The importance of the dental examination in the primary diagnosis of Gardner's syndrome

Ömür Dereci; Sinan Ay; Esra Yesilova; Özgül Pasaoglu

doi:10.4103/2321-3841.157527

CASE REPORT

Year : 2015 | Volume : 3 | Issue : 2 | Page : 63-66

The importance of the dental examination in the primary diagnosis of Gardner's syndrome

Ömür Dereci¹, Sinan Ay¹, Esra Yesilova¹, Özgül Pasaoglu²
¹ Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Eskişehir Osmangazi University, Eskişehir, Turkey
² Department of Pathology, Faculty of Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey

Date of Web Publication

22-May-2015

Correspondence Address:
Dr. Ömür Dereci
Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Eskişehir Osmangazi University, Meşelik Campus, Eskişehir 26480
Turkey

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/2321-3841.157527

Abstract

Gardner's syndrome (GS) is an autosomal dominant, well-documented disease with characteristic systemic and maxillofacial manifestations. The diagnosis of GS is established by the presence of simultaneous findings of intestinal or colorectal polyposis and jaw pathologies. Multiple polyps of GS are likely to become carcinomas at older ages. Cooperation between medical disciplines is important in the diagnosis of GS. Patient with multiple adenoid polyposis should be referred to dental examination in order to discard GS. Dental radiographic examination may be crucial in the primary diagnosis of the disease. In this case report, a case of GS is presented with prominent histologic features and the importance of the dental examination in the diagnosis of GS is discussed.

Keywords: Familial intestinal polyposis, Gardner′s syndrome, osteoma

How to cite this article:
Dereci Ö, Ay S, Yesilova E, Pasaoglu Ö. The importance of the dental examination in the primary diagnosis of Gardner's syndrome. J Oral Maxillofac Radiol 2015;3:63-6

How to cite this URL:
Dereci Ö, Ay S, Yesilova E, Pasaoglu Ö. The importance of the dental examination in the primary diagnosis of Gardner's syndrome. J Oral Maxillofac Radiol [serial online] 2015 [cited 2023 Mar 24];3:63-6. Available from: https://www.joomr.org/text.asp?2015/3/2/63/157527

Introduction

Gardner's syndrome (GS) is an autosomal dominant disease which is characterized by hereditary intestinal polyposis and jaw pathologies such as impacted teeth, osteoma and cutaneous lesions. ^[1] GS is also known as familial adenomatous polyposis (FAP) and caused by a mutation in the adenomatous polyposis coli (APC)-gene. ^[2] The diagnosis of GS is based on simultaneous occurrence of innumerable precancerous adenomatous polyps in colon or rectum and miscellaneous jaw pathologies.

The maxillofacial manifestations of the disease are mostly asymptomatic and include compact osteomas with varying dimensions, enostoses, exostoses, odontomas, supernumerary teeth, impacted teeth and focal sclerotic bone islands. ^[2],[3]

Patients affected with GS become symptomatic at first and second decades of life with rectal bleeding, constipation, diarrhea and abdominal pain. Colorectal polyps inevitably transform into colorectal carcinomas if left untreated. Dental examination can be useful in the primary diagnosis of GS. In this case report, a case of GS which is diagnosed based on the dental panoramic and cone beam computerised tomography (CBCT) imaging techniques is presented.

Case Report

A 35-year-old female patient was referred to Eskişehir Osmangazi University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery for routine preprosthetic dental examination. In intraoral examination, a bony prominence was observed with bidigital palpation on left molar region of the mandible. Radiographic examination with panoramic radiograph revealed odontoma-like lesion on the right canine region and multiple sclerotic bone islands on mandible [Figure 1]. CBCT imaging was considered for further evaluation of simultaneous jaw pathologies. An odontoma at right mandibular canine region [Figure 2], a protuberated osteoma-like lesion on the left corpus of the mandible and dense osteosclerotic areas were observed in CBCT sections [Figure 3]. Medical anamnesis revealed that the patient had undergone colon excision operation with a diagnosis of multiple adenomatous polyposis. The provisional diagnosis of GS was made with obtained findings of maxillofacial multiple pathologic manifestations and history of adenomatous polyposis.

Figure 1: An odontoma is located on right mandibular canine region. Diffuse sclerotic areas can also be seen on the mandible

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Figure 2: Odontoma is clearly seen on the right canine region in the horizontal cone beam computerised tomography section of the mandible

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Figure 3: Frontal cone beam computerized tomography section shows early peripheral osteoma on the right corpus. Sclerotic bone changes are evident on the both sides of the mandible

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Patient was operated under local anesthesia for removal of odontoma and osteoma-like lesion. Odontoma was exposed with sulcular and vertical incisions and excised with the removal of circumferential folicular tissue [Figure 4]. Osteoma-like lesion was approached with a horizontal incision on the mucogingival junction of molar and second premolar teeth. Lesion was in its early stage and mildly protuberated [Figure 5]. It is removed with osteotomes and contour-shaping was performed with surgical burs. Surgical sites of odontoma and osteoma were irrigated with sterile saline solution and closed with 3-0 silk sutures. Surgical specimens were sent for pathologic examination [Figure 6].

Figure 4: Intraoperative image of the bone cavity after odontoma was removed

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Figure 5: Peripheral osteoma is surgically exposed with mucogingival incision and removed. Arrows show the borders of the lesion

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Figure 6: Postsurgical specimens of both lesions

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Examination of both surgical specimens microscopically confirmed the diagnoses of odontoma and osteoma [Figure 7] and [Figure 8]. Healing of the surgical sites was uneventful at 10 ^th day after surgery.

Figure 7: Histologic appearance of odontoma shows simialarity with a mandibular anterior tooth (H and E, ×40)

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Figure 8: Histological examination of cortical osteoma reveals cortical lamellar bone and several osteocytes in their lacunes (H and E, ×200)

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Discussion

Gardner's syndrome is also called FAP and characterized by multiple innumerable polyps in the colon and rectum with concomitant occurrence of jaw pathologies and dental abnormalities. ^[2] It is autosomal dominant and associated with the mutation of the APC tumor suppressor gene. ^[4]

Osteomas are the prominent jaw pathologies in GS. ^[4] Osteomas of GS differ in size and are between 3 mm and 4 cm in diameter. They may occur both in central and peripheral form. ^[5],[6] The presence of three or more osteomas on jaws is highly suggestive of GS. ^[7] Osteomas are mostly asymptomatic but they need the resection if they restrict mandibular movement, or the patient is visually displeased. ^[8],[9] There was an early peripheral osteoma on surface of the mandibular left molar buccal region in the present case. Osteoma was indistinct on panoramic radiography. However, it was evident in bidigital palpation of left molar region. Lesion was excised for eliminating further disfiguration of mandible.

Odontomas, supernumerary teeth, impacted teeth and sclerotic changes on jaw bones are also seen in GS. ^[4] Several authors reported that sclerotic radiographic masses on both jaws can be a distinctive sign of the disease and may be confused with fibro-osseous dysplasias. ^[10],[11] In our case, a compound odontoma was removed from the right mandibular canine region. There were also sclerotic changes on both two quadrants of mandible. However, no surgical intervention was considered.

The diagnosis of the GS is based on gastrointestinal endoscopy. Multiple adenomatous polyps, approximately 100 or more, are observed in colon or rectum of the affected patients. ^[4] If patients with GS are asymptomatic and not diagnosed until adultery, oral and maxillofacial manifestations become important for diagnosis. Colonal adenomas will inevitably progress into carcinomas unless they are treated until the age of 40. ^[4] Dental professional should be alert when examining the patient. Maxillofacial manifestations of GS mostly exhibit a syndromic appearance on dental pantograph with mixed dental abnormalities and pathologic conditions.

Panoramic radiography is commonly used in routine dental examination and can be useful in the early detection of the GS. CBCT provides more data regarding the extent and dimension of maxillofacial lesions. ^[12],[13] Therefore, CBCT imaging is an important tool to determine the exact location of maxillofacial lesions in GS. ^[13] There are few publications that report CBCT usage in the diagnosis stage of the disease. ^{[6],[11],[13]}

In the present case report, patient was unaware of her jaw bone pathologies and undergone into routine dental examination for dental implants. She had underwent colonal resection due to the multiple adenomas in the colon. However, she had not been referred to the dental practitioner. After dental examination in our clinic, a diagnosis of GS was established with the history of colonal resection and presence of dental findings. Patient was suggested that her children should be examined for the mutated APC-gene in a genetic facility.

Treatment of maxilllofacial manifestations of GS is surgical removal if the lesion is symptomatic. If limitation, decreased function or cyst formation is present, jaw lesions should be surgically curretated or removed. ^[3],[11] Asymptomatic lesions can be left untreated. However, they should be monitored in case they will become symptomatic. ^[13],[14]

Conclusion

Gardner's syndrome is diagnosed by simultaneous occurrence of multiple adenomatous polyposis of the colon and jaw pathologies such as odontomas, supernumerary teeth, compact or cancellous osteomas, osteosclerotic bone islands and impacted teeth. Dental practitioner should be aware of the maxillofacial manifestations of this entity. The simultaneous presence of odontomas, osteomas and impacted supernumerary teeth on jaws and a possible history of colonal resection should alert the clinician.

References

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