Craven Claudia L, Baudracco Irene, Thompson Simon D, Thorne Lewis, Watkins Laurence D, Toma Ahmed K
Victor Horsley Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, London, United Kingdom.
Victor Horsley Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, London, United Kingdom.
World Neurosurg. 2018 Feb;110:e514-e519. doi: 10.1016/j.wneu.2017.11.032. Epub 2017 Nov 16.
Complex hydrocephalus affecting lateral and fourth ventricles separately is occasionally managed with cerebrospinal fluid diversion via supratentorial and infratentorial ventricular catheters. The optimal configuration to reduce complications is currently unknown in adults. We describe a consistently similar clinical presentation of patients with complex hydrocephalus and a fourth ventricle separately drained by infratentorial shunt insertion.
This was a retrospective single-center case series. Medical notes were reviewed for clinical presentation, brain imaging, and neurophysiologic tests results. All patients underwent intracranial pressure monitoring (ICPM). Outcomes were determined by ventricular appearance on brain imaging computed tomography and symptomatic improvements postoperatively.
Five adult patients referred to the hydrocephalus service had separate infratentorial and supratentorial shunt systems. A common clinical presentation was observed, including lower motor neuron facial palsy (confirmed with electrophysiology), ophthalmoplegia, dysarthria, impaired gait headache, and nausea. We refer to this as transtentorial distortion syndrome. Twenty-four-hour ICPM demonstrated clear low pressures. All patients underwent shunt revision connecting the transtentorial shunts via a Y-connector and the addition of a distal valve. All subjects had improved ventricular appearance on computed tomography scans post revision, and normalization of ICPM was observed. In the follow-up period of 6 months, no patient required further shunt revision.
To prevent transtentorial distortion syndrome, supratentorial and infratentorial shunt constructs in adults with encysted fourth ventricles should be similar to the shunt systems widely known in the pediatric population with Dandy-Walker syndrome (i.e., joint output to a single valve distal to the connection of the 2 proximal drainage catheters).
分别影响侧脑室和第四脑室的复杂性脑积水偶尔通过经幕上和幕下脑室导管进行脑脊液分流来治疗。目前，在成人中，减少并发症的最佳配置尚不清楚。我们描述了复杂性脑积水患者以及通过幕下分流管单独引流第四脑室的患者一致相似的临床表现。
这是一项回顾性单中心病例系列研究。回顾医疗记录以获取临床表现、脑成像和神经生理学测试结果。所有患者均接受了颅内压监测（ICPM）。结局通过脑成像计算机断层扫描上的脑室外观以及术后症状改善来确定。
五名转诊至脑积水服务中心的成年患者有单独的幕下和幕上分流系统。观察到一种常见的临床表现，包括下运动神经元性面瘫（经电生理学证实）、眼肌麻痹、构音障碍、步态障碍、头痛和恶心。我们将此称为经幕扭曲综合征。24小时ICPM显示明显的低压。所有患者均接受了分流管修复，通过Y形连接器连接经幕分流管并增加了一个远端瓣膜。所有受试者在修复后的计算机断层扫描上脑室外观均有改善，并且观察到ICPM恢复正常。在6个月的随访期内，没有患者需要进一步的分流管修复。
为预防经幕扭曲综合征，患有包裹性第四脑室的成人的幕上和幕下分流结构应类似于患有丹迪-沃克综合征的儿童人群中广为人知的分流系统（即，两个近端引流导管连接点远端的联合输出至单个瓣膜）。
