A Case Report of Multiple odontogenic keratocysts associated with basal cell nevus syndrome.
- Author:
June Ho BYUN
1
;
Seong Hee PARK
;
Uc Kyu KIM
;
Hae Ryoun PARK
;
In Kyo CHUNG
Author Information
1. Department of Oral & Maxillofacial Surgery, College of Dentistry, Pusan National University.
- Publication Type:Case Report
- Keywords:
basal cell nevus syndrome;
multiple odontogenic keratocysts
- MeSH:
Basal Cell Nevus Syndrome*;
Crowns;
Dentigerous Cyst;
Genes, Dominant;
Humans;
Hypertelorism;
Jaw Cysts;
Nevus;
Odontogenic Cysts*;
Tooth, Unerupted
- From:Journal of the Korean Association of Oral and Maxillofacial Surgeons
2000;26(3):305-309
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
The basal cell nevus syndrome is a well recognized entity, the major symptoms of which are basal cell nevi, multiple jaw cysts, skeletal anomalies, and ectopic calcification. The syndrome follows a hereditary pattern, which is characterized by a highly penetrant, autosomal dominant gene with multiple and variable effects. The patient often has a characteristic face, with frontal and temporoparietal bossing, which results in an increased cranial circumference. The eyes may appear widely separated, and 40 percent of patients have true ocular hypertelorism. Jaw cysts are one of the most constant features of the syndrome and are present in at least 75 percent of the patients. The cysts are odontogenic keratocysts and frequently multiple. Radiographically, the cysts in patients with basal cell nevus syndrome do not differ significantly from isolated keratocysts. The cysts in patients with this syndrome are often associated with the crowns of unerupted teeth; on radiographs they may mimic dentigerous cysts. We report a case of multiple odontogenic keratocysts associated with basal cell nevus syndrome with the literature of review.
