抄録
The basal cell nevus syndrome (Gorlin-Goltz syndrome) is an autosomal dominant disorder. It is characterized by multiple basal cell nevi, multiple odontogenic keratocysts and skeletal abnormalities (ex. bifurcated rib, scoliosis).
Few reports of its clinical features have been published in the oto-rhino-laryngological literature. The diagnostic criteria are not yet clear, except in patients with co-existing skin anomalies and keratocysts. A 20-year-old male visited our clinic with a chief complaint of pain and swelling around the right bucca. He had multiple jaw cysts with an impacted tooth, cysts in both maxillary sinuses with an impacted tooth, protrusion of the frontal bone, a broad nasal root, increased distance between the inner canthi of the eyes, and mental retardation.
The histologic diagnosis of multiple jaw cysts and maxillary cysts was odontogenic keratocysts. So far, there has been no recurrence since the operation.
