Delayed dental development in a patient with Gorlin syndrome: case report.

G orlin syndrome is a relatively uncommon en-¯ tity which was first reported by Ancell in 1841 in a patient with multiple benign cysts of the jaws.~ In 196'5, this condition took on its current name when Gorlin catalogued the related findings of 150 patients , 2 the most common of which were multiple nevoid basal cell carcinomas of the neck and face, rib and spine abnormalities, multiple cysts of the jaws, and lemellar calcifications of the falx cerebri. 3 Since that time, the condition has been variously known as Gorlin-Goltz syndrome, Nevoid Basal Cell Carcinoma syndrome, and Basal Cell Nevus syndrome) This case presents an additional previously unreported finding of delayed dental development in a patient with Gorlin syndrome. Literature review Gorlin syndrome is inherited as an autosomal dominant trait with a high degree ofpenetrance and variable expressivity. 5 The disorder has a population prevalence of 1 per 57,000 people, with a 3:1 male sex predilec-tion. 6 There are more than 40 different diagnostic findings associated with Gorlin syndrome. The best known feature is the presence of multiple basal cell carcinomas, which typically appear between puberty and 35 years of age, 7 covering the trunk, neck, cheeks, nose, and eyelids of affected individuals. 3 The number of these lesions can vary from a few to several thousand , varying in size from 1 to 10 mm in diameter. 7 The second most common diagnostic finding in Gorlin syndrome is the presence of multiple odonto-genic keratocysts (OKCs), which occur in up to 75% of all cases. These cysts have a peak occurrence in the second and third decade of life and are more common in the body and ramus of the mandible (80%) than the maxilla. The OKCs may vary in size, be found singularly or in groups, and be unilateral or bilateral. 5 In young patients, the OKCs can cause displacement of developing permanent teeth, expansion of the bone, be associated with an unerupted tooth, and occasionally cause root resorption. 6 Radiographically, the OKC appears as a well-cir-cumscribed radiolucency with smooth or scalloped margins and a thin opaque border. They may be either unilocular or multilocular in appearance. 5 The OKCs have been reported to have a recurrence rate of between 20 and 60%, with a peak recurrence rate within the first 5 postoperative years. 8 Treatment of odontogenic keratocysts usually includes complete enucleation with aggressive curettage to ensure …