Non-atherosclerotic Abdominal Vasculopathies

Abstract Background Non-atherosclerotic abdominal arterial vasculopathies (NAV), including mesenteric and or renal artery dissection, aneurysm, stenosis, or vasculitis are rare but have great clinical significance. Patients may present emergently with life threatening complications including arterial rupture and hemorrhagic shock. Herein, we present our center’s experience with NAV and provide extensive literature review to close the gap in the scarce related literature. Methods From a single center retrospective data analysis, we identified and characterized subjects (age: 18-60) that presented with NAV between January 2000 and December 2015. Of the 1416 charts reviewed, 118 met inclusion criteria. Results Average age was 47.0 ± 9.9 years, mostly affecting women (64%). Primary diagnoses included: fibromuscular dysplasia (FMD) (25.4%), isolated aneurysms (24.6%) and median arcuate ligament syndrome (MALS) (15.3%). Less common diagnoses were localized vasculitis of the gastrointestinal tract (LVGT) (7.6%), isolated dissection (5.1%), microscopic polyangiitis and granulomatosis with polyangiitis (5.1%), trauma (4.2%), segmental arterial mediolysis (4.2%), Ehlers-Danlos syndrome (2.5%), Takayasu’s arteritis (2.5%), polyarteritis nodosa (1.7%), idiopathic abdominal aortitis (.8%), and Loeys Dietz syndrome (.8%). Females constituted 90% of patients with FMD, 77.8% with MALS, 77.8% with isolated aneurysms, 66.7% with Takayasu arteritis and 55.6% with LVGT. Prevalent comorbidities included tobacco use (43.6%) and hypertension (52.1%). Coil embolization was used in 14.4%, anticoagulation in 11.9%, angioplasty/stenting in 11.9%, open resection/surgical revascularization in 10.2%, and prednisone in 10.2% of the cases. Conservative management was pursued in 33.1% of patients. A high degree of symptom relief was shown in 91.7%. Conclusions NAV are rare and can be caused by different etiologies that primarily affect females. Hypertension and tobacco use were prevalent. Various imaging strategies revealed aneurysms, stenosis, dissection and or thrombosis affecting renal and celiac arteries. Most patients improved with conservative, medical, endovascular and or surgical approach. More research is needed to standardize management approach to patients with NAV.