1/48. melorheostosis in a family with autosomal dominant osteopoikilosis.We describe a 19-year-old woman with melorheostosis and osteopoikilosis (mixed sclerosing bone dysplasia). Her sister and mother had osteopoikilosis, but no evidence of melorheostosis. Isolated melorheostosis and melorheostosis with osteopoikilosis are sporadic disorders. osteopoikilosis is an autosomal dominant trait. Mixed sclerosing bone dysplasia in a family with autosomal dominant osteopoikilosis raises the possibility that the two bone disorders may be related. This family and that of Butkus et al. [1997: Am J Med Genet 72:43-46] suggest that the melorheostosis could be due to a second mutation at the same locus as that which causes autosomal dominant osteopoikilosis.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
2/48. Cranio-facial and humeral melorrheostosis.First described by LERY and JOANNY in 1992, melorrheostosis is an uncommon linear hyperostosis of unknown aetiology, which may be associated with soft tissures changes. Although all bones may be affected, cranio-facial involvement is very rare. Only six such cases have been found in the literature. This report describes a case of cranio-facial and left humeral melorrheostosis with symptomatic radial nerve involvement. Resection of the melorrheostotic bone was performed because of involvement of the radial nerve.- - - - - - - - - - ranking = 0.66666666666667keywords = bone (Clic here for more details about this article) |
3/48. Histopathological characterization of melorheostosis.Melorheostotic bone was examined histopathologically. In the severely affected areas, an abundance of osteoid and increased angiogenesis was observed. Increased osteoid without mineralization indicated the overproduction of bone matrix. Bone resorption also appeared to increase because osteoclasts were numerous in melorheostotic bone, thus suggesting a high rate of bone turnover. In addition, transforming growth factor-beta was immunolocalized in the periosteal fibroblasts, mesenchymal cells surrounding vessels, endothelial cells, and osteoblasts, while basic fibroblast growth factor was found in endothelial cells and mast cells near vessels. These cytokines may have some association with the exuberant bone matrix production and angiogenesis in melorheostosis.- - - - - - - - - - ranking = 1.6666666666667keywords = bone (Clic here for more details about this article) |
4/48. magnetic resonance imaging depiction of tight iliotibial band in melorheostosis associated with severe external rotation deformity, limb shortening and patellar dislocation in planning surgical correction.We present the case of a 14-year-old male with melorhesotosis and severe iliotibial band tightness which was associated with femoral shortening, severe external rotational deformity of the femur, genu valgum and patellar dislocation in the right lower extremity. Skeletal survey revealed irregular radiodense streaks involving the pelvis, femoral head, femoral shaft, distal femoral epiphysis, talus and middle phalangeal bones of the foot. Magnetic resonance (MR) imaging showed thickening of the iliotibial band in addition to low MR signal changes in the bone. Intraoperatively fibrosis in the subcutaneous layer and a thickened iliotibial band were found. MR images were very useful in understanding the soft tissue pathoanatomy in melorheostosis and planning surgical correction.- - - - - - - - - - ranking = 0.66666666666667keywords = bone (Clic here for more details about this article) |
5/48. melorheostosis: unusual presentation in a girl.melorheostosis is a rare bone disease of unknown etiology. It is characterized by tense and erythematous skin, contractures and shortening of the affected limbs. Radiographs reveal hyperostosis in long bones resembling melting wax, and spotty or patchy endostic deposits in short bones. An 11-year-old girl showed, since birth, contractural deformities in the right hand without bone abnormalities. At the age of 12 months, she complained of soft tissue tightness in the right buttock. At 3 years, limitation of flexion and extension of the right knee was evident. Radiographs revealed findings typical of melorheostosis.- - - - - - - - - - ranking = 7.7492273917877keywords = bone disease, bone (Clic here for more details about this article) |
6/48. melorheostosis with occlusion of dorsalis pedis artery.melorheostosis is an unusual sclerotic dysplasia of bone. The case of a 51-year-old female patient with melorheostosis and occlusion of the dorsalis pedis artery is described. Although numerous vascular anomalies have been noted in patients with melorheostosis, occlusion of the dorsalis pedis artery has not been reported previously.- - - - - - - - - - ranking = 0.33333333333333keywords = bone (Clic here for more details about this article) |
7/48. osteopoikilosis: report of a clinical case and review of the literature.osteopoikilosis is an uncommon benign sclerosing bone dysplasia. Herein we present a case of osteopoikilosis with characteristic roentgenographic findings and clinical findings of cervical myelopathy. The importance of the differential diagnosis in symptomatic cases of osteopoikilosis is emphasized with the review of the literature.- - - - - - - - - - ranking = 0.33333333333333keywords = bone (Clic here for more details about this article) |
8/48. osteosarcoma arising in a femur with melorheostosis and osteopathia striata.Osteopathia striata is an asymptomatic autosomal dominant or sporadically inherited disorder that causes dense striations at sites of endochondral bone formation, with a predilection for the metaphyses of long bones. melorheostosis is a mixed sclerosing dysplasia with disturbance of both endochondral and intramembranous ossification, in which disordered intramembranous ossification dominates. It presents typical radiological changes of cortical hyperostosis distributed along a sclerotome with variable associated cutaneous and clinical features. Overlap syndromes including one or more of these diseases are described. We report a 44-year-old man with both melorheostosis and osteopathia striata who presented with pain secondary to superimposed osteosarcoma. In reporting this case we discuss the relationship between sclerosing dysplasia and either coexisting or complicating sarcoma.- - - - - - - - - - ranking = 0.66666666666667keywords = bone (Clic here for more details about this article) |
9/48. Atypical form of active melorheostosis and its treatment with bisphosphonate.We present the case of a 38-year-old man in whom extensive bilateral melorheostosis was associated with elevated serum alkaline phosphatase, swelling of the right foot and progressive deformity of the left hand, left leg and right foot. radiography, computed tomography and bone scintigraphy were performed. Following treatment with bisphosphonate (30 mg/day of pamidronate for 6 days) infusion, the pain and swelling of his right foot showed improvement and his elevated serum alkaline phosphatase decreased.- - - - - - - - - - ranking = 0.33333333333333keywords = bone (Clic here for more details about this article) |
10/48. Mixed sclerosing bone dysplasia--a case report with literature review.We report a case of mixed sclerosing bone dysplasia in a 26-year-old man. This is a very rare disorder characterized by a variable combination of melorheostosis, osteopoikilosis and osteopathia striata. The disease may be generalized or may show unilateral involvement.- - - - - - - - - - ranking = 1.6666666666667keywords = bone (Clic here for more details about this article) |
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