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PubMed1989

Hemorrhage into fibrous dysplasia following minor head injury--effective decompression for the ophthalmic artery and optic nerve.

We performed an effective optic canal decompression in a patient with ethmoid and sphenoid fibrous dysplasia and visual impairment following minor head trauma. On admission, the patient's left visual acuity consisted only of light perception, and the left ophthalmic artery was not visible by angiography. A hematoma and fibrous dysplasia tissue in the sphenoid sinus were excised and the left optic canal was decompressed. The vision was markedly improved to an ability to count fingers in the following 2 weeks. The left ophthalmic artery was fully opacified in the postoperative angiogram.

Arterial Occlusive Diseases↗
PubMed2005

FGF23 and disorders of phosphate homeostasis.

It is well known that fibroblast growth factor (FGF) family members are associated with embryonic development and are critical for basic metabolic functions. This review will focus upon fibroblast growth factor-23 (FGF23) and its roles in disorders associated with phosphate handling. The discovery that mutations in FGF23 were responsible for the isolated renal phosphate wasting disorder autosomal dominant hypophosphatemic rickets (ADHR) has ascribed novel functions to the FGF family. FGF23 circulates in the bloodstream, and animal models demonstrate that FGF23 controls phosphate and Vitamin D homeostasis through the regulation of specific renal proteins. The ADHR mutations in FGF23 produce a protein species less susceptible to proteolytic processing. X-linked hypophosphatemic rickets (XLH), tumor-induced osteomalacia (TIO), and fibrous dysplasia of bone (FD) are disorders involving phosphate homeostasis that share phenotypes with ADHR, indicating that FGF23 may be a common denominator for the pathophysiology of these syndromes. Our understanding of FGF23 will help to develop novel therapies for phosphate wasting disorders, as well as for disorders of increased serum phosphate, such as tumoral calcinosis, a rare disorder, and renal failure, a common disorder.

Animals↗
PubMed2002

Magnetic resonance imaging of benign bone lesions: cysts and tumors.

A benign bone lesion may have a typical appearance on plain radiographs. This is the case with benign cortical defects and osteochondroma. With most other lesions, cross-sectional imaging is needed to complete the study of the tumor. The nidus of osteoid osteoma is well demonstrated on computed tomography, but magnetic resonance imaging also will show the nidus in most cases. Magnetic resonance imaging is considered the modality of choice for evaluation of other benign musculoskeletal lesions because it is highly sensitive to changes in the signal intensity of bone marrow and adjacent soft tissues. It provides useful information for diagnosis of the lesion as in primary or secondary aneurysmal bone cyst, chondroblastoma, osteoblastoma, fibrous dysplasia, and osteofibrous dysplasia, and it helps differentiate these lesions from osteomyelitis, Langerhans' cell histiocytosis, and stress fracture. Bone scanning is most useful for depicting multiple silent lesions as may be seen in multiple osteochondromatosis, nonossifying fibromas, and polyostotic fibrous dysplasia.

Adolescent↗
PubMed1991

[The use of allogeneic bone and tendon tissue from a central bone bank].

During the period January 1989 to December 1990 the use of bone and related soft tissue allografts from our bone bank was reviewed retrospectively. Data were complete for 278 patients, who received 403 allografts in total. Forty-nine patients were transplanted with a massive deep frozen bone allograft, 41 patients received a deep frozen soft tissue allograft, while 313 units of freeze dried bone allografts were transplanted into 188 patients. Massive deep-frozen bone allografts were used mainly in patients with bone tumours, fibrous dysplasia of the neck of the femur and for revision arthroplasty. Soft tissues were used mainly for reconstruction of ruptured cruciate ligaments. Freeze dried bone allografts such as cancellous chips as well as cortical cancellous chips were used for spinal fusion, arthroplasty, treatment of pseudarthrosis, fractures, tumours and fibrous dysplasia, and in maxillofacial defects. Cancellous blocks were used specifically for spinal fusion. Demineralized cortical dust was used mainly in maxillofacial surgery.

Bone Diseases↗
PubMedDate not supplied

[Hypophyseal gonadotropin secretion in sexual precocity with McCune-Albright's syndrome].

In three young girls with isosexual precocity and fibrous dysplasia of bones, plasma gonadotropins (LH and FSH) response to releasing hormone (LH-RH) has been much under the mean response in true female precocious puberty. These data and those previously reported in the literature suggest that MacCune-Albright's syndrome correlates to an hypothalamo-pituitary dysfunction rather than to a true prematurity.

17-Hydroxycorticosteroids↗
PubMedDate not supplied

A boy with McCune-Albright syndrome associated with GH secreting pituitary microadenoma. Clinical findings and response to treatment.

The McCune-Albright Syndrome (MAS) is a sporadic rare disease first described in 1936 by McCune and separately by Albright. MAS is characterized by a triad of physical signs: café-au-lait spots, polyostotic fibrous dysplasia and autonomous endocrine hyperfunction. MAS is predominantly observed in girls and is rarely reported in males. We report the case of a 9-year old boy with gonadotropin independent precocious puberty, café-au-lait spots, polyostotic fibrous dysplasia and growth hormone hypersecretion.

Adenoma↗
PubMedDate not supplied

Pituitary adenoma in McCune-Albright syndrome: MR demonstration.

The magnetic resonance (MR) demonstration of a pituitary adenoma associated with McCune-Albright syndrome is presented. A useful role for routine MR imaging of the sella turcica in all cases of clinically suspected McCune-Albright syndrome is suggested. This is especially important in patients with leontiasis ossea secondary to polyostotic fibrous dysplasia involving the craniofacial bones and skull base.

Adenoma↗
PubMed1999

Cushing's syndrome caused by nodular adrenal hyperplasia in children with McCune-Albright syndrome.

McCune-Albright syndrome consists of fibrous dysplasia of bone, café-au-lait skin pigmentation, and endocrine dysfunction (usually precocious puberty). Other endocrine abnormalities occur in a minority of patients, and of these, Cushing's syndrome is the least often recognized. We present 5 children (4 girls) with features of McCune-Albright syndrome who had Cushing's syndrome in the infantile period (<6 months). In 2 children spontaneous resolution occurred, but the remaining 3 required bilateral adrenalectomy. In addition, all 4 girls have experienced precocious puberty, and 3 children demonstrated radiologic evidence of nephrocalcinosis. Understanding of the underlying defect causing McCune-Albright syndrome emphasizes the importance of searching for other endocrine dysfunction in these children.

Adolescent↗
PubMed2000

A comparative study of fibrous dysplasia and osteofibrous dysplasia with regard to Gsalpha mutation at the Arg201 codon: polymerase chain reaction-restriction fragment length polymorphism analysis of paraffin-embedded tissues.

Fibrous dysplasia and osteofibrous dysplasia are both benign fibro-osseous lesions of the bone and are generally seen during childhood or adolescence. Histologically, the features of these bone lesions sometimes look quite similar, but their precise nature remains controversial. Mutation of the alpha subunit of signal-transducing G proteins (Gsalpha), with an increase in cyclic adenosine monophosphate (cAMP) formation, has been implicated in the development of multiple endocrinopathies of the Albright-McCune syndrome and in the development of fibrous dysplasia. We studied Gsalpha mutation at the Arg201. codon in seven cases of fibrous dysplasia (six monostotic lesions and one polyostotic lesion) and seven cases of osteofibrous dysplasia using formalin-fixed, paraffin-embedded tissue, by means of polymerase chain reaction-restriction fragment length polymorphism and direct sequencing analysis. All of the seven cases of fibrous dysplasia showed missense point mutations in Gsalpha at the Arg201 codon that resulted in Arg-to-His substitution in three cases and Arg-to-Cys substitution in four cases. On the other hand, the seven cases of osteofibrous dysplasia and the normal bone used as a control showed no such mutation. These data suggest that fibrous dysplasia and osteofibrous dysplasia have different pathogeneses and that the detection of Gsalpha mutation at the Arg201 codon is quite useful for distinguishing between these lesions.

Adolescent↗
PubMed1988

Positive gallium-67 citrate uptake in a patient with polyostotic fibrous dysplasia.

Fibrous dysplasia is an uncommon bone condition with characteristic radiologic features. It is well known that there is increased uptake of Tc-99m hydroxymethylene diphosphonate (HMDP) and methylene diphosphonate (MDP) in fibrous dysplasia. There are no reports of uptake of Ga-67 citrate by fibrous dysplasia. A case is reported in which positive Ga-67 uptake was seen in a patient with polyostotic fibrous dysplasia.

Fibrous Dysplasia of Bone↗
PubMed2002

Normal vision despite narrowing of the optic canal in fibrous dysplasia.

BACKGROUND: Fibrous dysplasia of bone frequently involves the anterior base of the cranium and results in encasement of the optic-nerve canals. It has been assumed that such encasement leads to constriction and eventual blindness. There is controversy about whether patients should be regularly monitored or whether they should undergo prophylactic decompression of the optic nerve. This question is of particular concern in patients with normal vision, since the risks associated with surgical decompression include blindness. METHODS: We studied 38 patients with fibrous dysplasia of the lesser wing of the sphenoid bone. The patients underwent a detailed neuro-ophthalmologic examination and computed tomography of the face and skull, reformatted to measure the extent of involvement of the optic canal and the area of the canals. The results were compared with those of 38 age- and sex-matched controls. RESULTS: Of the 38 patients, 15 were male and 23 female, and their mean age was 26 years. Twelve had polyostotic fibrous dysplasia, and 26 had the McCune-Albright syndrome. Sixty-seven optic canals were affected by fibrous dysplasia; in 49 of them (73 percent) there was complete encasement. The mean (+/-SD) areas of the right and left canals were 9.6+/-3.8 mm2 and 9.9+/-3.6 mm2, respectively, in the patients, as compared with 12.0+/-2.9 mm2 and 11.9+/-2.7 mm2 in the controls (P=0.009 for the comparison of the right areas and P=0.03 for the comparison of the left areas by the paired t-test). In all but two of the patients, the results of neuro-ophthalmologic examination were normal. In the two patients with monocular visual impairment, the areas of the optic canals were similar on the normal and abnormal sides. CONCLUSIONS: Encasement of the optic canal in fibrous dysplasia causes narrowing of the canal, but that in itself does not result in visual loss. Therefore, prophylactic decompression of the optic nerve does not appear to be indicated on the basis of the presence of fibrous dysplasia on diagnostic images alone, since it does not correlate with visual loss.

Adolescent↗
PubMed1997

Maxillary malignant mesenchymoma and massive fibrous dysplasia.

This is the first report (to our knowledge) that describes a patient with massive polyostotic fibrous dysplasia involving the calvaria and facial skeleton that subsequently underwent transformation to a malignant mesenchymoma with elements of chondrosarcoma, osteosarcoma, and rhabdomyosarcoma arising in the maxilla. Malignant transformation occurred in the absence of prior radiation exposure, osteomyelitis, or known bony infarction. A review of the literature did not reveal any similar cases of massive fibrous dysplasia of the maxilla degenerating to multiple simultaneous malignant histotypes.

Adult↗