Low-Pressure Hydrocephalus: A Case Report and Review of the Literature.
影响因子：1.723 PMID：28962963
期刊年卷：World Neurosurg 2018 Jan;109
BACKGROUND:The entity of low-pressure hydrocephalus remains poorly understood and thoroughly debated. Symptomatic improvement accompanied by decrease in ventricular size after prolonged subatmospheric drainage has been well documented, and this method has been considered the criterion standard of management. Few studies have examined alternative treatment options, either to avoid the risks associated with prolonged external ventricular drainage or because of the failure of traditional methods.
OBJECTIVES:This study compiled and examined reported cases of low-pressure hydrocephalus in an attempt to provide an up-to-date summary of the condition.
METHODS:A literature search was conducted by use of Ovid Medline and PubMed filtered for the past 25 years with specifickey terms, inclusion criteria, and exclusion criteria. Selected case studies and case series were then compared, and statistical analysis was performed where appropriate.
RESULTS:Over 25 years, 17 articles met our criteria. In addition to our case, 90 cases of LPH were reported. Magnetic resonance elastography (MRE) has proved to be an effective means of studying the viscoelastic properties of the brain. Endoscopic third ventriculostomy(ETV) appears to be a strong alternative, or additional, treatment.
CONCLUSION:MRE may prove to be effective instudying LPH because of its ability to quantify viscoelastic properties inresponse to therapy. Additionally, ETV should be considered in cases of LPH,although there is little evidence in the current literature to support its use.There are suggestions that it may lead to fewer shunt-dependent patients. Future studies are needed because there are few documented examples.
背景：人们低颅压脑积水的实质仍然知之甚少。长期的低于大气压引流后，症状改善伴有脑室缩小的证据已被充分记录，该方法已被视为治疗LPH的标准。很少有学者研究替代疗法，其原因或者是避免与延长脑室外引流时长相关的风险，或者是因为传统方法的失败。
方法：使用Ovid Medline和PubMed在过去25年中进行文献检索，包括特定的关键术语，纳入标准和排除标准。然后比较选定的案例研究和案例系列，并在进行统计分析。
结果：在25年中，有17篇文章符合本研究的标准。除本病例外，还报告了90例LPH。磁共振弹性成像（MRE）已被证明是研究脑粘弹性的有效手段。第三脑室造瘘术（ETV）似乎是一种强有力的替代方法。
结论：MRE可能被证明对LPH的研究有效，因为它能够量化对治疗效果的粘弹性质。此外，LPH可以考虑使用ETV，尽管在当前文献中几乎没有证据支持它的使用。有建议认为，这可能会较少分流依赖。但没有记录在案的病例，需要进一步的研究。
本文对LPH进行了一个病例分析并将近25年关于LPH的研究进行总结。
病例分析不痛不痒，汇报了一个后颅窝星形细胞瘤术后5年的9岁患者，术后2周做了VP+ETV，现因头痛入院，MR示脑室扩大，予负压引流后（反复调整负压水平）症状缓解，最后重置ETV阀门并更换脑室腹腔分流管后好转出院。
对25年文献的回顾用两个表格就能一清二楚。
本文的讨论比较有意思，通过3类来分析。（LPH的分类及定义；病理生理；治疗）
回顾文献表明，最早使用LPH指的是现在的正压性脑积水（NPH）。自Pang和Altschuler的报告发表以来，各学者使用了许多不同的术语来区分患者间的差异。 很难为LPH的诊断定义严格的标准，因为LPH向NPH的过渡仍然很模糊。仔细阅读已发表的案例后，很难用ICP来代替患者对治疗的反应。确实，LPH至NPH的患者需要负压引流以改善临床状况可能与负压患者一样危险。
病理生理学：
the viscoelastic model of Pang and Altschuler （粘弹性模型）
the poroelastic model （多孔弹性模型 → 改变脑室周渗透率，eg. SAH，颅内感染）
physiologic hysteresis （生理滞后）
an increase in parenchymal extracellular fluid rather than a decrease（脑实质细胞外液的增加而不是减少）
Post-Lumbar punture （腰穿后）
pathologic changes in the posteriorfossa （后颅窝相关的病理改变）
▲magnetic resonance elastography(MRE) 磁共振弹性成像
MRE可以帮助量化治疗后粘弹性的变化。MRE不仅可以用于预测治疗效果，还可以用于识别LPH的高危患者，并可能在LPH谱上区分临床表现相似的患者。
治疗：
1.负压引流（subzero drainage）：低于外耳道水平的引流
2.机械辅助的脑脊液引流
3.定制的大直径分流导管
4.强制休息
5.ETV（Hamiltonand Price showed that the long-term requirement for a shunt was 20% for thepatients treated with ETV versus 70% for those without ETV.）
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