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加德纳综合征,伴有多发性骨瘤、肠息肉病和表皮样囊肿。

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

作者信息

Koh Kwang-Joon, Park Ha-Na, Kim Kyoung-A

机构信息

Department of Oral and Maxillofacial Radiology, School of Dentistry and Institute of Oral Bioscience, Chonbuk National University, Jeonju-si, Republic of Korea.

出版信息

Imaging Sci Dent. 2016 Dec;46(4):267-272. doi: 10.5624/isd.2016.46.4.267. Epub 2016 Dec 20.

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.

摘要

加德纳综合征被认为是家族性腺瘤性息肉病的一种变体。该综合征的特征为多发性肠道息肉、骨瘤和表皮样囊肿。此外,牙齿异常包括多发性牙瘤的发生率增加,以及多生牙和阻生牙。作者报告了一例7岁男性加德纳综合征患者的病例。影像学检查结果显示上颌骨两侧有多个骨瘤,上下颌均有多个弥漫性骨岛,左下颌体有一个复合性牙瘤。两年后,在头皮上发现了多个表皮样囊肿。由于怀疑该患者患有加德纳综合征,作者建议进行胃肠内镜检查以检查是否存在肠道息肉。胃肠内镜检查发现上消化道和胃底有多个息肉。最终诊断为加德纳综合征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/10b1/5192025/0c9db178f2b7/isd-46-267-g001.jpg

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