Cases reported "Fibrous Dysplasia of Bone"

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11/315. Visual loss associated with fibrous dysplasia of the anterior skull base. Case report and review of the literature.

    The authors present a case of visual loss associated with fibrous dysplasia of the anterior skull base and the surgical management of this case. Preoperative computerized tomography scanning in this patient demonstrated a patent optic foramen and a rapidly growing cystic mass within the orbit, which was responsible for the patient's visual loss. A literature review revealed that this case is typical, in that cystic mass lesions of various types are frequently responsible for visual loss associated with fibrous dysplasia. The authors did not find significant evidence in the literature to support the notion that visual loss associated with fibrous dysplasia is the result of progressive optic canal stenosis, thus raising questions about the value of prophylactic optic canal decompression. Instead, as demonstrated by this case and those uncovered in the literature review, most instances of visual loss result from the rapid growth of mass lesions of cystic fibrous dysplasia, mucoceles, or hemorrhage. Findings of the literature review and the present case of fibrous dysplasia of the anterior skull base support a role for extensive surgical resection in these cases and indicate a need for additional prospective analysis of a larger number of patients with this disease.
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12/315. Cystic fibrous dysplasia mimicking giant cell tumor: MRI appearance.

    We report the case of a 43-year-old man who presented with an osteolytic and expansive lesion in the left distal femur mimicking a giant cell tumor. Magnetic resonance imaging (MRI) showed that most of the lesion was cystic, and histological examination revealed fibrous dysplasia with marked cystic degeneration. Radiographic findings of cystic fibrous dysplasia in the end of a long bone may be similar to those of a giant cell tumor, and a biopsy is essential for the final diagnosis.
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keywords = dysplasia
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13/315. Fibrous dysplasia of the temporal bone: report of a case and a review of its characteristics.

    Fibrous dysplasia is an uncommon benign disorder of unknown etiology. It represents a disturbance of normal bone development--specifically a defect in osteoblastic differentiation and maturation that originates in the mesenchymal precursor of the bone. Because fibrous dysplasia shows a predilection for the facial and cranial bones, where it causes deformity and dysfunction, the disease is of particular interest to the otolaryngologist. In this paper, we report a case of fibrous dysplasia of the temporal bone, the first symptom of which was a mixed hearing loss. We discuss the characteristic features of this specific location of the disease, the differential diagnosis, and the treatment policy. We also address the issue of secondary sensorineural hearing loss.
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ranking = 0.77777796202616
keywords = dysplasia, defect
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14/315. Fibrous dysplasia arising from the calcaneus.

    A case of an 18-year-old woman with fibrous dysplasia arising in the calcaneus, which is extremely rare, is reported, with the emphasis placed on differential diagnosis from low-grade central osteosarcoma. She had a severe pain in her left ankle after sprain. Plain radiographs showed a radiolucent lesion measuring 6.3 x 2.5 cm with a sclerotic margin in the left calcaneus. CT scans showed a well-defined lytic lesion with disruption of the lateral cortex and an ossification or calcification in its center. On MR imaging, the lesion had isointensities and high intensities on T1 and T2 weighted images, respectively, but its central portions showed lower intensities both on T1 and T2 weighted images. The lesion was enhanced with gadolinium except for the central portions. The specimen obtained by open biopsy consisted of fibrous tissue and foci of irregular woven bone. None of the nuclear atypia, mitoses, longitudinal stream of bone or invasive nature of growth was detected. The diagnosis of fibrous dysplasia was histologically made. The lesion was curetted and packed with autogenous bone chips. No evidence of recurrence was noted postoperatively.
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keywords = dysplasia
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15/315. Focal fibrocartilaginous dysplasia of the femur.

    Two additional cases of femoral fibrocartilaginous dysplasia are reported. The condition produces such significant angulation of the articular surfaces of the knee that progressive deformity is of concern to parents and surgeon alike. In both the reported cases, corrective distal femoral osteotomy confirmed the histologic diagnosis and ensured satisfactory gait in later childhood.
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ranking = 0.55555555555556
keywords = dysplasia
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16/315. Two stage reconstruction for the Shepherd's crook deformity in a case of polyostotic fibrous dysplasia.

    Polyostotic fibrous dysplasia leads to progressive and disabling deformity involving the proximal femur. Conventional methods of treatment have been ineffective in controlling this problem. Two stage reconstruction was carried out in a case of polyostotic fibrous dysplasia with bilateral shepherd's crook deformity. Bilateral subtrochanteric osteotomies with intramedullary fixation in the first stage and intertrochanteric osteotomies in the second stage with nail plate fixation was done to provide definitive control of the deformity. Bone graft was not used.
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ranking = 0.66666666666667
keywords = dysplasia
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17/315. Repeated dedifferentiation of low-grade intraosseous osteosarcoma.

    Low-grade intraosseous osteosarcoma is an uncommon bone tumor that is characterized by minimum cytological atypism and a much better prognosis than conventional osteosarcoma. This report describes a patient who had a low-grade osteosarcoma that mimicked fibrous dysplasia (FD). The tumor had an area of high-grade sarcoma at the initial diagnosis. Ten years after incomplete resection of FD-like tumor, local recurrence with areas of high-grade tumor developed. This case illustrates the potential of dedifferentiation in low-grade intraosseous osteosarcoma.
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keywords = dysplasia
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18/315. Focal fibrocartilaginous dysplasia of long bones: report of eight additional cases and literature review.

    We report eight additional cases of focal fibrocartilaginous dysplasia (FFCD) in the proximal tibia (five), distal ulna (one), and distal femur (two). Spontaneous, complete resolution of the lesion was observed in two tibiae and one ulna. Three tibial lesions with genu varum deformity were managed with osteotomy. Two femoral FFCDs caused persistent or progressive deformity: one genu valgum with patellar dislocation, and one genu varum. These patients underwent concomitant deformity correction and lengthening by the Ilizarov method. The final results were satisfactory in all patients except one, who underwent valgus tibial osteotomy and developed mild postoperative genu valgum. The analysis of a total of 46 cases in the literature and our experience suggests that (a) FFCD has a wide histopathologic spectrum, ranging from purely dense, fibrous tendon-like tissue to benign fibrocartilaginous tissue; (b) at least 45% of tibial FFCD demonstrates progressive, spontaneous resolution; (c) in contrast, femoral and humeral FFCDs appear to have a slim possibility of spontaneous regression of the deformity; and (d) corrective osteotomy is indicated when the deformity is increasing or persistent, or when the existing deformity is severe enough to jeopardize adjacent joint mechanics and alignment.
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ranking = 0.55555555555556
keywords = dysplasia
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19/315. A rare, radiographic 'sunray' appearance in fibrous dysplasia.

    We report a case of a 19-year-old Thai woman who had fibrous dysplasia (FD) with a rare radiographic 'sunray' appearance. The sunray appearance could have been due to either an unusual pattern of calcification within the lesion or a periosteal reaction.
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ranking = 0.55555555555556
keywords = dysplasia
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20/315. Management of aggressive midface and orbital fibrous dysplasia.

    OBJECTIVE: To discuss cosmetic and functional implications in the evaluation and treatment planning of large, aggressive midfacial fibrous dysplasias. patients AND methods: Eight patients (aged 2-38 years) with large fibrous dysplasias of the maxilla, zygomatic, and ethmoid bones requiring varying degrees of intervention and reconstruction were retrospectively reviewed. patients with smaller lesions of these regions not requiring resection and reconstruction, as well those requiring sinus surgery alone were excluded from this review. RESULTS: All fibrous dysplasias in this review were monostotic, 6 originating in the maxilla, 1 in the zygoma, and 1 in the ethmoid. Five lesions (4 maxillary, 1 zygomatic) caused cosmetic deformity without functional deficits and required resection and/or contouring only with minimal reconstruction. The remaining lesions were invasive such that function of the eye and/or dentition was affected. These lesions were treated by aggressive resection and various degrees of reconstruction to optimize function. CONCLUSIONS: While fibrous dyplasia is classified as a benign process, local expansion can cause significant functional and aesthetic deformities. Each lesion should be thoroughly evaluated and, when vital structures are involved or threatened, total or subtotal resection should be considered. A variety of options should be available to the surgeon for definitive primary reconstruction.
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keywords = dysplasia
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