[Fibrous Dysplasia, Polyostotic].
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Skeletal changes leading to a suspicion of the presence of a tumour frequently occur in childhood with the roentgenological manifestation of a cyst. X-ray morphology can differ depending upon the localisation and the course. In childhood, however, such findings are mainly classified as tumour-like bone lesions. This group comprises, inter alia, the juvenile bone cyst, the aneurysmatic bone cyst and fibrous dysplasia. However, it is necessary to exclude by differential diagnosis--even though the main age of manifestation is after completion of growth--genuine bone tumours with cystic phenomena, such as the giant cell tumour, chondroma or chondroblastoma. Verification of the diagnosis can be effected via radiologic-diagnostic methods such as tomography. The use of scintigraphy of the skeleton can likewise be indicated. Numerous laboratory parameters can be used in individual cases to exclude certain diagnoses. Taking these aspects into consideration, the article reviews differential diagnosis of the most frequent skeletal affections in childhood. Great emphasis is given to the ranking and importance of the individual diagnostic methods.
The fibrous dysplasia of the bones is a big disease, but it has to be considered as an expansive process with local malignancy in the cranio-maxillo-facial localizations. After a review of the literature, 7 cases of fibrous dysplasia are presented. The only curative treatment is surgical, generally mutilating, it has to be completed by a restoring intervention in same time. The abstention and the modeling surgery can be indicated exceptionally, it requires a regular X ray examination, and a clinical follow-up.
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Scintigraphic manifestations of fibrous dysplasia were analyzed in 59 lesions of 26 patients (12 monostotic, 14 polystotic). Bone imaging with Tc-99m MDP revealed a high percentage of increased uptake of radioisotope in the lesions of fibrous dysplasia. Four (14%) of 29 cystic lesions and two (7%) of 30 lesions with the appearance of ground glass showed no increase in radioisotope uptake, although roentgenograms showed marked changes. Therefore, care must be taken in the diagnosis of fibrous dysplasia with bone imaging alone. Nuclear methods, however, are indispensable in evaluating the dynamic aspects of bone mineral behavior and in demonstrating disease where none was suspected, or in visualizing polyostotic involvement in those cases where only monostotic disease was suspected clinically. It is concluded that both scintigrams and roentgenograms are complementary procedures in the diagnosis of fibrous dysplasia.
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Three applications of free vascularised bone grafts are described. Free fibular grafts have been performed in 19 cases of avascular necrosis of the femoral head. The grafts are placed along the anterior femoral neck and into the femoral head with a microvascular anastomosis between the nutrient vessels of the fibula and the anterior circumflex pedicle of the hip. The operative technique is described but the post-operative follow-up is still too short to draw any definite conclusions. This operation has been performed in young patients in whom the only alternative would be a total hip replacement. Free vascularised transfers of iliac crest bone are described for bone defects such as infected non-unions of the tibia with soft tissue and bony loss. Free vascularised fibula grafts have also been employed with cancellous grafting for cystic defects such as fibrous dysplasia and large bone cysts. The use of free vascularised fibula grafts is also described in four cases of congenital pseuodarthrosis of the tibia.
Computed tomography has become the primary radiological procedure to evaluate disease of the orbit, to localize it exactly and to demonstrate its effect on neighbouring structures. In the past few years, considerable progress has resulted from improvement in the equipment, especially in three dimensional visualization of the area by introduction of the coronal and sagittal planes of section either indirectly by computer reconstruction of the coronal and sagittal plane from data obtained from routine transverse scanning or directly by appropriate positioning of the patient in these planes. Three dimensional CT technique is essential for precise localisation, delineation and differentiation of combined lesions of the face, the skull base and the orbits. Although by all this improvement of CT the possibility for exact diagnosis has increased, conventional neuroradiological examinations are still often necessary as a complimentary study to CT, especially in disease which involves the bone.
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