A case of Maffucci's syndrome with hemifacial hypertrophy.

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Maffucci's syndrome is a dyschondroplasia manifested by multiple hemangiomas and is generally considered a relatively rare disorder.Recently we encontered a case of Maffucci's syndrome with apparent hemihypertrophy of the face, jaws, teeth and tongue.<BR>A woman, 17 years old, presented with: generalized dark-red vascular naevus, distributed geographically and extensively; scoliosis; and asymmetry of the extremities, face, and head. Examination of the oral cavity revealed that the alveolar bones and tooth widths on the right were markedly larger than those on the left and that the maxillary center and raphe palati were deviated to the left with high palate, petechiae scattered on the palatal mucosa and lower gingiva on the lavial surface, and fibrous gingival hypertrophy. The tongue showed hypertrophy on the right half with the fungiform papillae hypertrophied like a strawberry. X-ray study revealed an accessory impacted tooth at the right premolar area.
特定非営利活動法人日本口腔科学会の論文