A case of segmental mediolytic arteriopathy involving both intracranial and intraabdominal arteries
A case of segmental mediolytic arteriopathy involving both intracranial and intraabdominal arteries
复制标题
DOI：
10.1078/0344-0338-00290
复制
发表时间：
2002-01-01
影响因子：
2.8
通讯作者：
Takatama, M
中科院分区：
文献类型：
--
作者：
Sakata, N;Takebayashi, S;Takatama, M
节段性中膜溶解性动脉病（SMA）是一种不常见的非动脉粥样硬化性和非血管炎性动脉病。这种疾病的特点是溶解性变性的动脉介质，壁内夹层和血栓形成或破裂的动脉瘤。SMA主要累及腹腔内动脉系统，导致腹腔内和腹膜后出血。然而，只有少数病例SMA累及颅内动脉。在这里，我们提出了一个SMA发展蛛网膜下腔出血的情况下，由于夹层的颈内动脉和椎动脉。该患者为48岁男性，入院后13天死于突然意识丧失。计算机断层扫描显示蛛网膜下腔出血。尸检时，受累血管包括右侧椎动脉、左侧颈内动脉、上级肠系膜动脉、双侧肾动脉和左侧髂外动脉。组织学上，动脉表现为节段性溶解变性和中膜平滑肌细胞消失，中膜夹层和假性动脉瘤形成，其壁由外膜的薄膜组成。这些组织病理学特征模拟了颅内夹层动脉瘤的全壁夹层类型，其仅影响椎基底动脉系统。因此，SMA应被视为自发性颅内动脉夹层患者的可能基础疾病。
Segmental mediolytic arteriopathy (SMA) is an uncommon nonatherosclerotic and nonvasculitic arteriopathy. This disease is characterized by lytic degeneration of the arterial media, intramural dissection and thrombosed or ruptured aneurysm. SMA mainly involves the intraabdominal arterial system, resulting in intraabdominal and retroperitoneal hemorrhage. However, only a few cases of SMA with involvement of intracranial arteries have been reported. Here, we present a case of SMA developing subarachnoid hemorrhage due to dissection of the internal carotid and vertebral arteries. This patient was a 48-year-old male who died 13 days after admission for sudden loss of consciousness. Computed tomography showed subarachnoid hemorrhage. At autopsy, the affected vessels included the right vertebral, left internal carotid, superior mesenteric, bilateral renal and left external iliac arteries. Histopathologically, the arteries showed segmental lytic degeneration and disappearance of medial smooth muscle cells, medial dissection and formation of pseudo-aneurysms, the wall of which consisted of a thin membrane of the adventitia. These histopathological features mimicked an entire wall dissection type of intracranial dissecting aneurysm, which exclusively affects the vertebro-basilar system. Thus, SMA should be considered a possible underlying disease in patients with spontaneous dissection of intracranial arteries.