Abstract
Two brothers with multiple visceral artery aneurysms or dilatations and diffuse connective tissue fragility who did not have clinical features of Marfan syndrome are reported. One presented with retroperitoneal hemorrhage during angiography, and idiopathic medionecrosis was proved by resection of the aneurysms. These cases belong to the heterogeneous group of Marfan syndrome. The angiographical features (multiple dilation of visceral arteries) suggests fragility of connective tissue and is predictive of hazards during and after a catheterization and operation.
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References
Robbins SL (1974) Pathologic Basis of Disease. Saunders, Philadelphia, pp 612–614
Crivello MS, Porter DH, Kim D, Critchlow JF, Scoutt L (1986) Isolated external iliac artery aneurysm secondary to cystic medial necrosis. Cardiovasc Intervent Radiol 9:139–141
Francke U, Furthmayr H (1994) Marfan’s syndrome and other disorders of fibrillin. N Engl J Med 330:1384–1385
Stanley JC, Wakefield TW, Graham LM, Whitehouse WM Jr, Zelenock GB, Lindenauer SM (1986) Clinical importance and management of splanchnic artery aneurysms. J Vasc Surg 3:836–840
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Kubota, J., Tsunemura, M., Amano, S. et al. Non-marfan idiopathic medionecrosis (cystic medial necrosis) presenting with multiple visceral artery aneurysms and diffuse connective tissue fragility: Two brothers. Cardiovasc Intervent Radiol 20, 225–227 (1997). https://doi.org/10.1007/s002709900143
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DOI: https://doi.org/10.1007/s002709900143