Case ReportSegmental arterial mediolysis of varying phases affecting both the intra-abdominal and intracranial vertebral arteries: an autopsy case report
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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Cited by (25)
A Ruptured Blood Blister–Like Aneurysm Associated With Intraperitoneal Hemorrhage Due to Segmental Arterial Mediolysis: A Case Report And Literature Review
2020, World NeurosurgeryCitation Excerpt :Many of the articles on SAM associated with SAH are from Japan: Of these 13 cases, 10 cases were Japanese (Table 1). Ro et al.21 reported an autopsy case of SAM being presented with a ruptured gastroepiploic artery aneurysm, which had various phases of SAM-related arteriopathy occurring in both intracranial vertebral and intra-abdominal arteries. The various phases of histopathologic characteristics including medial hematoma, granulation, and fibrous tissues suggested that SAM might have a stable and subclinical presentation.6
Subarachnoid Hemorrhage From a Distal Middle Cerebral Artery Aneurysm Possibly Related to Segmental Arterial Mediolysis
2019, World NeurosurgeryCitation Excerpt :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.
Segmental arterial mediolysis: A systematic review of 85 cases
2014, Annals of Vascular SurgerySegmental arterial mediolysis: Report of 2 cases and review of the literature
2011, American Journal of Kidney DiseasesCitation Excerpt :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
Clinical/scientific notes
2011, NeurologyCitation Excerpt :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