Fibromuscular Dysplasia (FMD) Involving the Mesenteric Vasculature: An Unusual Cause of Mesenteric Ischemia

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Purpose: FMD is a non-inflammatory, non-atherosclerotic angiopathy, which usually involves the renal and cerebral vasculature. Here, we present a case of FMD affecting the mesenteric vasculature, without concomitant renal or cerebral vessel involvement, which presented clinically as chronic mesenteric ischemia. Case Presentation: A 47 year-old Caucasian, non-smoking woman presented with chronic nausea and abdominal pain. She had no significant past medical or surgical history. Her medications included Nexium® and oral contraceptive pills. Family history was notable only for hypercholesterolemia. Abdominal examination was notable only for a sharp crescendo, decrescendo bruit in the upper mid abdomen. For evaluation, an abdominal vascular ultrasound revealed markedly elevated pressures in the superior mesenteric artery suspicious for the presence of a stenosis. Magnetic resonance imaging confirmed these findings. A mesenteric arteriogram showed near complete occlusion of the celiac artery and superior mesenteric artery (Figure 1). She was treated originally with angioplasty and had complete resolution of her symptoms. However, she had symptom recurrence requiring repeat angioplasty with stent placement to the superior mesenteric artery twenty months later. Finally, a surgical bypass graft was performed 3 days after the repeat stenting secondary to an acute in-stent thrombosis while on anti-platelet therapy. Ultimately, the patient had a positive outcome with surgical bypass, including eight month follow-up. Discussion: Due its low incidence and relatively little knowledge concerning its risk factors and etiology, actual diagnosis of FMD involving the mesenteric vessels requires a very high degree of suspicion. Upon review of the few case reports of FMD causing mesenteric ischemia, it is clear that therapeutic interventions are rarely discussed and that positive outcomes are even more uncommon. Our case demonstrates that FMD should be considered early on in young female patients who present with chronic abdominal pain and weight loss.Figure: T1 post-contrast sagittal view which demonstrates smooth segment narrowing of the proximal SMA and celiac artery.