Fibrous dysplasia is an uncommon benign disorder of unknown etiology. The disease was first described by McCune and Albright in separate publications in 1937. The term, fibrous dysplasia was suggested by Lichtenstein in 1938. The disease has since been found to have 3 different variants: monostotic, polyostotic, and McCune-Albright syndrome. It is a slowly progressive bony disorder where normal bone is replaced by abnormal fibrosseous tissue. Involvement of fibrous dysplasia of the temporal bone is usually unilateral. The squama becomes thickened and the pneumatic system is obliterated. Because fibrous dysplasia shows a predilection for the facial and cranial bone, where it causes deformity and dysfunction. In this paper, we report a case of fibrous dysplasia of the temporal bone. We discuss the characterisitic features of this specific location of the disease, the differential diagnosis, and the treatment policy. We also address the issue of vertigo.
Congenital Abnormalities ; Diagnosis, Differential ; Dizziness ; Fibrous Dysplasia, Monostotic* ; Fibrous Dysplasia, Polyostotic ; Hearing Loss* ; Hearing* ; Temporal Bone* ; Vertigo

Fibrous dysplasia of bone is a condition affecting one or more bones, usually not disabling, of slow progress, and showing a tendency to become arrested. The etiology of fibrous dysplaia is unknown but it is now believed to be a developmental error in which primitive fibrous tissue proliferates within the bony medulIa and encroaches upon the cortex from within, often producing expansion. The process may be localized to a small segment or may involve almost the entire shaft of the bone, and it may be monostotic, monomelic or polyostotic. In the latter form some patients exhibit a remarkable combination of skin pigmentation and endocrine disturbances. This paper reports the one case of polyostic and three cases of monostotic fibrous dysplasia. The disgnosis was made by clinical, radiological and histological findings.
Fibrous Dysplasia of Bone ; Fibrous Dysplasia, Monostotic ; Humans ; Skin Pigmentation

Monostotic Fibrous dysplasia is a relatively rare condition. Three such conditions are experienced at the Dept. of Orthopedic Surgery, Han-II Hospital during the period of September 1970 to June 1972. Two of three cases were involing shaft of left and right tibia respectively, one of which was complicated by pathologic fracture. The third case was on greater trochanter of right femur. All of three cases were treated with curertage and bone graft. Excllent results were obtained.
Femur ; Fibrous Dysplasia of Bone ; Fibrous Dysplasia, Monostotic ; Fractures, Spontaneous ; Orthopedics ; Tibia ; Transplants

Fibrous dysplasia of bone is a disorder in which one or more bone lesions are present, but in which extraskeletal abnormalities not infrequently form part of the total disease complex. The etiology is unknown but it is probably a developmental fault of bony development, in which an abnormal proliferation of fibro-osseous tissue replaces only the cancellous tissue and erodes the cortex from within, eventually causing distortion and expansion. It may appear as a solitary lesion or more than one lesion in single bone, and sometimes it may be monomelic or polyostotic. This paper report a case of a monostotic fibrous dysplasia in one-month-old infant with a review of the literatures.
Cytochrome P-450 CYP1A1 ; Fibrous Dysplasia of Bone ; Fibrous Dysplasia, Monostotic ; Humans ; Infant

Fibrous dysplasia of bone is a relatively rare condition characterized by fibrous tissue replacement of skeleton, usually not disabling, of slow progress, and showing a tendency to become arrested. It may be monostotic (confined to one bone) or Polyostotic (situated in many bones). The etiology of fibrous dysplasia is unkown but it is now believed to be a developmental error in which primitive fibrous tissue proliferates within the bony medulla and encroaches upon the cortex from within. This paper reports the one case of polyostotic fibrous dysplasia. The diagnosis was made by clinical, radiological and histological findings. The review of literatures was made briefly.
Diagnosis ; Fibrous Dysplasia of Bone ; Fibrous Dysplasia, Polyostotic ; Skeleton

Fibrous dysplasia is a developmental anomaly of bone formation, which is characterized by a replacement of the normal bone and marrow by fibrous tissue as well as small, woven spicules of bone. These lesions may exist in a monostotic or polyostotic form and are generally found in the 4th decade of age or younger with a slightly higher incidence in girls than boys. We report a case of monostotic fibrous dysplasia that was associated with a pathologic fracture and a kyphotic deformity in the cervical spine.
Bone Marrow ; Congenital Abnormalities ; Fibrous Dysplasia, Monostotic ; Fractures, Spontaneous ; Incidence ; Osteogenesis ; Spine

Malignant transformation of fibrous dysplasia to osteosarcoma is rare. We report a case in which monostotic fibrous dysplasia of the proximal femur treated with curettage, Ender nailing and bone grafting was differentiated into the osteosarcoma in a 58-year-old female.
Bone Transplantation ; Curettage ; Female ; Femur ; Fibrous Dysplasia, Monostotic ; Humans ; Middle Aged ; Osteosarcoma

Mazabraud's syndrome is rare benign disease characterized by the association of intramuscular myxoma and fibrous dysplasia of bone. We present a case of multiple intramuscular myxomas of the right forearm with polyostotic fibrous dysplasia. X-rays suggested fibrous dysplasia of adjacent bones and CT scans showed two intramuscular cystic masses on the right forearm. 99mTc bone scintigraphy also showed a markedly increased uptake in the skull, both scapulas, both clavicles and both humeri. The masses were successfully removed. Fibrous dysplasia of the radius and myxoma were confirmed by pathologic study.
Clavicle ; Fibrous Dysplasia of Bone ; Fibrous Dysplasia, Polyostotic ; Forearm ; Myxoma ; Radionuclide Imaging ; Radius ; Scapula ; Skull ; Tomography, X-Ray Computed

McCune-Albright syndrome is a rare disease. It's triad is 1) precocious puberty, 2) fibrous dysplasia of bone and 3) caf-al-lait spots on skin. The cause is known as the mutation of subunit of G protein. Although there are some suggestions of treatment regimens including antiestrogen and GnRH agonist, at present, the accurate management is not known. We have experienced a case of McCune-Albright syndrome who has precocious puberty and fibrous dysplasia without caf-al-lait spot, and we present the case with a review of related literatures.
Estrogen Receptor Modulators ; Fibrous Dysplasia of Bone ; Fibrous Dysplasia, Polyostotic* ; Gonadotropin-Releasing Hormone ; GTP-Binding Proteins ; Puberty, Precocious ; Rare Diseases ; Skin

Fibrous dysplasia is a slowly progressive benign fibro-osseous disorder of unknown cause. The predilection sites of the craniofacial bones are maxilia and mandible, but fibrous dysplasia of the temporal bone is rarely noted, The usual clinical manifestations of fibrous dysplasia of the temporal bone are mainly otologic complications such as progressive stenosis of the external auditory canal and conductive hearing loss. But its neurologic complications are very rarely reported except facial nerve paresis. We present a case of monostotic fibrous dysplasia of the temporal bone leading to intracranial hypertension with central venous thrombosis. To our knowledge, this association has not been previously described in the literature.
Constriction, Pathologic ; Ear Canal ; Facial Nerve ; Fibrous Dysplasia, Monostotic ; Hearing Loss, Conductive ; Intracranial Hypertension* ; Mandible ; Paresis ; Temporal Bone* ; Venous Thrombosis*