Elsevier

Cardiovascular Pathology

Volume 19, Issue 4, July–August 2010, Pages 248-251
Cardiovascular Pathology

Case Report
Segmental arterial mediolysis of varying phases affecting both the intra-abdominal and intracranial vertebral arteries: an autopsy case report

https://doi.org/10.1016/j.carpath.2009.02.002Get rights and content

Abstract

We report an autopsy case of segmental arterial mediolysis (SAM) of various phases occurring in both the intracranial vertebral artery (IVA) and intra-abdominal arteries. The patient was a 70-year-old male found dead in his house. The cause of death was massive intra-abdominal hemorrhage owing to a ruptured right gastroepiploic artery. Histopathological examination revealed that there was a broad arterial dissection as long as 20 cm in the right gastroepiploic artery associated with SAM in the injurious phase. In addition, SAM in the reparative phase was observed as organized arterial dissections in the left gastric artery. Furthermore, SAM in the reparative phase was detected as an arterial dissection in the right IVA undergoing an organizing process. These three lesions were considered to have developed at different times. SAM occurring in both the intra-abdominal and intracranial vertebral arteries is extremely rare. This coincidence may provide a clue to the relationship between SAM and spontaneous IVA dissection.

Introduction

Segmental arterial mediolysis (SAM) is a rare arterial degenerative disease first described by Slavin and Gonzalez-Vitale [1] in 1976. It usually occurs in the small intra-abdominal artery resulting in intra-abdominal hemorrhage owing to the rupture of an arterial dissection [2], [3], [4], [5]. SAM is often clinically identifiable in the acute injurious phase, while the reparative phase of SAM is rarely detected [4], [5]. Furthermore, SAM combined with dissection of the intracranial vertebral artery (IVA) is extremely rare [3].

To our knowledge, an autopsy case of SAM affecting both intra-abdominal arteries in the injurious phase and the IVA in the reparative phase has not been previously described.

Section snippets

Case report

A 70-year-old male with a history of schizophrenia, hypertension, and cardiac angina was found dead in his house. He suffered from chronic bronchitis presenting with a 6-month history of cough. He complained of poor physical condition the day before his death. He had neither family history of SAM nor past histories of other connective tissue disease.

Medicolegal autopsy was performed 30 h postmortem. On gross examination, the cadaver was 162 cm tall and weighed 41 kg. Hematoma up to 1000 ml was

Discussion

SAM is an arterial noninflammatory degenerative disease proposed by Slavin and Gonzalez-Vitale [1]. It usually occurs in intra-abdominal muscular arteries. Multiple mediolysis easily induces arterial dissections resulting in rupture and subsequent massive intra-abdominal hemorrhage.

SAM is such a rare arterial disease that the largest SAM review of literature handled only 52 patients [5]. According to the review, SAM patients were usually in middle age over 40 years old and have a gender

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    The reparative phase follows the injurious phase. The reparative phase shows proliferated granulation tissue in the arterial wall and fibrous deposition in the injured site.9 The histopathologic findings in our case corresponded with the reparative phase of SAM with a partially defective media, and the thickness of the intima with fibrous tissue observed.

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    Coronary artery involvement also has been described, but mainly in neonates.27 Combined intra-abdominal and cerebral involvement, as in our case, is unusual, but has been reported.25,28,29 The pathogenesis of SAM is uncertain, but is thought to be a response to repeated episodes of vasospasm.3-5

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    The cause of sCAD is unknown, with congenital or acquired arteriopathies such as fibromuscular dysplasia (FMD), Ehlers-Danlos syndrome type IV, and Marfan syndrome only being associated with a minority of cases.1 Here we report a case of fatal bilateral spontaneous internal carotid artery dissections secondary to SMA which is a rare arteriopathy usually involving small to medium-sized mesenteric vessels but has been reported to involve cervical and cerebral arteries.2 Segmental mediolytic arteriopathy was proposed in 1976 and was thought to be a possible predisposing factor to sCAD.3

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