscent of a congenital pseudarthrosis but the involvement of the fibula is a useful diagnostic clue. The most important feature of the condition is that it heals spontaneously.
The course of an organoid naevus phakomatosis (Schimmelpenning-Feuerstein-Mims syndrome) was observed in one patient over 25 years. Although the syndrome was fully developed, it has so far remained relatively benign, without limiting the patient's ability to work. During the course of the disease various investigations were carried out (CT, brain scintigrams, chromosome analysis). These confirm that the outstanding feature consisting of fibrous bone dysplasia of the skull continues to progress into adult life. In addition, the presence of intracerebral calcification could be demonstrated. Examination of the chromosomes suggests that further similar cases should also be examined, since the aetiology and pathogenesis of this phakomatosis are still unknown.
In a 43-year-old woman with fibrous dysplasia of the ribs and facial bone and in a 40-year-old man with fibrous dysplasia of the facial bone, there were considerable increases in the uptake of Tc-99m methylenediphosphonate (Tc-99m MDP) and Gallium-67 citrate. Fibrous dysplasia should be added to differential diagnosis of high Gallium-67 citrate uptake in the bone lesions.
Fibrous dysplasia is a rare disease which occasionally affects the temporal bone. In its monostotic form, only 27 cases have been reported until today. We present a case of monostotic fibrous dysplasia of the temporal bone coincident with a cholesteatoma of the EAC, operated on by mean of a modified radical mastoidectomy, with good results.
Fibrous dysplasia of the temporal bone is a rare disease which may lead to progressive stenosis of the external auditory canal and the development of cholesteatoma. We present a case in which minimal symptoms were present despite a massive temporal bone fibrous dysplasia. Cholesteatoma resulted most probably secondary to external auditory canal stenosis. Retroauricular fistula developed as a result of destructive effect of cholesteatoma, that influenced previous diagnosis and treatment of this clinically silent disease.
Polyostotic fibrous dysplasia affects many bones of the skeleton and usually on one side only. In monostotic variety only one bone is affected. The condition which usually starts in early childhood is at first active, gradually becomes quiescent and finally with termination of skeletal growth becomes inactive and ceases to grow. These stages can be seen histologically as well as radiologically. The main feature is increasing calcification of the mass. In half of our cases there was an intracranial mass with signs of raised intracranial or intra-orbital pressure. In all of these cases the mass originated from the base of skull. Those growing from the vault of the skull grew outward. Pathogenesis, diagnosis and treatment are discussed.
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Fibrous dysplasia of the femoral neck is difficult to treat. In a series of fifteen young patients, ten had a monostotic lesion and five, the polyostotic form of the disease. Twelve patients were first seen with a fatigue fracture. Grafts of cortical bone were used. The objectives of relief of pain, union of the fracture, and prevention of deformity were achieved in all fifteen patients. Two patients required a repeat procedure. None of the patients had important deformity of the femoral neck, and none needed an osteotomy.