Cases reported "Bone Diseases"

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1/57. Hemophiliacs bone pseudotumors.

    Four cases of proved hemophiliac pseudotumors caused by intraosseous bleeding are reported. Five lesions were found at the uncommon locations involving the cranial vault, mandible, phalanx, distal femur and distal tibia. The conventional radiographic and computed tomographic findings are expansile osteolytic destruction, cortical thinning, partial breaking cortex or pathological fracture, and sometimes associated soft tissue mass. Ultrasonographic feature of one case at the phalanx shows cortical expansion and thinning contained mixed echogenicity in the medullary canal with soft tissue extension. T99m DTPA of one case at the distal femur shows increased vascular flow and uptake at right distal tibia and left distal femur.
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2/57. Two sibs with an unusual pattern of skeletal malformations resembling osteogenesis imperfecta: a new type of skeletal dysplasia?

    We report a 6 year old boy with multiple fractures owing to bilateral, peculiar, wave-like defects of the tibial corticalis with alternative hyperostosis and thinning. Furthermore, he had Wormian bones of the skull, dentinogenesis imperfecta, and a distinct facial phenotype with hypertelorism and periorbital fullness. collagen studies showed normal results. His sister, aged 2 years, showed the same facial phenotype and dental abnormalities as well as Wormian bones, but no radiographical abnormalities of the tubular bones so far. The mother also had dentine abnormalities but no skeletal abnormalities on x ray. This entity is probably the same as that described in a sporadic case by Suarez and Stickler in 1974. In spite of the considerable overlap with osteogenesis imperfecta (bone fragility, Wormian bones, and dentinogenesis imperfecta), we believe this disorder to be a different entity, in particular because of the unique cortical defects, missing osteopenia, and normal results of collagen studies.
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3/57. Patient with Weismann-Netter and Stuhl (toxopachyosteosis) syndrome with communicant hydrocephalus and arachnoid cyst.

    Weismann-Netter and Stuhl (toxopachyosteosis) syndrome is a rare bone disease, manifesting with anterior bowing of the tibiae, short stature and mild mental retardation. We report a patient with Weismann-Netter and Stuhl syndrome with an unusual manifestation communicant hydrocephalus and arachnoid cyst.
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4/57. Bilateral metachronous periosteal tibial amyloid tumors.

    Localized primary periosteal amyloid tumors are extremely rare. A case of bilateral tibial amyloid tumor is presented. A 62-year-old woman initially presented with a painful mass in the anterior aspect of the right leg. There was no evidence of underlying systemic disease, including chronic infection or malignancy. Based on the results of resistance with congo red staining to treatment with potassium permanganate and positivity for kappa light chain, we classified this particular case as AL-type amyloidosis. The patient noticed a swelling in the opposite leg 2 years later. The second tumor was also an AL-type amyloidoma. Amyloid tumors are generally solitary. This is the first case of bilateral periosteal amyloid tumors of the AL-type occurring in the tibiae.
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5/57. Campomelic dwarfism.

    The campomelic syndrome is a short-limb, usually fatal, neonatal dwarfism. It is characterized by bowed lower limbs, especially the tibia, usually associated with a cutaneous dimple over the anterior skin. There is delayed calcification of the epiphyses and delayed mineralization of the spine and pelvis. The cartilages of the tracheobronchial tree are hypoplastic, contributing to death from respiratory problems. Other associated abnormalities are unusual appearing facies, cleft palate, absent olfactory nerves and talipes equinovarus. Less commonly seen are cardiac, renal and brain anomalies.
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6/57. erdheim-chester disease with extensive marrow necrosis: a case report and literature review.

    erdheim-chester disease is a rare systemic disorder characterized by a fibrosing xanthogranulomatous infiltration of multiple organs. We report a case of erdheim-chester disease with diffuse necrosis leading to difficulty in making a prompt diagnosis. Radiologically, osteosclerotic lesions with osteolytic element involved metadiaphyses of both proximal tibia, and retroperitoneal infiltrations encasing both kidneys, both adrenals, and aorta were found. A biopsy of the tibia showed diffuse infiltration of foamy histiocytes, Touton-type giant cells, and fibroblastic cells associated with extensive coagulative necrosis. Immunohistochemically, foamy histiocytes were positive for CD68 and peanut agglutinin and negative for S-100 protein. A few Langerhans' cells, which were difficult to identify in hematoxylin-eosin stain, were highlighted by immunostain for S-100 protein. The patient received supportive therapy and was alive 1 1/2 years after diagnosis, with newly developed bilateral retrobulbar lesions and worsened heart failure.
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7/57. erdheim-chester disease. A case report.

    A 63-year old man had a history of diabetes insipidus, arthralgias and myalgias, weight loss, relapsing fever and malaise. Increased uptake of Tc-99m was found predominantly in distal antebrachia, in distal femurs and in both trochanters and tibias on the bone scintigraphy. The chest radiograph showed reticulonodular pattern and the high resolution computed tomography (HRCT) scans revealed diffuse infiltrative lung disease with small multiple nodules and widening of interlobular septs. Videothoracoscopic lung biopsy and biopsy of tibial lesion were perfomed. The histopathologic examination proved non-Langerhans cell histiocytosis-erdheim-chester disease. Treatment with prednisone reduced the pain and fever and improved the vital capacity of the lungs while the changes in the lungs and bones remained unchanged.
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8/57. Congenital pseudarthrosis of the tibia: successful one stage transposition of the fibula into the distal tibia. A case report.

    An infant male presented at age 17 months with an established pseudarthrosis in a kyphoscoliotic right tibia. During the ensuing 6 years, three unsuccessful McFarland bypass bone grafts were performed. Then the distal end of the proximal fibula was placed into the medullary cavity of the proximal end of the distal tibia without disturbing the proximal portion of the fibula. This was augmented by autogenous bone graft. Union occurred in one year. The child has been followed for 7 years and the pseudarthrosis remains healed.
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keywords = tibia
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9/57. Tibial cyst formation after anterior cruciate ligament reconstruction using a new bioabsorbable screw.

    We report a case of tibial cyst formation twelve months after anterior cruciate ligament reconstruction using hamstring graft. A composite bioabsorbable interference screw made of 'beta tricalcium phosphate and poly L-lactide' fixed the graft distally. The patient presented with acute symptoms of pain and swelling over the proximal tibia. curettage of the cyst resulted in complete recovery within 3 months.
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10/57. Intraosseous proliferative sparganosis: a case report and review of the literature.

    Intraosseous proliferative sparganosis is an extremely rare parasitic disease in which the larvae of incomplete differentiated sparganum proliferate in the human bone. We present the first case of intraosseous proliferative sparganosis arising in the long bone. The patient was a 51-year-old man who complained of a slow growing painful mass on his right leg. The radiographic findings showed an infiltrative osteolytic lesion with speckled calcification at the proximal tibia the clinical diagnosis of which favored chondrosarcoma. Incisional biopsy revealed an innumerable number of small globular shapes, whitish parasites. Histologically, the parasites were composed of a few layers of smooth muscle and several calcerous bodies that were enclosed within a single row of tegumental cells. The latter exhibited a wavy appearance and coated with microvilli. These morphologic findings confirmed the nature of these maldifferentiated larvae. The patient was treated by partial resection of the lesion. This should remind clinicians that parasitic infection of the bone can produce a tumor-like lesion.
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