Department of Neurosurgery, SUNY Upstate Medical University, Syracuse, NY 13210, USA.
Transl Pediatr. 2014 Jul;3(3):185-94. doi: 10.3978/j.issn.2224-4336.2014.07.02.
Hydrocephalus is a central nervous system disorder characterized by excessive accumulation of cerebrospinal fluid (CSF) in the ventricles of the brain. Cognitive and physical handicap can occur as a result of hydrocephalus. The disorder can present at any age as a result of a wide variety of different diseases. The pathophysiology of hydrocephalus is unclear. While circulation theory is widely accepted as a hypothesis for the development of hydrocephalus, there is a lack of adequate proof in clinical situations and in experimental settings. However, there is growing evidence that osmotic gradients are responsible for the water content of the ventricles of the brain, similar to their presence in other water permeable organs in the body. Therefore, brain disorders that results in excess macromolecules in the ventricular CSF will change the osmotic gradient and result in hydrocephalus. This review encompasses several key findings that have been noted to be important in the genesis of hydrocephalus, including but not limited to the drainage of CSF through the olfactory pathways and cervical lymphatics, the paravascular pathways and the role of venous system. We propose that as osmotic gradients play an important role in the water transport into the ventricles, the transport of osmotically active macromolecules play a critical role in the genesis of hydrocephalus. Therefore, we can view hydrocephalus as a disorder of macromolecular clearance, rather than circulation. Current evidence points to a paravascular and/or lymphatic clearance of these macromolecules out of the ventricles and the brain into the venous system. There is substantial evidence to support this theory, and further studies may help solidify the merit of this hypothesis.
脑积水是一种中枢神经系统疾病，其特征是脑室内脑脊液（CSF）过度积聚。脑积水可导致认知和身体残疾。由于各种不同疾病的原因，这种疾病可在任何年龄出现。脑积水的病理生理学尚不清楚。虽然循环理论被广泛接受为脑积水发展的假说，但在临床和实验环境中缺乏足够的证据。然而，越来越多的证据表明，渗透梯度是导致脑室含水量的原因，类似于它们在体内其他可渗透水的器官中的存在。因此，导致脑室 CSF 中大分子过量的大脑疾病会改变渗透梯度并导致脑积水。本综述涵盖了几个被认为对脑积水发生重要的关键发现，包括但不限于 CSF 通过嗅觉途径和颈部淋巴途径、脉络丛途径以及静脉系统的引流，我们提出，由于渗透梯度在水向脑室的转运中起着重要作用，因此渗透活性大分子的转运在脑积水的发生中起着关键作用。因此，我们可以将脑积水视为一种大分子清除障碍，而不是循环障碍。目前的证据指向这些大分子从脑室和脑通过脉络丛途径和/或淋巴途径进入静脉系统的清除。有大量证据支持这一理论，进一步的研究可能有助于确定这一假设的价值。
