Gardner's Syndrome

Abstract

Gardner's syndrome is an autosomal dominant disease and is a subtype of familial adenomatous polyposis. It is characterized by adenomatous intestinal polyps, multiple osteomas in the skull, maxillae, mandible, and multiple cutaneous and subcutaneous masses (epidermoids and desmoid). Intestinal polyps, if not treated, have 100% chance of becoming malignant. We report a case of a 25-year-old female patient with Gardner's syndrome, with clinical manifestations including impacted supernumerary teeth, odontomes, sebaceous cyst on the scalp, and osteomas. It is important for the general dental practitioners to be aware of the clinical and radiological characteristics of Gardner's syndrome.

Keywords: Familial intestinal polyposis; gardner's syndrome; impacted teeth; osteoma.

Figure 1

Extraoral osteomas. A 25-year-old female patient with diffuse bilateral swellings on the lower third of face along the mandibular border.

Figure 2

Postoperative radiograph after removal of osteomas, impacted teeth, and implant placement, with respect to tooth 21.

Figure 3

Posteroanterior radiograph shows extra-oral osteomas along the mandible (white arrows).

Figure 4

OPG demonstrates impacted teeth 21 and 23, homogenous radiopaque masses over the right and left inferior aspect of the mandible suggestive of osteomas (large arrows). Also multiple small, homogeneous radiopaque masses surrounded by a radiolucent halo could be seen throughout the maxilla and mandible suggestive of complex odontomes (small arrows).Diffuse sclerotic masses are present throughout the body of mandible giving it a mottled appearance

Figure 5

Maxillary occlusal radiograph shows impacted teeth 21, 23 (large arrows) and odontomes (small arrows) in the near vicinity.

Figure 6

Photomicrograph shows the presence of compact lamellar bone with haversian canal, lacunae, histiocytes and reversal and resting lines suggestive of osteoma. (Hematoxylin-Eosin stain 10×).