Oi Shizuo, Inagaki Takayuki, Shinoda Masaki, Takahashi Satoshi, Ono Shigeki, Date Isao, Nomura Sadahiro, Miwa Tomoru, Araki Takashi, Ito Susumu, Uchikado Hisaaki, Takemoto Osamu, Shirane Reizo, Nishimoto Hiroshi, Tashiro Yuzuru, Matsumura Akira
Health Sciences Asia Executive Dean Office, Japan International University, 2-18-1 Nakoji, Amagasaki, Hyogo 661-8530, Japan.
Childs Nerv Syst. 2011 Oct;27(10):1563-70. doi: 10.1007/s00381-011-1541-7. Epub 2011 Sep 17.
Hydrocephalus does not indicate a single clinical entity, but includes a variety of clinicopathological conditions caused by excessive cerebrospinal fluid (CSF) based on the disturbed circulation. Recent progress in prenatal neuroimagings such as MRI and ultrasound echoencephalography on fetus enables to understand clinicopathological conditions of CSF circulation disorder in conjunction with morphological changes in the central nervous system properly. It has been revealed that the CSF dynamics develop in the theory of evolution from the immature brain, as in the animals with the minor CSF pathway predominance, towards matured adult human brain together with the completion of the major CSF pathway: the "Evolution Theory in CSF Dynamics". Now, we can analyze CSF circulation dynamically and also analyze the flow velocity and direction of CSF movement. CENTER OF EXCELLENCE-FETAL HYDROCEPHALUS TOP 10 JAPAN: Along with this technical improvement, the standards of clinicopathological evaluation of hydrocephalus as well as the classification and concept of hydrocephalus shall undergo a major upgrade. Based on such remarkable improvement in the recent practical diagnostic evaluation of fetal hydrocephalus, it is now required to update the guideline for management and treatment of fetal and congenital hydrocephalus, and a nationwide study group; Center of Excellence-Fetal Hydrocephalus Top 10 Japan, was organized in 2008 in Japan. The retrospective analysis of 333 cases of congenital hydrocephalus indicated a fact that 43% of these cases were diagnosed prenatally, and the majority of cases were treated in these top 10 institutes in Japan. Now, congenital hydrocephalus diagnosed immediately after birth is regarded as to be based on embryonic stage; brain disorder in patients with congenital hydrocephalus should be considered in conjunction with neuronal mature process of embryonic stage. The fact is supported by the current trends in hydrocephalus research represented by "Perspective Classification of Congenital Hydrocephalus" and "Multi-categorical Hydrocephalus Classification". The ultimate goal of hydrocephalus treatment remains achieving arrested hydrocephalus by shunt surgeries. In the future, to achieve arrested hydrocephalus, minimum quantity of CSF to be drained should be elucidated. Consideration for accurate operative indication of ETV along with new neuroendoscopic device development and analysis of CSF circulation is expected in the future. The data in this prospective multicenter analysis in this guideline are credited in Oxford Evidence level 2b (Grade II).
脑积水并非单一的临床实体，而是包括因脑脊液（CSF）循环紊乱导致脑脊液过多所引起的多种临床病理状况。胎儿产前神经影像学检查（如MRI和超声脑回波描记术）的最新进展，使人们能够结合中枢神经系统的形态变化，恰当地了解脑脊液循环障碍的临床病理状况。现已发现，脑脊液动力学在进化理论中是从不成熟的大脑发展而来的，就像在脑脊液次要通路占主导的动物中一样，随着主要脑脊液通路的完善，逐渐发展为成熟的成人大脑，即“脑脊液动力学进化理论”。现在，我们可以动态分析脑脊液循环，还能分析脑脊液流动的速度和方向。日本胎儿脑积水卓越中心排名前十：随着这项技术的进步，脑积水临床病理评估的标准以及脑积水的分类和概念都将经历重大升级。基于近期胎儿脑积水实际诊断评估的显著改善，现在需要更新胎儿及先天性脑积水管理和治疗的指南，2008年在日本成立了一个全国性研究小组——日本胎儿脑积水卓越中心排名前十。对333例先天性脑积水病例的回顾性分析表明，其中43%的病例是在产前诊断出来的，并且大多数病例在日本这前十家机构接受了治疗。现在，出生后立即诊断出的先天性脑积水被认为是基于胚胎期的；先天性脑积水患者的脑部疾病应结合胚胎期的神经元成熟过程来考虑。“先天性脑积水的视角分类”和“多分类脑积水分类”所代表的当前脑积水研究趋势支持了这一事实。脑积水治疗的最终目标仍然是通过分流手术实现脑积水不再进展。未来，为了实现脑积水不再进展，需要明确应引流的脑脊液的最小量。预计未来会考虑准确的第三脑室造瘘术（ETV）手术指征，同时开发新的神经内镜设备并分析脑脊液循环。本指南中这项前瞻性多中心分析的数据属于牛津证据水平2b（二级）。
