Kita Daisuke, Park Cheho, Hayashi Yasuhiko
Department of Neurosurgery, Noto General Hospital, Nanao, Ishikawa, Japan.
Department of Neurosurgery, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan.
NMC Case Rep J. 2019 May 25;6(3):83-86. doi: 10.2176/nmccrj.cr.2018-0244. eCollection 2019 Aug.
Developmental venous anomalies (DVAs) are generally asymptomatic; however, they can sometimes cause central nervous disorders. Aqueductal stenosis caused by DVAs is so rare that only 14 cases have been reported to date. Moreover, most patients are children or young adults, presenting with headaches or consciousness disturbances, associated with raised intracranial pressure. Here, we report on an 83-year-old man presenting with mimic symptoms of idiopathic normal pressure hydrocephalus (cognitive disorder, gait disturbance, and urinary urgency: Hakim's triad) because of obstructive hydrocephalus caused by a DVA located in the aqueduct. Endoscopic third ventriculostomy (ETV) was performed to relieve his symptoms, and the opening pressure of the lateral ventricle was recorded to be 10 cm-HO. Endoscopic examination of the intraventricular system clearly revealed a vein within the aqueduct converging with the adjacent subependymal vessels. These findings were compatible with the characteristics of DVAs. His symptoms improved after the ETV. This case suggested that DVAs within the aqueduct, despite of their congenital nature, could give rise to decompensated obstructive hydrocephalus even in elderly patients, resulting in Hakim's triad.
静脉发育异常(DVAs)通常无症状;然而,它们有时会引起中枢神经系统疾病。由DVAs引起的导水管狭窄极为罕见,迄今为止仅报道过14例。此外,大多数患者为儿童或年轻人,表现为头痛或意识障碍,并伴有颅内压升高。在此,我们报告一例83岁男性患者,因导水管处的DVA导致梗阻性脑积水,出现特发性正常压力脑积水的模拟症状(认知障碍、步态障碍和尿急:哈基姆三联征)。行内镜下第三脑室造瘘术(ETV)以缓解其症状,侧脑室开放压力记录为10 cm-H₂O。对脑室系统进行内镜检查清楚地显示导水管内有一条静脉与相邻的室管膜下血管汇合。这些发现符合DVAs的特征。ETV术后他的症状有所改善。该病例表明,导水管内的DVAs尽管具有先天性,但即使在老年患者中也可能导致失代偿性梗阻性脑积水,从而出现哈基姆三联征。