Oral and radiological characteristics of gardner syndrome patients: a report on three cases of members of the same family

Introduction: Gardner Syndrome is a dominant autosomal phenotypical variant of Familial Adenomatous Polyposis (FAP), due to mutations in the APC gene in the 5q21–22 chromosome. Its main features are multiple adenomatous colorectal polyps and subsequent elevated risk of malignization in the third and fourth decade. Patients with Gardner’s also feature dermoid and epidermoid cysts, sigmoid tumors and congenital hypertrophy of the retinal pigmentary epithelium. Dental and maxillofacial alterations include: mandibular osteomas, odontomas and multiple supernumerary teeth. The appearance of oral signs usually precedes the appearance of gastrointestinal signs.

Case report: Patient No. 1; 18-year-old female inquired about the absence of tooth 2.3, presented FAP and history of colectomy and removal of sebaceous cysts. Intraoral examination revealed only absence of 2.3 and prevalence of 8.3. Orthopantomography revealed multiple unerupted supernumerary teeth and odontomas impeding the eruption of 2.3 and 4.3. There were also areas of sclerosis in the mandibular body corresponding to enostosis or osteomas. These lesions did not alter the mandibular contour enough to be perceived through intraoral palpation. The patient will undergo orthodontic and surgical treatment to remove the odontomas and fenestrate 2.3 and 4.3. Two sisters and a cousin (patients no. 2, 3, 4) of patient no. 1 are also diagnosed with FAP and have colectomies. The radiological findings are similar to the above. Both sisters shall be treated orthodontically and surgically.

Conflict of interest: None declared.

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Feb 5, 2018 | Posted by in Oral and Maxillofacial Surgery | Comments Off on Oral and radiological characteristics of gardner syndrome patients: a report on three cases of members of the same family
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