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PubMed2003

[Fibrous dysplasia of the petrous bone].

Fibrous dysplasia named after Jaffè and Lichtenstein is a benign, self-limiting disorder of the bone structure. The normal bone is replaced by cellular fibrous connective tissue. Fibrous dysplasia can be differentiated into monostotic and polyostotic forms and the McCune-Albright syndrome (combination of polyostotic areas in the bone with hyperpigmentation of the skin and endocrinological disturbances). The rare disease is predominantly found in the 1st to 3rd decade of life in the femur or humerus. In about 15% of all cases it affects the facial bones, and one-third of these is located in the jaw bone. Extremely rare is the invasion of the petrous bone. A 45-year-old female patient was examined because of tinnitus persisting for 1 year. In addition, she was suffering from increased hearing reduction and recurrent vertigo. The CT and MRT showed a milky glass-like tumor, which was limited by an osteolytic process situated in the left petrous bone. The process was removed via the mastoid. Histological examination revealed fibrous dysplasia. A highly differentiated osteosarcoma and an ossifying fibroma as important differential diagnoses have to be excluded because of the different therapeutic consequences.

Cerebellopontine Angle↗
PubMed1987

Monostotic fibrous dysplasia of the temporal bone.

Fibrous dysplasia is a rare but interesting benign condition of the bones which affects either a single bone or multiple bones, with or without endocrine abnormalities. Only 75 cases affecting the temporal bone have been reported in the world literature, and to these we add another case. Although systemic involvement in the form of endocrine abnormalities and skin pigmentation has been reported in the past, no case has yet been reported with a congenitally absent kidney in the monostotic form of the disease.

Fibrous Dysplasia of Bone↗
PubMed1985

Middle ear surgery in fibrous dysplasia of the temporal bone.

Fibrous dysplasia of the temporal bone is uncommon. When it does occur, surgery may be necessary because of cholesteatoma or chronic infection behind bony obstruction of the external auditory canal. Cosmetic surgery may also be performed because of bony deformity. When a surgical procedure is undertaken for hearing improvement, special problems may be encountered. The case presented illustrates middle ear pathologic changes found with fibrous dysplasia and specific surgical difficulties.

Adolescent↗
PubMed2005

[Fibrous dysplasia of the temporal bone].

Fibrous dysplasia of the temporal bone is a rare disease which may lead to progressive stenosis of the external auditory canal with conductive hearing loss. Sensorineural hearing loss has usually been attributed to involvement of the otic capsule or inner auditory canal. The case of fibrous dysplasia of the temporal bone in 11 years old girl was described. The conductive hearing loss with sensorineural component improved after surgical treatment.

Audiometry↗
PubMed1999

Fibrous dysplasia of the temporal bone.

Fibrous dysplasia is characterized by a progressive replacement of normal bone elements by fibrous tissue. It is seen in the two first decades of life and is equally distributed in relation to the sex of patients. The temporal bone is involved in 18% of the cases. Fibrous dysplasia of the temporal bone is more commonly manifested in the external auditory canal (80%) and the inner and medium ear can also be involved. Sarcomatous alterations are rare. Diagnosis is based on radiological images. Treatment is conservative and surgery is reserved for preserving function and preventing complications. The case of a male patient, 16 years old, who presented with progressive hearing loss over a 1-year period is reported.

Adolescent↗
PubMed2004

The role of radionuclide bone scintigraphy in fibrous dysplasia of bone.

PURPOSE: The purpose of this study was to study the characteristics of fibrous dysplasia (FD) of bone on bone scans and to evaluate the diagnostic value of radionuclide bone scans in FD. METHODS: Radionuclide bone scans were performed in 42 cases of histopathologically proven FD and the results were compared with other imaging modalities. A retrospective study method was used to analyze the imaging results. RESULTS: Although FD showed nonspecific increased 99m-Tc MDP uptake, its appearance is different than bone metastates and other bone diseases. Combining scans with x-rays and other imaging modalities can improve the diagnostic accuracy of this disease. CONCLUSIONS: Radionuclide bone scans are of certain value in the diagnosis of FD. The diagnostic specificity of FD with radionuclide bone scanning can be improved in association with other imaging modalities such as x-rays.

Adolescent↗
PubMed1999

Monostotic fibrous dysplasia of the temporal bone.

Fibrous dysplasia is a slowly progressive bony disorder where normal bone is replaced by abnormal fibro-osseous tissue. Its monostotic variety in the temporal bone is very rare and such a case is presented here. Computed tomography (CT) may be adequate for the diagnosis and follow-up of these patients. Limited surgery should only be considered in cases of symptomatic disease.

Fibrous Dysplasia, Monostotic↗
PubMed2006

Fibrous dysplasia of the frontal bone.

Fibrous dysplasia typically occurs in patients between the ages of 5 and 15 years. We report the case of a middle-aged woman with longstanding monostotic fibrous dysplasia who responded well to conservative treatment. She had presented with a very long (15 yr) history of frontal headache and pressure over the frontal sinus. On examination, a protuberance was noted in the region of the right frontal sinus. No other symptoms were evident, and findings on the ENT examination were normal. Computed tomography (CT) detected a widening of the frontal bone with osteolytic changes of the cancellous bone of the diploe. Partial multiphase skeletal scintigraphy and single-photon emission CT of the cranium showed increased bone metabolism in the area of the right frontal bone. No other hot spots were seen. In view of the absence of any complications, no tissue biopsy was performed. The patient was prescribed bisphosphonates for symptom relief. Follow-up CT 1 year later revealed no progression of the lesion. Overtreatment of fibrous dysplasia should be avoided because most patients respond to conservative management. In those cases that are refractory to medical treatment, surgery--in experienced hands--can result in a good functional and cosmetic outcome.

Bone Density Conservation Agents↗