Dysplasia of the aorta An autopsy case of dysplasia of the abdominal aorta accompanied with thrombosis in the course of nephrotic syndrome

Abstract

Fibromuscular dysplasia is one of the diseases in idiopathic nonarteriosclerotic syndrome of the artery. The disease causes luminal stenosis of the artery by proliferation and/or separation of the smooth muscle cells, elastic fibers and another fibrous elements in the media. Only one case of the arterial dysplasia of the aorta has been reported by Hill et al (1965). A 31-year-old male suffered from nephrotic syndrome and schizophrenia for about 9 years. During the course of the illness he was attacked by sudden paresthesia and felt coldness and pain in the bilateral lower extremities. He was then treated with urokinase and heparin under suspicion of thrombosis in the abdominal aorta and the iliac arteries. Two days later he died of respiratory failure. Autopsy revealed thrombi obstructing almost of the luminal spaces of the lower abdominal aorta and the bilateral iliac arteries. The media of the thrombosed arteries showed dysplastic changes such as an alveolar arrangement of the smooth muscle cells and hypoplasia of the elastic fibers. Mild thickening of the intima also occurred in the abdominal arota. The authors discussed the nomenclature of the aortic dysplasia, pathogenesis and its correlation of medionecrosis aortae idipathica cystica.