Pure arterial malformation with associated aneurysmal subarachnoid hemorrhage: Two case reports and literature review

Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2021 Feb 28;46(2):200-206. doi: 10.11817/j.issn.1672-7347.2021.190673.
[Article in English, Chinese]

Abstract

In recent years, in the absence of venous component, dilated, overlapping, and tortuous arteries forming a mass of arterial loops with a coil-like appearance have been defined as pure arterial malformation (PAM). It is extremely rare, and its etiology and treatment have not yet been fully elucidated. Here, we reported 2 cases of PAM with associated aneurysmal subarachnoid hemorrhage in this paper. Both patients had severe headache as the first symptom. Subarachnoid hemorrhage was found by CT and computed tomography angiography (CTA) and PAM with associated aneurysm was found by digital subtraction angiography (DSA). In view of the distribution of blood and the location of aneurysms, the aneurysm rupture was the most likely to be considered. Based on the involvement of the lesion in the distal blood supply, only the aneurysm was clamped during the operation. It used to be consider that PAM is safety, because of the presentation and natural history of previously reported cases. Through the cases we reported, we have doubted about "the benign natural history" and discussed its treatment. PAM can promote the formation of aneurysms and should be reviewed regularly. The surgical indications for PAM patients with aneurysm formation need to be further clarified. Management of PAM patients with ruptured aneurysm is the same as that of ruptured aneurysm. Whether there are indications needed to treat simple arterial malformations remains to be further elucidated with the multicenter, randomized controlled studies on this disease.

近年来,在没有静脉参与的情况下,拉长、扩张、重叠和扭曲甚至呈螺旋环状的动脉被定义为纯动脉畸形(pure arterial malformation,PAM)。PAM十分罕见,其病因和治疗方法尚未完全阐明。本文报告2例PAM伴动脉瘤破裂蛛网膜下腔出血患者。患者均以剧烈头痛为首发症状,CT示蛛网膜下腔出血,CT血管造影(computed tomography angiography,CTA)示动脉瘤破裂伴蛛网膜下腔出血,进一步的数字减影血管造影(digital subtraction angiography,DSA)发现PAM合并动脉瘤。鉴于出血的分布和动脉瘤的位置,动脉瘤破裂出血的可能性最大。考虑到责任动脉参与远端血供,术中仅予以动脉瘤夹闭。以往一般认为对于PAM保守治疗是安全的。此2例患者的情况与所谓的“良性自然史”说法不相符。PAM可进展形成动脉瘤,并可能进一步发展为动脉瘤破裂出血,因此对PAM患者的长期随访是相当重要的。PAM伴动脉瘤形成的患者的手术指征有待进一步明确。对PAM伴动脉瘤破裂出血患者的处理同动脉瘤破裂出血患者,但是否同时处理单纯动脉畸形团仍有待对该疾病的进一步多中心、随机对照研究加以阐明。.

Keywords: aneurysm; pure arterial malformation; subarachnoid hemorrhage.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aneurysm, Ruptured* / complications
  • Aneurysm, Ruptured* / diagnostic imaging
  • Aneurysm, Ruptured* / surgery
  • Angiography, Digital Subtraction
  • Cerebral Angiography
  • Humans
  • Intracranial Aneurysm* / complications
  • Intracranial Aneurysm* / diagnostic imaging
  • Intracranial Aneurysm* / surgery
  • Subarachnoid Hemorrhage* / diagnostic imaging
  • Subarachnoid Hemorrhage* / etiology