Lee Byung Do, Lee Wan, Oh Seung Hwan, Min Seung Ki, Kim Eun Cheol
Department of Oral and Maxillofacial Radiology, School of Dentistry, Wonkwang University, Iksan City, Korea.
Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009 Mar;107(3):e68-72. doi: 10.1016/j.tripleo.2008.10.018. Epub 2009 Jan 20.
Gardner syndrome (GS) is an abnormality of familial adenomatous polyposis accompanied by characteristic jaw lesions. Gardner syndrome intestinal polyps have a 100% risk of undergoing malignant transformation; consequently, early identification and surgical intervention of the disease are important to prolong the life of the patient. We present a case of GS in a 55-year-old woman. Familial adenomatous polyposis, osteomatous jaw and ocular lesions, several dental abnormalities, and an abdominal desmoid tumor are the characteristic features of this case. This case demonstrates the presence of unusual, widespread, hereditary, osteomatous jaw lesions that caused diagnostic confusion with familial gigantiform cementoma.
加德纳综合征(GS)是一种伴有特征性颌骨病变的家族性腺瘤性息肉病异常。加德纳综合征肠道息肉发生恶变的风险为100%;因此,该疾病的早期识别和手术干预对于延长患者生命很重要。我们报告一例55岁女性的加德纳综合征病例。家族性腺瘤性息肉病、颌骨骨瘤和眼部病变、多种牙齿异常以及腹部硬纤维瘤是该病例的特征性表现。该病例显示存在不寻常的、广泛的、遗传性颌骨骨瘤病变,这导致与家族性巨大牙骨质瘤的诊断混淆。