A Case of Marfan's Syndrome Associated with Ruptured Abdominal Aortic Aneurysm Following Bentall's Operation / 日本心臓血管外科学会雑誌

ABSTRACT

A 27-year-old man had received Bentall's operation for annuloaortic ectasia with Marfan's syndrome 4 years previously. He was admitted to our hospital because of sudden abdominal pain and lumbago. The abdominal pulsatile mass with tenderness was palpated and dilatation of abdominal aorta was revealed by abdominal ultrasonography. An emergency operation was performed under a diagnosis of ruptured abdominal aortic aneurysm. At operation, the infrarenal abdominal aorta formed a fusiform aneurysm of which maximum diameter was 6cm. The aneurysm had a thin wall, and ruptured opening about 2cm in diameter at the posterior wall, but no thrombus inside. Graft replacement was done from the infrarenal abdominal aorta to the bilateral common iliac artery using knitted Dacron vascular prosthesis, and reconstruction of inferior mesenteric artery with wrapping of the proximal anastomosis were performed. Histopathological examination of the aneurysmal wall revealed medial necrosis and degeneration, by which Marfan's syndrome was diagnosed. Although abdominal aortic aneurysm is rarely associated with Marfan's syndrome, it often shows rapid development and has a high risk of rupture. Therefore, we suggest that strict observation and early operation are important for abdominal aortic aneurysm associated with Marfan's syndrome.