1/24. A rare case of polyostotic fibrous dysplasia assessed by bone scintigraphy with Tc-99m methylene diphosphonate (MDP).A 66-year-old man affected by polyostotic form of fibrous dysplasia in consequence of worsening of lower extremity bone pain aggravated by walking and concomitant increase of serum alkaline phosphatase and osteocalcin, was subjected first to a radionuclide study. Bone scan demonstrated a pathological uptake of the radiotracer in the craniofacial bones, right scapula, left and right posterior ribs, right hemipelvis and lower extremities confirming the diagnosis but establishing especially the extent of bone involvement, greater than expected on the basis of symptoms and X-ray findings, underlying the importance of nuclear medicine imaging in the assessment and follow-up of this rare disease.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
2/24. A case of atypical McCune-Albright syndrome requiring optic nerve decompression.McCune-Albright syndrome (MAS) is a disease of noninheritable, genetic origin defined by the triad of cafe-au-lait pigmentation of the skin, precocious puberty, and polyostotic fibrous dysplasia. This syndrome, which affects young girls primarily, has also been reported with other endocrinopathies, and rarely with acromegaly and hyperprolactinemia. The fibrous dysplasia in MAS is of the polyostotic type and, apart from the characteristic sites such as the proximal aspects of the femur and the pelvis, the craniofacial region is frequently involved. A male patient with MAS presented with juvenile gigantism, precocious puberty, pituitary adenoma-secreting growth hormone and prolactin, hypothalamic pituitary gonadal and thyroid dysfunction, and polyostotic fibrous dysplasia causing optic nerve compression. Visual deterioration and its surgical management are presented.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
3/24. Familial polyostotic fibrous dysplasia.A case of polyostotic fibrous dysplasia of the craniofacial type is presented, together with substantial evidence that this condition had a genetic basis in this patient. A review of the literature indicates that there is absolutely no previous evidence of a genetic basis to this condition. The possibility that the propositus suffered from polyostotic fibrous dysplasia of the Jaffe or the Albright type was excluded.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
4/24. Craniofacial fibrous dysplasia complicated by acute, reversible visual loss: report of two cases.We report two cases of craniofacial fibrous dysplasia which presented with acute visual loss. The first patient had a sphenoid sinus mucocele compressing the optic chiasm. In the second patient the optic canal was narrowed by dysplastic bone. In both cases optic nerve decompression restored vision to normal.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
5/24. Craniofacial fibrous dysplasia associated with primary hyperparathyroidism.Isolated presentation of fibrous dysplasia or primary hyperparathyroidism is a common finding. Only few cases of craniofacial dysplasia associated with hyperparathyroidism have been reported in the literature. A case of a patient with fibrous dysplasia of craniofacial bones associated with primary hyperparathyroidism without additional endocrinophaties or associated disorders, will be presented. Beside the facial dysmorphism caused by dysplasia, the only clinical symptoms were due to the primary hyperparathyroidism.- - - - - - - - - - ranking = 2keywords = craniofacial (Clic here for more details about this article) |
6/24. frontal sinus mucocele: a rare complication of craniofacial fibrous dysplasia.We present plain radiographic, computed tomographic and magnetic resonance imaging (MRI) findings in a 25-year-old female patient with craniofacial fibrous dysplasia (FD). Although FD has a tendency to involve craniofacial bones in a unilateral fashion, the involvement was bilateral and extensive in this case. An additional feature was the presence of a frontal sinus mucocele, presumably due to the involvement of the sinus recess by the dysplastic process. This complication of the craniofacial FD has been reported very infrequently in the literature.- - - - - - - - - - ranking = 7keywords = craniofacial (Clic here for more details about this article) |
7/24. Craniofacial surgery and optic canal decompression in adult fibrous dysplasia.A 29-year-old female had a 3-year history of bony swelling over the right frontal area. For 3 months she noticed proptosis of her right eye. Investigations revealed fibrous dysplasia involving the right half of the frontal bone and the right greater and lesser wings of the sphenoid bone. Visual evoked potentials (VEP) showed delayed latencies on the involved side. A craniofacial surgery with optic canal decompression was performed. Follow-up after 2 years revealed normalization of VEP.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
8/24. Unilateral exophthalmos associated with severe fibrous dysplasia.Fibrous dysplasia is a benign developmental anomaly of bone, often affecting craniofacial bones. We report on a 9-year-old boy, who presented for routine checkup. Clinical examination revealed unilateral exophthalmos of his left eye without decrease of visual acuity or double vision. Radiologic studies showed characteristic extensive changes of fibrous dysplasia involving the left frontal bone, left orbital bones, maxillary and sphenoid bones. The patient was started on conservative therapy and the condition of the affected eye remained stable. As fibrous dysplasia of the orbital bones can be a cause of significant dysfunction and a treatable cause of blindness, early diagnosis is very important. These patients are most likely to present with complaints of facial asymmetry, including axial, vertical, or horizontal displacement of the globe, or visual loss. Therefore the ophthalmologist plays an important role in the early diagnosis of fibrous dysplasia.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
9/24. A case of McCune-Albright syndrome associated with Gs alpha mutation in the bone tissue.The syndrome of McCune-Albright syndrome (MAS) is clasically defined as a triad presentation with the findings of polyostotic fibrous dysplasia, cafe-au-lait spots, and sexual precocity. However, not all patients present with complete symptoms. A 52-year-old man was diagnosed as having a variant of McCune-Albright syndrome with the following findings: polyostotic fibrous dysplasia, acromegaly due to pituitary tumor and subclinical hyperthyroidism due to toxic multinodular goiter. Sexual precocity and cafe-au-lait spots were not noted. acromegaly was confirmed by laboratory examination (IGF-1, glucose suppression test and TRH stimulation test). Long acting somatostatin analogue was used as treatment. Although the pituitary tumor could not be removed due to technical problems, mass lesions on the cranium were removed subtotally. Histopathological evaluation demonstrated that the lesion complied with fibrous dysplasia. Genomic DNAs were isolated from the craniofacial bones and peripheral leucocytes of the patient. After amplifying the related regions, Gs alpha (Gs alpha) gene was analysed by automatic dna sequence analysis. An activating mutation of the Gs alpha gene (Arg 201 Cys) was found in the genomic dna isolated from the bone tissue of the patient, but not in the genomic dna isolated from the blood. We described a case of MAS associated with Gs alpha mutation in the bone tissue, presenting with polyostotic fibrous dysplasia, subclinical hyperthyroidism and acromegaly.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
10/24. Surgical treatment of nasal obstruction secondary to craniofacial fibrous dysplasia.Craniofacial fibrous dysplasia, a skeletal disorder most often affecting the mandible or maxilla, can cause a variety of complications. nasal obstruction secondary to this unusual disorder presents a diagnostic and therapeutic challenge. The nasal obstruction in this case is due to expansion of fibrous lesions in adjacent bones as well as direct involvement of the turbinates with fibrous dysplasia. We describe the surgical correction of nasal obstruction in this unique setting and present a brief update of this intriguing disorder.- - - - - - - - - - ranking = 4keywords = craniofacial (Clic here for more details about this article) |
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