Oral and maxillofacial considerations in Gardner’s syndrome: a report of two cases

Abstract

Gardner’s syndrome (GS) is a genetic disorder characterised by intestinal polyps, multiple osteomas, and soft-tissue tumours. Dentists play an important role in the syndrome diagnosis considering that craniomaxillofacial osteomas are a major criteria for Gardner’s syndrome diagnosis. This study aimed to describe the main stomatological manifestation of GS and the importance of dentists in its diagnosis. Two patients presenting GS were evaluated. The first one had two osteomas in the mandible and GS was suspected. The colonoscopy confirmed the presence of polyposis and a prophylactic proctocolectomy was performed. The other patient had a late-stage diagnosis of GS and developed a rectum adenocarcinoma. The presence of craniomaxillofacial osteomas are a hallmark of the disease. Early-stage GS diagnosis may enable early diagnosis and preventive strategies in carriers. Other dental abnormalities, such as supernumerary teeth, hypercementosis and odontomas, can also be observed.

Gardner syndrome with maxillofacial manifestation: A case report

“…Ideal management of Gardner syndrome cases would require genetic counseling . Our patient did not have access to such evaluation, and we agree that this is a weakness in our report.…”

Genodermatoses with teeth abnormalities

“…Anodontia, conical and missing teeth, and short beaded hair are features of monilethrix (Vora, Anjaneyan, & Mehta, ). On the other hand, Gardner syndrome is characterized by polydontia, colon polyps, and an increased risk of colon cancer (Pereira et al, ). Enamel hypoplasia and dental caries are common in epidermolysis bullosa patients who also suffer from easy blistering of the skin and mucous membranes following minor trauma (Figure ; Wright, ).…”

Oral and Maxillo-Facial Manifestations of Systemic Diseases: An Overview

“…More than 50% of patients with familial adenomatous polyposis show osteomas in the oral and maxillofacial region (jaw and paranasal sinuses) which can considered typical signs. Less frequently is the association with odontomas, supernumerary teeth, focal increases in bone density within the jaws but such signs, often occasionally detected in not-still diagnosed patients or in young with an already known or not familial history, in the majority of cases represent the early clues of the disease ( Figure 17 a,b) [ 169 , 170 , 171 , 172 ].…”