1/20. Enlargement of mandibular canal without hypesthesia caused by extranodal non-Hodgkin's lymphoma: a case report.A rare condition of enlargement of the mandibular canal caused by an extra-nodal non-Hodgkin's lymphoma in a 59-year-old Japanese woman was reported. The patient had a swelling of the hard palate and protrusion of both ocular bulbs, which had been present for 10 years. A panoramic radiograph revealed that the right mandibular canal was widely enlarged, extending from the mandibular foramen to the mental foramen, without bone destruction. The continuous dilation of the mandibular canal to an approximate 15-mm width was associated with peripheral bony sclerosis. Computed tomography and magnetic resonance imaging showed a soft tissue tumor inside the mandibular canal. The lesion demonstrated expansive growth in the orbits, extending to the skull base through the superior orbital fissures and cavernous sinus. The lymphoma was suspected to have grown so slowly that the adjacent mandibular canal and ocular bulbs enlarged without destroying the normal bone and nervous tissue.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
2/20. Concomitant sickle cell disease and skeletal fluorosis.Skeletal fluorosis typically manifests as a diffuse increase in bone density, whereas avascular necrosis of the epiphyses and diaphyseal marrow are the main skeletal manifestations of sickle cell disease. The diagnostic and therapeutic challenges raised when both disorders are present are illustrated by two cases in Senegalese patients from an area characterized by high fluoride contents in the water and soil. Both had SS sickle cell disease. Skeletal fluorosis was diagnosed during evaluation for avascular necrosis in one patient and in the wake of septic arthritis in the other. Femoral head necrosis is difficult to identify in a patient with skeletal fluorosis. The bone lesions due to sickle cell disease and those due to fluorosis can mimic other bone diseases, most notably metastases. The combination of sickle cell disease and fluorosis results in significant medullary canal narrowing due to cortical thickening and to accumulation of necrotic bone. When performing hip replacement surgery, careful reaming of the medullary canal may reduce the risk of iatrogenic femoral fracture and inappropriate stem placement.- - - - - - - - - - ranking = 0.22222222222222keywords = canal (Clic here for more details about this article) |
3/20. multiple myeloma presenting with widespread osteosclerotic lesions.Sclerotic lesions are rare in malignant monoclonal gammopathies, although they are occasionally associated with poems syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes). In most cases, osseous lesions in poems syndrome present as an isolated sclerotic deposit or a combination of both lytic and sclerotic lesions. Diffuse osteosclerosis is extremely rare and may lead to the diagnosis of multiple myeloma, classically known to present as lytic lesions in the skeleton, with or without diffuse osteoporosis. We report a 74-year-old woman with widespread and substantial osteosclerotic lesions, associated with IgA-lambda myeloma, and with no other criteria for poems syndrome, and who was rapidly diagnosed with compression of the spinal cord. Detailed knowledge of imaging features in myeloma emphasises the need to consider plasma cell neoplasm in the differential diagnosis of any pattern of bone sclerosis. Although exceptional, multiple myeloma must be borne in mind in the presence of diffuse bone sclerosis.- - - - - - - - - - ranking = 0.038565824009823keywords = spinal (Clic here for more details about this article) |
4/20. Osteopathia striata with cranial sclerosis: clinical, radiological, and bone histological findings in an adolescent girl.Osteopathia striata with cranial sclerosis (OS-CS) is a rare skeletal dysplasia characterized by linear striations of the long bones, osteosclerosis of the cranium, and extra-skeletal anomalies. We provide a comprehensive description of the skeletal phenotype in a French-Canadian girl with a moderate to severe form of sporadic OS-CS. Multiple medical problems, including anal stenosis and the Pierre-Robin sequence, were evident in the first few years of life. At 14 years, she was fully mobile, with normal intellect and stature. She suffered chronic lower extremity pain in the absence of fractures, as well as severe headaches, unilateral facial paralysis, and bilateral mixed hearing loss. Biochemical indices of bone and mineral metabolism were within normal limits. Bone densitometry showed increased areal bone mineral density in the skull, trunk, and pelvis, but not in the upper and lower extremities. An iliac bone biopsy specimen revealed an increased amount of trabecular bone. Trabeculae were abnormally thick, but there was no evidence of disturbed bone remodeling. In a cranial bone specimen, multiple layers of periosteal bone were found that covered a compact cortical compartment containing tightly packed haversian canals. Bone lamellation was normal in both the iliac and skull samples. Osteoclast differentiation studies showed that peripheral blood osteoclast precursors from this patient formed functional osteoclasts in vitro. Thus, studies of bone metabolism did not explain why bone mass is increased in most skeletal areas of this patient. Cranial histology points to exuberant periosteal bone formation as a potential cause of the cranial sclerosis.- - - - - - - - - - ranking = 0.11111111111111keywords = canal (Clic here for more details about this article) |
5/20. Bilateral visual loss in craniodiaphysial dysplasia.PURPOSE: To report a rare case of craniodiaphysial dysplasia (CDD) that resulted in a profound loss of vision in both eyes. DESIGN: Observational case report. methods: A 2-year-old girl presented with midfacial anomaly. Marked thickening and sclerosis in the calvaria and facial bones were detected on the plain x-rays, which were compatible with CDD. Two years later, she visited our clinic because of visual loss in both eyes. RESULTS: The visual acuity was light perception in both eyes. The optic disk swelling with temporal pallor was observed in her both eyes. Orbital computed tomography scan revealed near-total obliteration of the optic canal in both eyes. CONCLUSIONS: CDD is a severe bone disorder characterized by massive generalized hyperostosis and sclerosis, especially involving the facial bones. Bony encroachment on the cranial foramina causes optic nerve compression, and this may lead to progressive visual impairment and ultimately to blindness.- - - - - - - - - - ranking = 0.11111111111111keywords = canal (Clic here for more details about this article) |
6/20. Osteosclerotic myeloma with spinal leptomeningitis and severe polyneuropathy: A case report.Osteosclerotic myeloma is a rare plasma cell disorder often associated with a polyneuropathy. We describe a young patient with progressive polyneuropathy associated with vertebral bone marrow lesions and unusual meningeal infiltration at cauda level. The diagnosis of osteosclerotic myeloma was confirmed by bone marrow biopsy findings including the chromosome 13q14 deletion in 37% of the cells. Leptomeningeal involvement was demonstrated by spine magnetic resonance imaging with gadolinium administration and fat-suppression technique.- - - - - - - - - - ranking = 0.15426329603929keywords = spinal (Clic here for more details about this article) |
7/20. Osteosclerotic myeloma.A 49-year-old man with an osteosclerotic lesion of a thoracic vertebra had peripheral neuropathy, lymphadenopathy, and elevated serum alkaline phosphatase and monoclonal immunoglobulin levels in the cerebrospinal fluid. These findings, not usually seen in ""classical'' myeloma, have been noted in different combinations in the osteosclerotic variety.- - - - - - - - - - ranking = 0.038565824009823keywords = spinal (Clic here for more details about this article) |
8/20. Endemic fluorosis with spinal cord compression. A case report and review.We report a case of spinal cord compression in a Mexican immigrant due to vertebral osteosclerosis from chronic fluoride intoxication. Endemic fluorosis is acquired through drinking water. groundwater sources with high fluoride content occur worldwide. The epidemiology, metabolism, and clinical features of fluorosis are reviewed. Greater physician awareness of this entity is important to identify correctly patients with this unusual and potentially devastating clinical disorder.- - - - - - - - - - ranking = 0.19282912004912keywords = spinal (Clic here for more details about this article) |
9/20. Autosomal dominant osteosclerosis associated with familial spinal canal stenosis.We studied a family with autosomal dominant osteosclerosis associated with familial spinal canal stenosis. The propositus, a 44-year-old Japanese woman, had a 9-month history of occipitalgia and left tinnitus, and also had a 2-month history of pain and numbness of the right upper limb. Radiographic skeletal survey showed osteosclerotic changes in the neurocranium, diaphysis of the long bone, mandible, shoulder, clavicle, and ribs. serum alkaline phosphatase was normal, and no periosteal excrescences were seen. The inheritance pattern was autosomal dominant. The propositus and her daughter, both with severe osteosclerosis, showed spinal canal stenosis, but her son, whose osteosclerosis was moderate, did not. This is the first report of autosomal dominant osteosclerosis associated with familial spinal canal stenosis.- - - - - - - - - - ranking = 11.984564961144keywords = spinal canal, canal, spinal (Clic here for more details about this article) |
10/20. Sclerosteosis involving the temporal bone: histopathologic aspects.Sclerosteosis is a rare, potentially lethal, autosomal recessive, progressive craniotubular sclerosing bone dysplasia with characteristic facial and skeletal features. The temporal bone changes include a marked increase in overall size, extensive sclerosis, narrowing of the external auditory canal, and severe constriction of the internal auditory meatus, fallopian canal, eustachian tube, and middle ear cleft. Attenuation of the bony canals of the 9th, 10th, and 11th cranial nerves, reduction in size of the internal carotid artery, and severe obliteration of the sigmoid sinus and jugular bulb also occur. Loss of hearing, generally bilateral, is a frequent symptom. It often manifests in early childhood and initially is expressed as sound conduction impairment. Later, a sensorineural hearing loss and loss of vestibular nerve function often develop. Impairment of facial nerve function is another feature occasionally present at birth. In the beginning, a unilateral intermittent facial weakness may occur which eventually progresses to a bilateral permanent facial paresis. The histologic examination of the temporal bones from a patient with sclerosteosis explains the mechanisms involved in the progressive impairment of sound conduction and loss of cochlear, vestibular, and facial nerve function. There is a decrease of the arterial blood supply to the brain and an obstruction of the venous drainage from it. The histopathology reveals the obstacles to decompression of the middle ear cleft, ossicular chain, internal auditory and facial canals, and the risks, and in many instances the contraindications, to such procedures. On the other hand, decompression of the sigmoid sinus and jugular bulb should be considered as an additional life-saving procedure in conjunction with the prophylactic craniotomy recommended in all adult patients.- - - - - - - - - - ranking = 0.44444444444444keywords = canal (Clic here for more details about this article) |
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