Narisawa T, Yamada M, Funami M, Tanaka H, Inoue K, Takaba T
Department of Surgery, School of Medicine, Showa University, Tokyo, Japan.
Nihon Kyobu Geka Gakkai Zasshi. 1994 Nov;42(11):2091-4.
The occurrence of familial dissecting aortic aneurysms without Marfan syndrome is rare. This report describes two aged siblings who underwent successful surgery for this disease. Neither patient had the clinical features of Marfan syndrome, and both had histories of systemic hypertension. Case 1: A 76-year-old man had dissecting aortic aneurysm, DeBakey type IIIa, which was localized and had multiple lumina with thrombi. Case 2: A 71-year-old man (younger brother of case 1) had retrograde dissecting aortic aneurysm, DeBakey type IIIa. Histological examinations of the aortae showed cystic medial necrosis in both cases, and also showed atherosclerotic changes in case 1. These two siblings had no familial history of cardiovascular incidents and were much older than previously reported cases. Therefore it may be possible that the dissecting aortic aneurysms of these two cases occurred coincidently rather than due to hereditary factors.
