Segmental Arterial Mediolysis: Report of 2 Cases and Review of the Literature

Filippone, Edward J.; Foy, Andrew B.; Galanis, Taki; Pokuah, Marian; Newman, Eric D.; Lallas, Costas D.; Gonsalves, Carin F.; Farber, John L.

doi:10.1053/j.ajkd.2011.05.031

Cited by 30 publications

(32 citation statements)

References 36 publications

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“…A definitive diagnosis of segmental arterial mediolysis requires tissue examination. 134,135 This lesion of segmental arterial mediolysis is characterized by the vacuolar degeneration of smooth muscle cells in the outer media that may extend to the inner aspect with increased deposition of ground substance. Smooth muscle cells are progressively lost with the development of arterial gaps, intramural hemorrhage, and fibrin deposition along the media-adventitia interface.…”

Section: Segmental Arterial Mediolysismentioning

confidence: 99%

Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions

Olin¹,

Gornik²,

Bacharach³

et al. 2014

Circulation

388

490

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Section: Segmental Arterial Mediolysismentioning

confidence: 99%

Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions

Olin¹,

Gornik²,

Bacharach³

et al. 2014

Circulation

388

490

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“…3,20,[35][36][37] Atherosclerosis, embolic disease from atrial myxomas or cholesterol deposits, segmental arteriolysis medial, calciphylaxis lesions, thrombotic disorders (antiphospholipid syndrome), fibromuscular dysplasia, and ergot use can mimic vasculitis of medium-sized arteries. 41,42 If disease affects the central nervous system, Moyamoya disease, MELAS (mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes), CADASIL (cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy), and granulomatous angiitis of the central nervous system are considerations. In children, Kawasaki disease affects medium-sized blood vessels, in particular the coronary arteries.…”

Section: Differential Diagnosismentioning

confidence: 99%

Polyarteritis Nodosa

Forbess¹,

Bannykh²

2015

Rheumatic Disease Clinics of North America

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Polyarteritis nodosa (PAN) is a systemic disease, but variants are cutaneous PAN and single-organ disease. Histologic confirmation of vasculitis in medium-sized arteries is desirable, and biopsies should be obtained from the symptomatic and least invasive sites. Angiography can show multiple microaneurysms in the viscera. Treatment includes high-dose corticosteroids, which are combined with immunosuppressive agents when internal organs are involved and with life-threatening disease. Once remission is achieved, maintenance agents are initiated. PAN is becoming a rare disease. International collaborative efforts are under way to establish better diagnostic and classification for all vasculitides, including PAN.

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“…The most common sites are celiac axis, splenic, superior mesenteric, renal, and inferior mesenteric arteries. The involvement of cerebral vasculature is less frequent, affecting a younger population [9]. Usually, the immediate small branches of the affected arteries also show features of SAM [4].…”

Section: Discussionmentioning

confidence: 99%

“…Vasospasm is thought to be the underlying aetiological agent based on morphologic and ultra-structural features [9,10,12]. Factors including hypoxia, exogenous vasopressors, pulmonary hypertension, central nervous system lesions can initiate the dysfunction of endothelial paracrine system causing intense vasoconstriction.…”

Section: Discussionmentioning

confidence: 99%

“…Progression results in transmedial mediolysis with loss of intima creating arterial gaps bridged by fibrin. These can enlarge by detachment of residual connections to the wall and develop vascular aneurysms which is the most frequent angiographic feature of SAM [4,9,10]. The smaller saccular aneurysms might decrease in size or resolve, while the larger ones may thrombose or bleed requiring radiologic or surgical intervention.…”

Section: Discussionmentioning

confidence: 99%

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Segmental arterial mediolysis of left gastric artery: a case report and review of pathology

et al. 2013

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BackgroundSegmental arterial mediolysis (SAM) is a rare non inflammatory vascular disease that can present with massive haemorrhage, which may lead to death without prompt surgical intervention.Case presentationA 60 years old Aboriginal female presented with life threatening, spontaneous intra-abdominal bleeding requiring an emergency laparotomy. The source of bleeding was found to be ruptured left gastric artery. A total gastrectomy was performed as a damage control procedure. A staged Roux-en-Y oesophago-jejunostomy with Hunt Lawrence pouch reconstruction was undertaken thirty six hours later. Histopathological findings revealed evidence of non-inflammatory segmental vascular damage with microaneurysm, consistent with segmental arterial mediolysis.ConclusionPrompt resuscitation and surgical intervention can decrease the morbidity and mortality in this rare clinical entity.

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Segmental Arterial Mediolysis: Report of 2 Cases and Review of the Literature

Cited by 30 publications

References 36 publications

Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions

Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions

Polyarteritis Nodosa

Segmental arterial mediolysis of left gastric artery: a case report and review of pathology

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