Department of Neurosurgery, Heinrich-Heine-University, Düsseldorf, Germany.
J Neurointerv Surg. 2011 Jun;3(2):160-2. doi: 10.1136/jnis.2010.003004.
Moyamoya disease (MMD) is a rare cerebrovascular disease usually characterized by progressive bilateral distal internal carotid artery stenosis or occlusion and its consequences. Direct (ie, extracranial-intracranial bypass) or indirect cerebral revascularization procedures are the most established and effective treatment strategies for MMD. The case history is presented of a patient with MMD with delayed severe intracranial and subarachnoid hemorrhage following intracranial carotid artery stent placement.
An 18-year-old women presented with a history of seizures, recurrent episodes of transient right hemiparesis and aphasia. Cerebral catheter angiography and perfusion CT scan with azetozaolamide challenge confirmed impaired left hemispheric perfusion due to severe bilateral carotid artery stenosis combined with pathological collaterals, consistent with moyamoya disease. Endovascular stenting of the left supraclinoidal internal cerebral artery was performed uneventfully. Five hours after treatment the patient presented with sudden headache, nausea and speech disorders and a CT scan revealed intracerebral and subarachnoid hemorrhage. Due to further rapid clinical deterioration, surgical removal of the hematoma and decompression were required. The patient's poor neurological outcome did not improve during the 2-year follow-up period.
This case report illustrates a severe intracerebral and subarachnoid hemorrhage following intracranial stenting of the internal carotid artery in a young patient with MMD. Potentially this complication could be attributed to high perfusion pressure breakthrough phenomenon, a known complication after cerebral high-flow bypass surgery. The role of intracranial stenting, despite established revascularization procedures in patients with MMD, therefore remains highly questionable.
烟雾病（MMD）是一种罕见的脑血管疾病，通常表现为双侧颈内动脉远端进行性狭窄或闭塞及其后果。直接（即颅内外旁路）或间接脑血管重建术是 MMD 最成熟和有效的治疗策略。本文报告了一例 MMD 患者，在颅内颈动脉支架置入后发生迟发性严重颅内和蛛网膜下腔出血。
一名 18 岁女性，有癫痫发作、反复发作性短暂右侧偏瘫和失语症病史。脑血管造影和 azetozaolamide 挑战灌注 CT 扫描证实由于严重双侧颈内动脉狭窄合并病理性侧支循环，导致左半球灌注受损，符合烟雾病。左颈内动脉虹吸段的血管内支架置入术顺利进行。治疗后 5 小时，患者出现突发性头痛、恶心和言语障碍，CT 扫描显示颅内和蛛网膜下腔出血。由于进一步迅速恶化的临床情况，需要进行血肿清除和减压手术。在 2 年的随访期间，患者的神经功能预后仍未见改善。
本例报告说明了年轻 MMD 患者颅内颈动脉支架置入后发生严重颅内和蛛网膜下腔出血。这种并发症可能归因于高灌注压突破现象，这是已知的脑高流量旁路手术后的一种并发症。尽管在 MMD 患者中采用了成熟的血管重建术，但颅内支架置入术的作用仍然存在很大的疑问。
