Bone and dental abnormalities as first signs of familial Gardner’s syndrome in a Chinese family: a literature review and a case report

Gardner’s syndrome (GS) is an autosomal dominant disease characterized by the presence of familial adenomatous polyposis (FAP) as well as extraintestinal manifestations such as osteomas, dental anomalies, epidermoid cysts and ocular abnormalities. These intestinal polyps carry a 100% risk of malignant change, so early diagnosis is crucial. As craniofacial osteomas and dental anomalies of GS usually precede gastrointestinal symptoms, otolaryngologists, oral surgeons and dentists play an important role in the diagnosis of GS. GS is extensively reported in literature in the Caucasian race but not in the Mongoloid race. We report a case of a 22-year-old patient with a manifestation of three features of GS - multiple osteomas, soft tissue tumors and dental anomalies in the craniofacial region, with no intestinal polyps at the time of reporting. A family pedigree with our patient as the proband was constructed and revealed 3 consecutive generations in his lineage with GS.

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Paranasal Sinus Osteomas and Gardner's Syndrome

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348940711600906 ◽

2007 ◽

Vol 116 (9) ◽

pp. 658-662 ◽

Cited By ~ 24

Author(s):

Alan A. Z. Alexander ◽

Alpen A. Patel ◽

Rick Odland

Keyword(s):

Paranasal Sinus ◽

Positive Family History ◽

Gastrointestinal Symptoms ◽

Soft Tissue Tumors ◽

Benign Tumors ◽

Gardner's Syndrome ◽

Colon Polyps ◽

Gardner’S Syndrome ◽

Dominant Disease ◽

History Of

Objectives: Osteomas are common benign tumors of the paranasal sinuses. The origin of these lesions is uncertain. Although most are asymptomatic, symptoms can include headaches, facial pain, rhinorrhea, and sinusitis. Osteomas are also seen as part of Gardner's syndrome, an autosomal dominant disease characterized by intestinal polyposis, osteomas, and cutaneous and soft tissue tumors. In affected individuals, the risk of developing colon cancer approaches 100%. On average, osteomas are detected 17 years before colon polyps appear. Methods: Three patients with maxillary or ethmoid osteomas and chronic sinusitis are presented. Results: One of the patients had evidence of Gardner's syndrome, based on the presence of gastrointestinal symptoms and a positive family history of polyposis. Conclusions: Otolaryngologists should be aware of the possibility of Gardner's syndrome in patients with paranasal sinus osteomas. Suspected patients should have a complete workup for Gardner's syndrome, including lower gastrointestinal tract endoscopy, barium enema imaging, and DNA testing.

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Gardner's Syndrome

Journal of Clinical Imaging Science ◽

10.4103/2156-7514.92187 ◽

2011 ◽

Vol 1 ◽

pp. 65 ◽

Cited By ~ 21

Author(s):

Sapna Panjwani ◽

Anjana Bagewadi ◽

Vaishali Keluskar ◽

Saurabh Arora

Keyword(s):

Autosomal Dominant ◽

Clinical Manifestations ◽

Gardner's Syndrome ◽

Adenomatous Polyposis ◽

Supernumerary Teeth ◽

Gardner’S Syndrome ◽

Sebaceous Cyst ◽

Intestinal Polyps ◽

Dental Practitioners ◽

Dominant Disease

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.

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Gardner’s syndrome (literature review and case report)

Pelvic Surgery and Oncology ◽

10.17650/2686-9594-2020-10-2-47-52 ◽

2020 ◽

Vol 10 (2) ◽

pp. 47-52

Author(s):

I. L. Chernikovskiy ◽

D. N. Korobkov ◽

N. V. Savanovich ◽

D. A. Chernikov ◽

P. V. Melnikov ◽

...

Keyword(s):

Case Report ◽

Soft Tissue ◽

Literature Review ◽

Soft Tissue Tumors ◽

Gardner's Syndrome ◽

Gardner’S Syndrome ◽

Treatment Methods ◽

Disease Epidemiology ◽

Genetically Determined

Gardner’s syndrome is a genetically determined disease characterized by a combination of colon polyposis, osteomas, and various soft tissue tumors. In this article, we discuss disease epidemiology, etiology, manifestations, and treatment methods. We also report a case of Gardner’s syndrome.

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