Imaging Sci Dent.  2016 Dec;46(4):267-272. 10.5624/isd.2016.46.4.267.

Gardner syndrome associated with multiple osteomas, intestinal polyposis, and epidermoid cysts

• Koh KJ
• Park HN
• Kim KA

• KMID: 2362745
• DOI: http://doi.org/10.5624/isd.2016.46.4.267

Abstract

Gardner syndrome is known as a variant of familial adenomatous polyposis. This syndrome is characterized by multiple intestinal polyposes, osteomas, and epidermoid cysts. In addition, dental abnormalities include an increased frequency of multiple odontomas, as well as supernumerary and impacted teeth. The authors report the case of a 7-year-old male patient with Gardner syndrome. Radiographic findings revealed multiple osteomas in both sides of the maxilla, multiple diffuse enostoses in both jaws, and a complex odontoma in the left mandibular body. Two years later, multiple epidermoid cysts on the scalp were found. Since this patient was suspected to have Gardner syndrome, the authors recommended gastrointestinal endoscopy to check for intestinal polyposis. Gastrointestinal endoscopic examination revealed multiple polyposes in the upper gastrointestinal tract and fundus of the stomach. As a result, the final diagnosis was Gardner syndrome.

Keyword

Gardner Syndrome; Osteoma; Adenomatous Polyposis Coli; Radiography, Dental

MeSH Terms

Adenomatous Polyposis Coli
Child
Diagnosis
Endoscopy, Gastrointestinal
Epidermal Cyst*
Gardner Syndrome*
Humans
Intestinal Polyposis*
Jaw
Male
Maxilla
Odontoma
Osteoma*
Radiography, Dental
Scalp
Stomach
Tooth, Impacted
Upper Gastrointestinal Tract

Figure

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  Fig. 1 A panoramic image shows relatively well-defined multiple radiopacities in both maxillary posterior regions encroaching the maxillary sinuses (black arrows), a well-defined round radiopacity surrounded with a radiolucent line in the left mandibular premolar area (white arrow), and multiple diffuse radiopacities in both jaws.

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  Fig. 2 A post-enhanced coronal computed tomography image shows multiple mildly enhanced soft tissue masses on the subcutaneous areas of the scalp.

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  Fig. 3 A. An axial cone-beam computed tomography (CBCT) image shows multiple radiopaque masses in the right maxilla encroaching the maxillary sinus (black arrows). B. A coronal CBCT image shows multiple diffuse radiopacities in the mandible (white open arrows), and multiple radiopacities encroaching the maxillary sinuses and ethmoidal sinuses (black arrows).

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  Fig. 4 A photograph of the patient's scalp shows multiple nodules on the scalp.

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  Fig. 5 Histopathologic examination of the nodules on the scalp shows a cystic wall with lining epithelium (H&E stain, ×200).

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  Fig. 6 A. An endoscopic examination shows multiple polyposes on the upper gastrointestinal tract. B. Endoscopic examination showing multiple polyposes on the fundus of the stomach.

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  Fig. 7 A. A photomicrograph of the decalcified section of the radiopaque mass in right maxillary posterior region shows dense and lamellar cortical bone with narrow bone marrow spaces (H&E stain, ×50). B. A photomicrograph of the decalcified section of radiopaque mass in the left mandibular body shows irregularly deposited cementum, dentin, and enamel matrix (H&E stain, ×50).

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  Fig. 8 A. Two years after removal of the complex odontoma in the left mandibular body and osteoma in the right tuberosity, a panoramic image shows the remaining radiopaque lesions in both sides of the maxilla and diffuse enostoses in both jaws (white open arrow). B. A sagittal cone-beam computed tomography image shows multiple enostoses with impaction of the left mandibular second premolar (white open arrow).

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