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一例伴有烟雾病现象的心脏黏液瘤罕见病例:一种疾病还是综合征?

A Rare Case of Cardiac Myxoma With Moyamoya Phenomenon: A Disease or Syndrome?

作者信息

Konduru Rayees A, Prasad Ankita, Cheriyath Pramil, Okere Arthur

机构信息

Anesthesiology, NewYork-Presbyterian Brooklyn Methodist Hospital, New York, USA.

Pediatrics, NewYork-Presbyterian Brooklyn Methodist Hospital, New York, USA.

出版信息

Cureus. 2024 Apr 30;16(4):e59381. doi: 10.7759/cureus.59381. eCollection 2024 Apr.

Abstract

Moyamoya disease (MMD) is a rare, idiopathic, progressive, obstructive, vasculopathy affecting primarily the terminal portions of the intracerebral internal carotid arteries, typically at the base of the brain. It is more commonly seen in people of East Asian descent. The moyamoya phenomenon refers to the characteristic appearance of the tangle of fine blood vessels, also described as a puff of smoke. Moyamoya syndrome (MMS) refers to the constriction-induced chronic brain ischemia that is believed to cause overexpression of proangiogenic factors, creating a fragile network of collateral capillaries. MMS refers to the moyamoya phenomenon in the presence of other congenital or acquired disorders. Intracerebral hemorrhage is the leading cause of death for MMS patients. Overall, the prognosis is variable. Cardiac myxoma can cause embolization of tumor cells, plaques, and thrombus, and recurrent thromboembolism can lead to chronic brain ischemia, which can lead to the development of collaterals. There have been cases reported where the moyamoya phenomenon resolved following myxoma resection. Here, we present the case of a female who had intraventricular bleeding and was diagnosed with MMD. Eighteen months later, she presented with shortness of breath and was diagnosed with cardiac myxoma with multiple valvular regurgitations. The myxoma was surgically removed.

摘要

烟雾病(MMD)是一种罕见的、特发性的、进行性的、阻塞性的血管病,主要影响大脑中颈内动脉的终末段,通常位于脑底部。它在东亚血统的人群中更为常见。烟雾现象是指细小血管缠结的特征性表现,也被描述为一团烟雾。烟雾综合征(MMS)是指由狭窄引起的慢性脑缺血,据信会导致促血管生成因子的过度表达,从而形成一个脆弱的侧支毛细血管网络。MMS是指在存在其他先天性或后天性疾病的情况下出现的烟雾现象。脑出血是MMS患者的主要死因。总体而言,预后因人而异。心脏黏液瘤可导致肿瘤细胞、斑块和血栓的栓塞,反复的血栓栓塞可导致慢性脑缺血,进而导致侧支循环的形成。有报道称,黏液瘤切除后烟雾现象消失。在此,我们报告一例女性患者,她曾发生脑室内出血,被诊断为烟雾病。18个月后,她出现呼吸急促,被诊断为患有心脏黏液瘤并伴有多处瓣膜反流。黏液瘤通过手术切除。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a56/11139051/19072a15995a/cureus-0016-00000059381-i01.jpg

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