가족성 섬유성 이형성증(Cherubism)에 대한 고찰

A REVIEW ON CHERUBISM

Cherubism is an uncommon, benign fibrosseous lesion of the jaws that causes a progressive, painless, symmetrical expansion of the maxilla and mandible. It's autosomal dominance pattern of inheritance has been confirmed. A characteristic deformity is specific to this disease: hypertrophy of the mandible, swelling of cheeks and sometimes hypertrophy of maxilla with eyes tending to look up ; that looks like the Renaissance cherubs. It usually makes figures during childhood between 2-4 years of age and progresses until puberty, after which it spontaneously regresses 10 most cases. As a result of this case review, the management strategies for cherubismic children in standpoint of pediatic dentistry can be summarized as follows: 1. It can be detected early in childen through its characteristic clinical and radiographic features, which is confirmed by histopathological examination and familial history. 2. The supervision of arch space is required against its frequent sequelae, the early missing of primary teeth and eruption disorders of permanent teeth. 3. It is highly recommended to continue the periodic check-up with clinical and radiographic examination, leading to surgical intervention in cases of aggravation.