Cases reported "Jaw Diseases"

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1/89. Intraosseous fibrous lesions of the jaws: a manifestation of tuberous sclerosis.

    Four patients previously diagnosed with tuberous sclerosis are reported with intraosseous fibrous lesions of the jaws. review of the literature revealed comparable pathosis occurring in extragnathic bones and several previous reports of similar lesions within the jaws. Therefore, these intraosseous fibrous proliferations are thought to represent an intraoral manifestation of tuberous sclerosis and not coincidental findings. In all 4 cases, the tumors demonstrated significant collagenization with numerous interspersed plump fibroblasts. Although histopathologically similar, the features of the lesions are not specific and also can be found in desmoplastic fibromas and simple odontogenic fibromas. The definitive diagnosis requires appropriate clinicopathologic correlation.
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2/89. erdheim-chester disease of the jaws: literature review and case report.

    erdheim-chester disease is a rare systemic lipogranulomatous disorder of adults that shares some histopathologic features similar to Langerhans' cell histiocytosis and that results in characteristic radiographic changes in the long bones. Relatively few cases have been reported in the jaws. We present a literature review of jaw cases and the first case report to describe detailed radiographic and pathologic features of jaw involvement, as well as clinical, radiographic, and histopathologic follow-up of the untreated jaw lesions.
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3/89. tooth eruption in a patient with craniometaphyseal dysplasia: case report.

    Craniometaphyseal dysplasia (CMD) is a very rare genetic disorder of bone remodeling caused by osteoclast dysfunction. The clinical and radiographical features of oral findings are presented in a sporadic case of CMD in a child (age 10 years, 7 months). An intraoral examination showed severe malocclusions, including anterior crossbite and deep bite. Furthermore, a radiographic examination showed increased radiopacity of the maxilla and mandibular bones due to hyperostosis and sclerosis of the jaw. There was no root resorption of the canines or molars in the primary dentition, although root formation of the permanent teeth was proceeding. Dental age was calculated to be approximately 1 year, 4 months younger than his chronological age. The eruption speed of the permanent lateral incisors after the gingival emergence was shown to be within normal values, and we discuss whether the canines and premolars in the permanent dentition could erupt or not.
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4/89. Gigantiform cementoma: clinicopathologic presentation of 3 cases.

    Gigantiform cementoma is a rare, benign fibro-cemento-osseous disease of the jaws, seen most frequently in young girls. Radiographically, it typically presents as multiquadrant, expansile, mixed radiolucent-opaque lesions that cross the midlines of the jaws. Although cases with a familial pattern are noted in a few publications, sporadic cases have been reported without a family history. The term gigantiform cementoma has been used interchangeably with designations of other fibro-osseous entities, yet its application should be restricted by the criteria defined herein. In this article, we report 3 cases of gigantiform cementoma. Clinical, radiographic, and microscopic features of these lesions are presented, along with criteria to differentiate gigantiform cementoma from other fibro-osseous diseases of the jaws. A possible pathogenetic mechanism and treatment recommendations are discussed.
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5/89. Langerhans cell granulomatosis: a case report of polyostotic manifestation in the jaw.

    We report the case of a 43-year-old woman who suffered from a polyostotic form of Langerhans cell granulomatosis (LCG) in the mandible and maxilla. The course of the disease was followed for more than 14 years. The disease's progression finally required subtotal resection of the mandible and reconstruction with a microvascular fibula graft. The literature is reviewed for diagnostic and treatment concepts based on the different stages of LCG.
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6/89. cherubism and its charlatans.

    cherubism is a rare hereditary condition characterised by progressive cystic proliferation of the mandible and maxilla in childhood, followed by post-pubertal involution of the process and jaw remodelling in adulthood. Its name is derived from the cherubic appearance that results from the jaw hypertrophy. Here, we present the case of a young boy with cherubism, in the context of his pedigree, to illustrate the clinical characteristics and their variable expression. We also seek to distinguish cherubism from central giant cell granuloma and giant cell tumour of the jaws, with which it holds a false synonymity.
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7/89. Neonatal permanent jaw constriction because of oral synechiae and Pierre Robin sequence in a child with van der Woude syndrome.

    OBJECTIVE: To report a newborn with van der Woude syndrome, Pierre Robin sequence, and oral synechiae. Pierre Robin sequence is a rare manifestation of van der Woude syndrome as are oral synechiae. We speculate that the oral synechiae may be causally related to the development of Pierre Robin sequence in this patient.
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8/89. Low-grade osteosarcoma of the jaw.

    We describe a case of low-grade osteosarcoma of the jaw. The patient is a 25-year-old woman who presented with large, nontender, slowly progressive, expansile left mandibular mass. X-ray showed a predominantly hypodense mass with erosive growth pattern affecting the angle, posterior body, and ramus of the left mandible. The patient underwent left hemimandibulectomy. The tumor was a white-tan, well-circumscribed, multinodular, firm mass that measure 7.0 cm. Microscopically, the tumor was composed of bland spindle cells, with minimal cellular atypia, absent mitotic figures, very focal osteoid formation with extensive bone destruction, and soft tissue infiltration. Immunhistochemical stains for a large panel of antibodies were noncontributory. At present the patient is alive and well 7 months after surgery.
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9/89. jaw claudication in primary amyloidosis: unusual presentation of a rare disease.

    We describe two patients with temporal artery biopsy-proven amyloidosis presenting with symptoms of jaw claudication, visual disturbance, and proximal muscle stiffness suggestive of giant cell arteritis (GCA) and polymyalgia rheumatica. At the onset of disease, neither patient had other characteristic symptoms to suggest primary amyloid. We point out similarities between GCA and primary amyloid that can lead to confusion in diagnosis.
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10/89. Reduced jaw opening from paradoxical activity of mandibular elevator muscles treated with botulinum toxin.

    The aim of the study was the effect of injections with botulinum toxin A (BTX-A) on reduced jaw opening, caused by paradoxical, antagonistic activity of jaw elevator muscles after brain stem lesions. The study included a male (51 years) and a female (69 years) patient. Subjective assessment, clinical recordings, muscle blocks and electromyography (EMG) were used to diagnose paradoxical activity, and to plan, guide and evaluate the treatment. The paradoxical innervation pattern was unilateral in the male and bilateral in the female. The paradoxical activity during jaw opening amounted to 24-109% of the level during maximum biting, and bursts of paradoxical activity were also present during chewing. EMG-guided blocks and later BTX-A injections of the affected muscles increased the opening by 9-23 mm from pre-treatment values of 15-18 mm, and normalized chewing. The study proved BTX-A to be an effective treatment for reduced jaw opening caused by paradoxical activity. Treatment was optimized by EMG evaluation of the current activity of the jaw elevator muscles, permitting individual treatment plans with longer intervals between BTX-A injections and lower doses than with conventional treatment for oromandibular dystonia. Thus the treatment only had to be repeated one to two times per year to maintain acceptable jaw mobility.
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