Cases reported "Cherubism"

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1/5. cherubism: clinicopathologic features.

    A case of cherubism in 6-year-old boy is reported. He presented with bilateral symmetrical enlargement of the jaw in addition to medially dislocated premature teeth, narrow V-shaped palatal vault, and mild upward turning of the eyes. Radiographs showed multiloculated osteolysis in both the mandible and maxilla. histology revealed a non-neoplastic fibrous lesion, rich in multinucleated giant cells, consistent with giant-cell reparative granuloma. Since the original description of cherubism, various histologic interpretations have been proposed, particularly that of fibrous dysplasia. However, it should be emphasized that cherubism is a disease histologically indistinguishable from giant-cell reparative granuloma.
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keywords = dysplasia
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2/5. cherubism and its orbital manifestations.

    cherubism is a rare, fibroosseous lesion of the jaws that may have orbital manifestations of proptosis, lower eyelid retraction, superior globe displacement, and visual loss. Management of the orbital tumors in two cases from the same family is described. The original description of the disorder is reviewed. The inheritance pattern is autosomal dominant. pathology findings show multinucleated giant cells in a vascular fibrous stroma. Teeth are often missing or displaced. Radiographic studies show symmetric cystic lesions in the jaws and sometimes involvement of the orbital floor and lateral orbital wall. Fibrous dysplasia is in the differential diagnosis, but can be distinguished by clinical characteristics. Treatment may involve curettement or recontouring of the bony lesions.
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keywords = dysplasia
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3/5. Fibrous dysplasia and cherubism as an hereditary familial disease. Follow-up of four generations.

    Five cases of dysplasia of the jaws in one family which has been under our observation since 1970 are reported. The disease appeared as a mixed display of jaw lesions, in some members as fibrous dysplasia and in others as cherubism. We were able to trace the disorder through an unbroken line of four generations, and thus to demonstrate autosomal dominant inheritance. cytogenetic analysis performed on three members of this family revealed a significantly increased rate of chromosomal breakage.
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ranking = 6
keywords = dysplasia
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4/5. cherubism. Histo-enzymological and ultrastructural study.

    The 3 cases of cherubism reported affected the mandible. They were all studied by means of histo-enzymological and ultrastructural methods. This study demonstrated 3 stages in the morphological evolution of the disease, corroborated by clinical data. The 1st stage was characterized by an osteolytic granuloma with round, fusiform and giant-cells and a high level of activity of acid phosphatase. The 2nd stage showed repair with proliferation of highly active fibroblasts (increase in activity of leucine aminopeptidase). The 3rd stage exhibited an osteogenesis with high activity of alkaline phosphatase and ATPase. The pathogenesis of this rare osteodysplasia is discussed.
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keywords = dysplasia
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5/5. cherubism: a radiological and clinical presentation.

    cherubism is the hereditary form of the fibrous dysplasia of the jaws, but it may be seen sporadically as well. The disease has a self-limited nature and is rarely apparent before the age of two. There is no need to interfere surgically with these lesions of the mandible or the maxilla unless the child is severely affected, i.e. the disease deteriorates respiration, deglutition, vision, or the psychiatric makeup of the child due to cosmetic reasons. The clinical presentation and radiological evaluation of these children are so typical that the pediatrician and pediatric otolaryngologist need to be informed about this rarely seen disease. A case of a cherubic child, with his clinical appearance as well as his radiological evaluation, and discussion about the clinical outcome are presented.
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ranking = 1
keywords = dysplasia
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