Noonan's syndrome associated with spontaneous rupture of the ascending aorta is rare. Review of the literature reveals only one such reported case. The authors describe a 41-year-old male with Noonan's syndrome who initially presented with lower extremity claudication. Computed tomography and digital subtraction angiography disclosed an abnormal dilation of the ascending thoracic aorta and a distal abdominal aortic dissection. The patient underwent successful aortoiliac bypass graft. Seven months later, he suffered rupture of his ascending aorta, resulting in death.
Get full access to this article
View all access options for this article.
References
1.
1. Hara K, Sasaki T, Miyauchi H, et al: Noonan phenotype associated with intracerebral hemorrhage and cerebral vascular anomalies: Case report. Surg Neurol39:31-36, 1993.
2.
2. Shachter N. Perloff J, Mulder D: Aortic dissection in Noonan's syndrome (46xy turner). Am J Cardiol54:464-465, 1984.
3.
3. Rubin K: Aortic dissection and rupture in Turner's syndrome. J Pediatr122:670-670, 1993.
5. Burch M, Sharland M, Shineboume E, et al: Cardiologic abnormalities in Noonan's syndrome: Phenotypic diagnosis and echocardiographic assessment of 118 patients. J Am Coll Cardiol22:1189-1192, 1993.
6.
6. Slater D, Mitchell M, Grundman M: Turner's syndrome associated with bicuspid aortic stenosis and dissecting aortic aneurysm. Postgrad Med J58:436-438, 1982.
7.
7. Bartlema K, Hogervorst M, Akkerskijk G, et al: Isolated abdominal aortic dissection in a patient with Turner's syndrome. Surgery117:116-117, 1995.
8.
8. Lin A, Lippe B, Geffner M, et al. Aortic dilation, dissection, and rupture in patients with Turner's syndrome. J Pediatr109:820-826, 1986.