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      Fatal hemoperitoneum due to segmental arterial mediolysis

      case-report

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          Abstract

          Spontaneous hemoperitoneum due to vascular injury is a life-threatening condition mostly associated with aortic or splanchnic arterial disease, which stems from atherosclerotic, inflammatory, or infectious origin. However, in 1976, Slavin and Gonzales described a nonatherosclerotic arterial disease that may render aneurysmal formation predominantly in the splanchnic arterial bed. The clinical presentation is diverse, but abdominal pain and shock prevail. We report the case of a middle-aged man who presented a hemoperitoneum due to a middle colic artery aneurysm rupture and died after undergoing a surgical treatment attempt. The preoperative imaging study revealed the presence of a huge hematoma in the epiplon retrocavity, and abdominal free liquid as well as extensive arterial disease with multiple aneurysms. The autopsy findings included hemoperitoneum, hematoma in the upper left abdominal quadrant, the surgical ligature of the middle colic artery, and histologic features consistent with segmental arterial mediolysis. The authors call attention to this rare entity and highlight the autopsy as a fundamental examination to accurately reach this diagnosis.

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          Most cited references20

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          Segmental mediolytic arteritis: a clinical pathologic study.

          A distinct arterial lesion was observed in the large abdominal muscular arteries in three autopsied patients. The salient histopathologic feature of this arterial lesion was either partial or total mediolysis. This was accompanied by a linear fibrin deposit between the media and adventitia and a variable nonpleomorphic inflammatory infiltrate. Total mediolysis led to the formation of arterial gaps. Disecting aneurysms frequently occurred and began either adjacent to arterial gaps or as a result of capillary hemorrhages in areas of partial mediolysis. Ruptured aneurysms led to massive intraabdominal hemorrhages. Arterial luminal occlusion, either by thrombi or dissection, resulted in ischemic bowel changes and renal infarcts. In addition, the arteries affected by mediolysis also showed medial degenerative changes, akin to cystic medial necrosis. Concomitant changes in the kidney showed mesangial hyperplasia; the heart exhibited histiocytic infiltrates and rare Aschoff-like bodies and capsular inflammation were seen in the spleen. The pathogenesis of the arterial lesions is unknown. A possible explanation is that this arteritis may have been induced by immune complexes and that local arterial medial degenerative changes predispose the involved arteries to immunologic injury.
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            Segmental arterial mediolysis: course, sequelae, prognosis, and pathologic-radiologic correlation.

            Segmental arterial mediolysis is a vascular disease of putative vasospastic origin that causes massive hemorrhages. Although once considered rare, awareness of this disease has resulted in increased reports in the pathology and radiology literature. Despite this, uncertainties concerning pathologic and radiologic correlations, the course of this disease, and aspects of its prognosis exist. This article addresses these issues. Thirteen radiologic reports of segmental arterial mediolysis are analyzed, and slides of 25 cases of segmental arterial mediolysis are searched for lesions analogous to the radiologic findings. Six angiographic presentations are identified: (a) arterial dilatation, (b) single aneurysm, (c) multiple aneurysms, (d) dissecting hematomas, (e) arterial stenosis, and (f) arterial occlusions. Pathologic correlations reveal that lytic loss of medial muscle causes arterial dilatation, dilated arterial gaps form aneurysms, dissections develop at arterial-medial gap junctions or from reparative granulation tissue and reparative alterations, and thrombi cause stenosis and occlusions. The most common radiologic findings at onset are aneurysms, arterial dilatation, and occlusions, while dissections and stenotic lesions often are delayed. These images correlate with the histologic evolution of segmental arterial mediolysis. Segmental arterial mediolysis is an acute limited disease. Sequelae recognized radiologically include aneurysms, dissecting hematomas, arterial stenosis, and occlusions. Generally, these persist, become smaller, or resolve, but symptomatic dissections with delayed onset occur. Sequelae of subclinical forms of segmental arterial mediolysis may cause isolated idiopathic aneurysms or may evolve into arterial lesions indistinguishable from fibromuscular dysplasia.
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              Segmental arterial mediolysis: a systematic review of 85 cases.

              Segmental arterial mediolysis (SAM) is a nonatherosclerotic, noninflammatory arteriopathy of unknown etiology with life-threatening manifestations. With advances in endovascular techniques, SAM is increasingly being managed without the need for major surgery.
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                Author and article information

                Journal
                Autops Case Rep
                Autops Case Rep
                Autopsy & Case Reports
                São Paulo, SP: Universidade de São Paulo, Hospital Universitário
                2236-1960
                30 September 2016
                Jul-Sep 2016
                : 6
                : 3
                : 7-15
                Affiliations
                [a ]Anatomic Pathology Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP – Brazil.
                [b ]Department of Pathology - Medical School - Universidade de São Paulo, São Paulo/SP – Brazil.
                [c ]Internal Medicine Department - Hospital Universitário - Universidade de São Paulo, São Paulo/SP – Brazil.
                [d ]Imaging Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP – Brazil.
                Author notes

                Conflict of interest: None

                Correspondence
 Aloísio Felipe-Silva
 Anatomic Pathology Service - Hospital Universitário - Universidade de São Paulo (USP)
 Avenida Professor Lineu Prestes, 2565
 CEP: 05508-000
 Phone: +55 (11) 3091-9384
 aloisiosilva@ 123456hu.usp.br
                Article
                autopsy006016
                10.4322/acr.2016.049
                5087978
                27818953
                48acfd48-5a0b-4b22-b317-7aa0d3ce9002
                Autopsy and Case Reports. ISSN 2236-1960. Copyright © 2016.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the article is properly cited.

                History
                : 02 September 2016
                : 19 September 2016
                Categories
                Article / Autopsy Case Report

                splanchnic circulation,aneurysm,abdominal pain,fibromuscular dysplasia,autopsy

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