第7巻 2011年10月　第4号

■症例報告

待機術直前に無症候性大動脈解離をきたしたと思われるMarfan症候群の1例
阿部　正徳^1,2, 渡辺　誠^1,2, 山内　仁紫³, 落　雅美³, 小川　俊一^1,2
1日本医科大学大学院医学研究科小児医学
²日本医科大学付属病院小児科
³日本医科大学外科学（内分泌・心臓血管・呼吸器部門）

A Case of Marfan Syndrome with Asymptomatic Aortic Dissection Diagnosed Just before Elective Surgery
Masanori Abe^1,2, Makoto Watanabe^1,2, Hitoshi Yamauchi³, Masami Ochi³ and Shunichi Ogawa^1,2
1Department of Pediatrics, Graduate School of Medicine, Nippon Medical School
²Department of Pediatrics, Nippon Medical School Hospital
³Department of Surgery (Divisions of Endocrine, Cardiovascular and Thoracic Surgery), Nippon Medical School

A 19-year-old man was admitted to our hospital for elective surgery to replace the ascending aorta. The patient's father had died suddenly of aortic dissection associated with Marfan syndrome. Because of the patient's family history and physical findings, we suspected Marfan syndrome and began regular observations of aortic size when he was an infant. When he was 18 years old, magnetic resonance cardiography showed a marked increase in the size of the ascending aorta, replacement of which was indicated. Computed tomography performed before the planned elective surgery incidentally revealed dissection of the ascending aorta, although the patient was asymptomatic. Thus, we failed to prevent the aortic dissection in this patient, although we had regularly observed the aorta since he was an infant. Surgery should have been performed earlier than it was. Our experience suggests more frequent observation and stricter indications for aortic surgery should be considered in Marfan syndrome.

日医大医会誌 2011; 7(4), 179-182

Key words
Marfan syndrome, annulo-aortic ectasia, asymptomatic dissection of aorta, childhood

Correspondence to
Masanori Abe, Department of Pediatrics, Nippon Medical School Tama Nagayama Hospital, 1-7-1 Nagayama, Tama Tokyo 206-8512, Japan
E-mail：masha@nms.ac.jp

受付：2011年4月27日　受理：2011年7月4日