-Case Reports-

Acute Aortic Dissection Associated with Cystic Medial Necrosis of Unknown Etiology

Koichi Akutsu¹, Masashi Kawamoto², Naoki Sato^1,3, Takeshi Yamamoto¹, Koichi Tamura⁴, Kyoichi Mizuno⁵ and Keiji Tanaka¹

¹Division of Intensive and Cardiac Care Unit, Nippon Medical School Hospital
²Diagnostic Pathology, Nippon Medical School Hospital
³Department of Internal Medicine, Nippon Medical School Musashi Kosugi Hospital
⁴Division of Surgical Pathology, Tokyo Teishin Hospital
⁵Division of Cardiology, Hepatology, Geriatrics, and Integrated Medicine, Department of Internal Medicine, Graduate School of Medicine, Nippon Medical School

A 61-year-old man without a Marfan-like phenotype was admitted to the hospital because of acute Stanford type A aortic dissection. The patient underwent surgical repair with total arch replacement. Histological examination of the excised aorta showed a connective tissue abnormality, which could have contributed to the development of aortic dissection. The cause of the connective tissue abnormality could not be determined through physical examination. Recently, however, many novel gene mutations have been found to be related to aortic diseases that do not always produce physical signs and symptoms. In this case, unknown causes of connective tissue abnormalities might be existed.

J Nippon Med Sch 2012; 79: 159-162

Keywords
aortic dissection, connective tissue abnormality, gene mutations

Correspondence to
Koichi Akutsu, MD, Division of Intensive and Cardiac Care Unit, Nippon Medical School Hospital, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan
koichi-a@nms.ac.jp

Received, September 14, 2011
Accepted, October 11, 2011