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A case of monostotic fibrous dysplasia of a long bone of the hand is reported. There has been only one similar case recorded, though it is not uncommon in other long bones. The diagnosis was proved by histology, and the tumour treated by subperiosteal excision. There has been no recurrence after 31 months, though in similar cases excision including the periosteum has been advised to reduce the likelihood of recurrence.
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An aneurysmal bone cyst (ABC) in the paranasal sinuses is rare with only four previous cases having been described in the sphenoid and ethmoid sinuses. This report describes a case of an ABC involving the sphenoid and ethmoid sinuses as well as the upper nasal fossae. It is currently believed that a preexisting primary bone lesion, such as fibrous dysplasia, initiates an osseous arteriovenous fistula, which creates a secondary reactive lesion of bone, the ABC. These lesions, which are comprised of multiple cysts containing liquid blood and serous fluid, are optimally studied by a combination of CT and MR scans.
The purpose of this report is twofold. First, to discuss fibrous dysplasia of bone as it relates to the neurosurgeon, and second to present a case of fibrous dysplasia of the skull with progressive involvement of the optic and trigeminal nerves. Of special interest in this case is the progression of the disease after puberty and the technical problems of decompression of the involved nerves. A combined approach to the lesion with otolaryngological assistance was undertaken.
It is extremely necessary to find an ideal filling material for the treatment of benign bone defect, particularly those benign osteolytic defect in children with a comparatively large cavity. From July, 1995 to December, 1996, 10 cases of benign osteolytic bone defects were treated, in which 5 cases of benign osteolytic defects, 3 cases of bone cysts, 1 case of fibrous dysplasia of bone and 1 case of non-ossifying fibroma. After through curettage of the cavity, the natural non-organic bone in cube-shaped was used to fill the prepared cavity. The results showed that the activities of the patients resumed normal in 3 to 6 months after operation. The roentgenographic examination after operation showed that the lucent spaces between NNB cubes disappeared with obvious new bone formation and the bony cavity was obliterated with newly-formed bone. There was no recurrence of the lesion during a follow-up of 7 to 24 months. It was concluded that to treat the osteolytic defect of bone with NNB was a method of choice. The new bone formation appeared early in large amount and increased rapidly.
OBJECTIVE: To evaluate the clinical results of repair of bone defect by embryonic bone transplantation. METHODS: From January 1994 to June 1999, 148 cases of bone defect were repaired by embryonic bone transplantation following alcohol treatment, there were 63 cases with bone cyst, 42 cases with fibrous dysplasia of bone, 26 cases with giant cell tumor of bone, and 17 cases with enchondroma among them. The maximal bone defect was 3.5 cm x 10.0 cm, while the minimal defect was 0.5 cm x 1.0 cm. RESULTS: All of those bone defect with benign tumor were bone union used by embryonic bone transplantation after 3 months to 1 year of operation, the average healing course was 6.2 months, followed up 1 to 6 years, averaged 14 months, no tumor recurrence and no obvious local or system response were observed. CONCLUSION: Embryonic bone can be used as a good repairing material of postoperative bone defect of benign tumors, the clinical results are satisfactory.
A patient with polyostotic fibrous dysplasia had several fractures of the right lower limb. An above-knee amputation was eventually performed, followed by arthrodesis of the hip. Five years later the stump became painful and swelled with dramatic rapidity. Biopsy showed that this was not due to malignant change, but that an aneurysmal bone cyst had developed in association with the fibrous dysplasia.
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An 18 year old man with fibrous dysplasia of bone and several endocrine abnormalities was investigated. Laboratory tests showed high levels of prolactin, TSH and GH and low levels of testosterone and LH. TRH and GnRH showed normal responsiveness of the pituitary in spite of the high hormonal levels. The circadian rhythms disclosed no abnormalities of hypothalamic control. The neuroradiological study revealed pathological tissue but no positive sign of a pituitary adenoma, although the sella turcica polytomograms did suggest erosion of the floor. Our data are compatible with the presence of a mixed adenoma or adenomas of the pituitary. We emphasize the importance of a correct study, including neuroradiological and neuroendocrine investigations, in patients with fibrous dysplasia of bone.
AIMS: To examine outcomes of pamidronate treatment on fibrous dysplasia of bone in three children with McCune-Albright syndrome (MAS). METHODS: Radiological evidence of fibrous dysplasia progress was reviewed for three children with MAS who were treated with pamidronate from age 2.5-5 years, for 8-10.5 years. RESULTS: Despite minimal pain and a low fracture rate in long bones, except where gross deformity exists, all dysplastic lesions present in long bones continued to undergo uncontrolled expansion. In contrast, there were no major new changes in facial configuration, no clinically obvious expansion of sphenoid wing lesions and no encroachment on optic foramina or visual field restriction in any patient. CONCLUSIONS: Despite previous reports of limitation or reduction in size of fibrous dysplasia lesions in adults and children, it is our experience that bisphosphonate treatment of polyostotic fibrous dysplasia in children with MAS does not arrest the expanding nature of these lesions.
The case of fibrous dysplasia localised in the maxilla in 7 years old girl was described. Attention was paid to etiology, symptoms and treatment. The differential diagnosis was discussed. The diagnosis was wet set on the bases of clinical examination, X-ray, scyntygraphy and histological examination.
At a time when significant attention is devoted worldwide to stem cells as a potential tool for curing incurable diseases, fibrous dysplasia of bone (FD) provides a paradigm for stem cell diseases. Consideration of the time and mechanism of the causative mutations and of nature of the pluripotent cells that mutate in early embryonic development indicates that, as a disease of the entire organism, FD can be seen as a disease of pluripotent embryonic cells. As a disease of bone as an organ, in turn, FD can be seen as a disease of postnatal skeletal stem cells, which give rise to dysfunctional osteoblasts. Recognizing FD as a stem cell disease provides a novel conceptual angle and a way to generate appropriate models of the disease, which will continue to provide further insight into its natural history and pathogenesis. In addition, skeletal stem cells may represent a tool for innovative treatments. These can be conceived as directed to alter the in vivo behavior of mutated stem cells, to replace mutated cells through local transplantation, or to correct the genetic defect in the stem cells themselves. In vitro and in vivo models are currently being generated that will permit exploration of these avenues in depth.