Ravindra Vijay M, Karsy Michael, Schmidt Richard H, Taussky Philipp, Park Min S, Bollo Robert J
Department of Neurosurgery, Clinical Neurosciences Center, University of Utah; and.
Division of Pediatric Neurosurgery, Primary Children's Hospital, University of Utah, Salt Lake City, Utah.
J Neurosurg Pediatr. 2016 Oct;18(4):463-470. doi: 10.3171/2016.5.PEDS16115. Epub 2016 Jul 1.
The authors report the case of a previously healthy 6-month-old girl who presented with right arm and leg stiffening consistent with seizure activity. An initial CT scan of the head demonstrated acute subarachnoid hemorrhage in the basal cisterns extending into the left sylvian fissure. Computed tomography angiography demonstrated a 7 × 6 × 5-mm saccular aneurysm of the inferior M division of the left middle cerebral artery. The patient underwent left craniotomy and microsurgical clip ligation with wrapping of the aneurysm neck because the vessel appeared circumferentially dysplastic in the region of the aneurysm. Postoperative angiography demonstrated a small remnant, sluggish distal flow, but no significant cerebral vasospasm. Fifty-five days after the initial aneurysm rupture, the patient presented again with an acute intraparenchymal hemorrhage of the left anterior temporal lobe. Angiogram revealed a circumferentially dysplastic superior division of the M branch, with a new 5 × 4-mm saccular aneurysm distinct from the first, with 2 smaller aneurysms distal to the new ruptured aneurysm. Endovascular parent vessel occlusion with Onyx was performed. Genetic testing revealed a mutation of the MYH11. To the authors' knowledge, this is the first report of rapid de novo aneurysm formation in an infant with an MYH11 mutation. The authors review the patient's clinical presentation and management and comprehensively review the literature on this topic.
作者报告了一名6个月大的既往健康女童的病例，该女童出现右臂和腿部僵硬，符合癫痫发作活动表现。头部初次CT扫描显示基底池急性蛛网膜下腔出血，延伸至左侧外侧裂。计算机断层血管造影显示左侧大脑中动脉M段下分支有一个7×6×5毫米的囊状动脉瘤。由于动脉瘤区域的血管在圆周方向上发育异常，该患者接受了左开颅手术和动脉瘤颈部包裹的显微外科夹闭术。术后血管造影显示有一小残余，远端血流缓慢，但无明显脑血管痉挛。在初次动脉瘤破裂55天后，患者再次出现左前颞叶急性脑实质内出血。血管造影显示M分支上部分支在圆周方向上发育异常，有一个与第一个不同的新的5×4毫米囊状动脉瘤，在新破裂动脉瘤远端还有2个较小的动脉瘤。遂进行了用Onyx栓塞供血血管的血管内治疗。基因检测显示MYH11突变。据作者所知，这是关于一名患有MYH11突变的婴儿中快速新生动脉瘤形成的首例报告。作者回顾了患者的临床表现和治疗情况，并全面回顾了关于该主题的文献。
