A case of basal cell nevus syndrome.

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We saw a patient who had multiple jaw cysts of deaf-mutism with a cleft lip and cleft jaw, and had several examinations on suspicion of a basal cell nevus syndrome. The patient, an 18-year-old woman was first seen at the First Department of Oral Surgery, Fukuoka Dental College, because of swelling in labial gingiva of the region from the left mandibular canine to the right mandibular canine. Roentgenographic examinations disclosed cystic radiolucencies in maxilla and mandible. Further, general examination revealed frontal bossing, a broad nasal root, hypertelorism, right heterochromie, numerous pigmental spots at the left neck, palmar and plantar pits in both palms, scoliosis and scoliokyphosis. Endocrinal and chromosomal examination showed nothing particular. Cysts were enucleated and their histopathological diagnoses were odontogenic keratocyst. She had satisfactory prognosis without relapse. We think it necessary to have a long term follow-up.
社団法人日本口腔外科学会の論文