Multiple odontogenic keratocysts associated with Gorlin-Goltz syndrome.
Gorlin-Goltz syndrome or Nevoid basal cell carcinoma syndrome is an autosomal dominant disorder with a predisposition to cancer. Features like basal cell carcinoma, odontogenic keratocysts, calcification of falx cerebri, bifid ribs, pits on palms and soles and hypertelorism are evident. A case of this rare disease seen on a 13 year old female patient is presented here, where multiple odontogenic keratocysts were causing disfigurement of the lower jaw as well as displacement and malocclusion of the lower teeth.
Dixit
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Dental Department, Kathmandu Medical College, Sinamangal, Nepal. siddharthdxt@gmail.com
Acharya
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Dixit
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D000293:Adolescent; D001478:Basal Cell Nevus Syndrome / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery; D005260:Female; D005489:Focal Dermal Hypoplasia / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D008336:Mandibular Diseases / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery; D009807:Odontogenic Cysts / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging* / Q000601:surgery; D035583:Rare Diseases; D018570:Risk Assessment; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome