Natural history and management outcomes of segmental arterial mediolysis

Background: Segmental arterial mediolysis (SAM) is a poorly understood, nonatherosclerotic, noninflammatory disease resulting from arterial medial degeneration. Patients may present with aneurysm, dissection, stenosis, or bleeding from visceral or renal arteries. Treatment algorithms are poorly characterized. Methods: A retrospective review of all patients diagnosed with SAM was performed at our institution. Patients were identified by established criteria that include clinical presentation in combination with radiographic and serologic findings. Demographics, presenting symptoms, diagnostic evaluation, management, and outcomes were reviewed. Results: There were 117 patients diagnosed with SAM between 2000 and 2016; 67.5% (n = 79) were male. Mean age was 52.7 years (range, 23.4-90 years); 69.2% (n = 81) presented with acute abdominal pain, 22.2% (n = 26) with flank pain, and 19.7% (n = 23) with back pain; 15.4% (n = 18) had abdominal pain longer than 30 days; 13.7% (n = 16) had acute hypertension, and 5.1% (n = 6) were hypotensive; 10.3% (n = 12) were asymptomatic. There were 93 (79.5%) dissections and 61 (52.1%) aneurysms. Hemorrhage was seen in 10 (8.5%). The celiac axis was affected in 54.7% (n = 64), renal arteries in 49.6% (n = 5 8), superior mesenteric artery in 43.6% (n = 51), and inferior mesenteric artery in 2.6% (n = 3). After diagnosis of SAM, aspirin was prescribed in 60.7% (n = 71). Statins were prescribed in 29.9% (n = 35). Antihypertensive medications were prescribed in 65% (n = 76), including beta blockers in 42.7% (n = 50); 40.2% (n = 47) of patients were prescribed anticoagulation. Interventions were performed in 26 (22%) patients; 13 had endovascular intervention only, 9 open surgery only, and 4 open and endovascular interventions. Of the 17 patients undergoing endovascular intervention, 19 procedures were performed, most commonly embolization (78.9% [n = 15]), followed by stenting (10.5% [n = 2]). Of the 13 patients undergoing open surgery, 14 procedures were performed, including arterial bypass (50% [n = 7]) and splenectomy with aneurysm ligation (15.4% [n = 2]). Other surgery involved thrombectomy (21.4% [n = 3]) and angioplasty (14.3% [n = 2]). Only 11.5% (n = 3) experienced a perioperative complication, including one hematoma, one abscess, and one death secondary to ongoing hemorrhage. Follow-up imaging was performed in 96.6% (n = 112). Mean follow-up was 1258 days (range, 2-5017 days). Of these, 27.7% (n = 31) had regression, 43.8% (n = 49) stability, and 28.6% (n = 32) progression. Average time between initial diagnosis and progression was 666 days. Conclusions: SAM is an uncommon disease that may require intervention; it is therefore important that the vascular surgery community be aware of this disease. Follow-up imaging is required to monitor for disease progression.