Cases reported "Melorheostosis"

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1/75. 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. ( info)

2/75. 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. ( info)

3/75. melorheostosis: case report with radiologic-pathologic correlation.

    melorheostosis is an unusual mesenchymal dysplasia, which commonly presents on radiographs as longitudinal bars of hyperostosis in osseous structures. We present a case of melorheostosis in the lower extremity of a 20-year-old woman for which detailed radiologic- pathologic correlation was achieved due to amputation of the involved limb. ( info)

4/75. Progressive melorheostosis in the peripheral and axial skeleton with associated vascular malformations: imaging findings over three decades.

    A 28-year old woman presented with Leri's disease (melorheostosis) and the rare combination of complex vascular malformations and lymphatic anomalies. Multifocal melorheostosis was segmental and unilateral, located in the left axial and peripheral skeleton, fifth thoracic vertebral body, fifth rib. left upper limb and lumbosacral spine (third lumbar body to first sacral segment). Sacral involvement was associated with spinal canal stenosis. Additionally the patient had multiple nevi and had suffered from left hemiplegia since birth. Lymphangiectasia of the mesentery and thorax led to chylothorax resistant to therapy for which the patient underwent a pleuropericardiectomy. death ensued due to respiratory failure. ( info)

5/75. 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. ( info)

6/75. 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. ( info)

7/75. 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. ( info)

8/75. Para-articular ossifications associated with melorheostosis Leri.

    A case of para-articular ossifications associated with melorheostosis gave differential diagnostic problems, as the only symptom referred to the ossifications in the left knee region. ( info)

9/75. 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. ( info)

10/75. 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. ( info)
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