Fibromuscular dysplasia is a rare nonatherosclerotic, noninflammatory angiopathy of uncertain etiology and high morbidity. Because of its propensity to affect medium-sized vessels in a variety of locations, presenting symptoms may vary substantially, resulting in a delayed or missed diagnosis. We describe a 57-year-old woman who, on multiple occasions, presented with progressive gastrointestinal symptoms and eventually underwent surgical revascularization for celiac and superior mesenteric artery stenosis of uncertain etiology. Her postoperative course was complicated by bowel ischemia, multiple organ failure, and death. Autopsy findings proved useful in determining the underlying disease process and cause of death. This case report and a review of the literature illustrate the high morbidity and mortality that are caused by mesenteric fibromuscular dysplasia, the challenge in establishing a correct diagnosis, and the importance of early detection and treatment.
Guill CK, Benavides DC, Rees C, Fenves AZ, Burton EC. Fatal Mesenteric Fibromuscular DysplasiaA Case Report and Review of the Literature. Arch Intern Med. 2004;164(10):1148-1153. doi:10.1001/archinte.164.10.1148