Pathophysiology of long-standing overt ventriculomegaly in adults
Pathophysiology of long-standing overt ventriculomegaly in adults
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DOI：
10.3171/jns.2000.92.6.0933
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发表时间：
2000-06-01
影响因子：
4.1
通讯作者：
Sato, O
中科院分区：
文献类型：
--
作者：
Oi, S;Shimoda, M;Sato, O
Object.成人长期明显脑室扩大症（LOVA）是一种独特的脑积水形式，在儿童时期发展，并在成年期表现出症状。本研究的目的是分析LOVA的具体病理生理特征。LOVA的具体诊断标准包括成人重度脑室扩大，与测量头围超过两个标准差的大头畸形和/或蝶鞍显著扩张或破坏的神经放射学证据相关。回顾性研究了20例符合这些标准的患者，14例男性和6例女性。这些患者的诊断年龄范围为15至61岁（平均39.4岁）。所有患者的症状和/或体征均表明脑积水首次发生于出生时或婴儿期，且无任何已知的基础疾病。作者进行了一项病理生理学研究，包括磁共振（MR）成像的特定变化，如液体衰减反转恢复和心脏门控电影模式成像;颅内压（ICP）监测;三维计算机断层扫描（CT）扫描;以及其他技术。所有患者的脑积水均由导水管狭窄引起。17例严重侧脑室和第三脑室扩大伴蝶鞍明显扩张或破坏。心脏门控电影磁共振成像并未显示导水管中的脑脊液有任何显着移动。三维CT脑室造影证实，扩大的第三脑室突出到鞍内，有时，延伸到憩室。14例患者出现症状和体征，提示ICP升高伴明显压力波。11例患者出现痴呆或智力低下，12例出现步态障碍，8例出现尿失禁; 7例患者出现上述三种症状。13例患者出现视力障碍。9例患者接受脑室腹腔分流植入术作为初始治疗，导致所有7例使用压差阀的患者术后硬膜下血肿。9例患者（其中3例最初接受分流术治疗）接受了神经内镜手术，主要用于第三脑室造口术。术后，颅内压恢复正常，并在电影MR成像上识别出接受内窥镜治疗的患者的脑室造口术部位有明显的来回脉动运动。然而，脑室扩大几乎没有改善。因此，所有患者最终都表现出对配有压力可调阀的分流或内窥镜手术的反应有所改善;然而，6例患者出现抑郁，需要精神科咨询或药物治疗。颅内顺应性显著降低而颅内压动态增高是LOVA的病理生理特征。治疗方案应根据个体的具体病理生理学组成来确定。
Object. Long-standing overt ventriculomegaly in adults (LOVA) is a unique form of hydrocephalus that develops during childhood and manifests symptoms during adulthood. The aim of the present study was to analyze the specific pathophysiological characteristics of LOVA.Methods. The specific diagnostic criteria for LOVA include severe ventriculomegaly in adults that is associated with macrocephalus measuring more than two standard deviations in head circumference and/or neuroradiological evidence of a significantly expanded or destroyed sella turcica. Twenty patients who fulfilled these criteria, 14 males and six females, were retrospectively studied. These patients' ages at diagnosis ranged from 15 to 61 years (mean 39.4 years). All had symptoms and/or signs indicating that hydrocephalus first occurred at birth or during infancy in the absence of any known underlying disease. The authors performed a pathophysiological study that included specific variations of magnetic resonance (MR) imaging, such as fluid-attenuated inversion recovery and cardiac-gated cine-mode imaging; intracranial pressure (ICP) monitoring; three-dimensional computerized tomography (CT) scanning; and other techniques.Hydrocephalus was caused by aqueductal stenosis in all patients. Severe ventriculomegaly involving the lateral and third ventricles was associated with a marked expansion or destruction of the sella turcica in 17 cases. Cardiac-gated cine-MR imaging did not reveal any significant movements of cerebrospinal fluid in the aqueduct. Three-dimensional CT ventriculography confirmed that the expanded third ventricle protruded into the sella and, sometimes, extended a diverticulum. Fourteen patients revealed symptoms and signs that indicated increased ICP with prominent pressure waves. Dementia or mental retardation was seen in 11 patients, gait disturbance in 12, and urinary incontinence in eight; all three of these symptoms were observed in seven patients. Thirteen patients experienced visual disturbance. Nine patients underwent ventriculoperitoneal shunt implantation as the initial treatment, leading to postoperative subdural hematoma in all seven cases in which a differential pressure valve was used. Nine patients, three of whom were initially treated by shunt placement, underwent a neuroendoscopic procedure, mainly for third ventriculostomy. Postoperatively, ICP returned to normal, and marked to-and-fro pulsatile movements at the site of ventriculostomy were recognized on cine-MR imaging in patients treated endoscopically. However, the ventriculomegaly was little improved. Consequently, all patients eventually demonstrated improvement in response to either a shunt equipped with a pressure-programmable valve or an endoscopic procedure; however, depression appeared in six patients, who required psychiatric consultation or medication.Conclusions. Such remarkably decreased intracranial compliance but relatively high ICP dynamics are the pathophysiological characteristics of LOVA. The therapeutic regimen should be determined based on the individual's specific pathophysiological makeup.