Gardner Syndrome With Unusual Maxillofacial Manifestation

Abstract

Gardner syndrome is a rare autosomal-dominant condition characterized by the presence of intestinal polyposis, multiple osteomas, and tumors of the hard and soft tissues. This paper describes a patient of Gardner syndrome with unusual maxillofacial manifestation with presence of fibromyxomatous injury in jaw, coronoid hyperplasia, and multiple osteomas diffusely distributed in the craniomaxillofacial skeleton. Imaging examinations have identified craniofacial manifestations and the patient was referred to the gastroenterologist who confirmed the diagnosis of Gardner syndrome. The early diagnosis of this syndrome is important since intestinal polyps have high potential for malignant transformation. It is therefore essential that dentists are familiar with the maxillofacial features of this condition, since they precede the intestinal polyposis and encourage early diagnosis. In addition to classic maxillofacial signs of this syndrome, one must consider that, although it is rare, other injuries may be present such as those described in this clinical patient.

Benign connective and soft‐tissue neoplasms of the oral and maxillofacial region: Cross‐sectional study of 1066 histopathological specimens

“…7,31,53,54 Multiple osteomas can be seen in Gardner syndrome, which is a genetic disorder also characterized by intestinal polyps, multiple supernumerary teeth, and dermoid tumors. 7,12,13 As observed in the single case in the present study, Gardner syndrome has an early onset and most patients have a family history. 12,13 It usually occurs equally in both sexes, with osteomas and dental abnormalities preceding other manifestations.…”

“…7,12,13 As observed in the single case in the present study, Gardner syndrome has an early onset and most patients have a family history. 12,13 It usually occurs equally in both sexes, with osteomas and dental abnormalities preceding other manifestations. Therefore, dentists play an important role in the early diagnosis of this syndrome.…”

“…Importantly, some oral and maxillofacial CSTNs can be the first recognized sign of syndromes of considerable systemic concern. [9][10][11][12][13][14][15][16][17] There is a lack of original studies encompassing benign oral and maxillofacial CSTNs as a group, which makes it The present study reports the demographic and clinical features of benign CSTNs of oral and maxillofacial region, comprising a large sample. To the best of our knowledge, no previous study has concentrated such a broad variety of benign oral and maxillofacial CSTNs.…”

“…It usually occurs equally in both sexes, with osteomas and dental abnormalities preceding other manifestations. Therefore, dentists play an important role in the early diagnosis of this syndrome 12‐17 …”

“…The literature on benign CSTNs of the oral and maxillofacial region is somewhat scarce and most original articles are focused on a single tumor or a specific group of tumors 6‐55 . Therefore, the aim of the present study was to describe the demographic and clinical features of benign CSTNs occurring in the oral and maxillofacial region in order to (1) characterize benign oral and maxillofacial CSTNs as a group and (2) identify similarities and differences that could aid in the clinical differential diagnosis.…”