Cherubismの1症例(Familial fibro-osseous disease of the jaws)

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A case of Cherubism, 7-years old boy, complaining of bilateral painless protrusion of his face with bilateral enlargement of the submandibular lymph nodes was reported. In other members of the family, his mother had enlarged mandible, received a osteoplastic operation about 10 years ago. Other members of the family had normal face appearances.The protrusion of his face was first noticed at 1.5 years of age, the protrusion then developed rapidly until the age of about 4-years and resulted in deformity of the face. His speech was normal. All teeth were missing. Another prominent facial feature was the exposure of a rim of sclera between the lower lid and iris. But vision was normal. All protruded areas were firm, nottender and immobile. Submaxillary lymph nodes increased in size. Serum alkaline phosphotase was slightly elevated otherwise serologic findings, routine blood studies and urinalysis were within normal limits. Radiographic examination showed cystic changes in the maxilla and mandible. A skeletal survey revealed no abnormalities in the skeletal, other than that reported in maxilla and mandible. Biopsy of protruded area in maxilla revealed a fibrous dysplasia.The diagnosis of CHERUBISM was made on the basis of the family history, the clinical findings, roentogenographic appearance and pathologic findings. No active treatment has been given, but the child is being kept under periodic observation.This disease has been reported under a variety of terms such as familial fibrous swelling of the jaws, familial fibrous dysplasia of the jaws, familial intraosseous fibrous swellings of the jaws, familial incidence of bilateral giant cell tumors of the jaws, bilateral giant-celled tumors of the mandible in siblings, disseminated juvenile fibrous dysplasia of the jaws, familial osseous dysplasia of the jaws and cherubism. The term “CHERUBISM” probably best denotes the condition and the author believes it should be the preferred designation. The differrential diagnosis may include many lesions, such as benign giant cell tumor (Osteoclastoma), giant cell reparative granuloma, facial fibrous dysplasia, hyperparathyroidism.The author made some comments on cherubism (familial fibro-osseous disease of the jaws).

Cherubismの1症例(Familial fibro-osseous disease of the jaws)

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