Unusual petal-like fibromuscular dysplasia as a cause of acute abdomen and circulatory shock

Abstract

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory segmental arterial occlusive disorder that involves primarily the renal and carotid arteries, and less often the coronary, iliac, and visceral arteries. We report the case of 78-year-old Japanese woman who presented with acute abdomen complicated by shock. Autopsy revealed hemorrhagic necrosis of the small intestine due to severe narrowing of the mesenteric arteries. Histologically, smooth muscles showed in-bundle hyperplasia surrounding the adventitia together with medial and perimedial fibrodysplasia of these arteries, forming the characteristic petal-like appearance of FMD. No occlusive thrombus was observed. Further, another medial fibrodysplasia type of FMD was also seen in the renal and left circumflex coronary arteries. Unusual proliferation of smooth muscles resulted in the petal-like atypical FMD at the superior mesenteric artery.