Background

Congenital hydrocephalus (HCP) is a developmental brain disorder characterized by the abnormal accumulation of cerebrospinal fluid within the ventricles. It is caused by genetic and acquired factors that start during early embryogenesis with disruption of the neurogerminal areas. As might be expected, early-onset hydrocephalus alters the process of brain development leading to irreparable neurological deficit. A primary alteration of the ependyma/neural stem cells (affecting vesicle trafficking and abnormal cell junctions) leads to its loss or denudation and translocation of neural progenitor cells (NPCs) and neural stem cells (NSCs) into the cerebrospinal fluid (CSF). Under these abnormal conditions, morphological and functional processes, underlying the concept of astroglial reaction, are initiated in an attempt to recover homeostasis in the periventricular zone. This astroglial reaction includes astrocyte hypertrophy, hyperplasia, and development of a new layer with reorganized functional features that resemble the ependyma. Despite decades of research, there is a lack of information concerning the biological basis of the brain abnormalities that are associated with HCP.

Discussion

The present review of current literature discusses the neuropathological changes during gestation following the onset of congenital hydrocephalus and the unanswered questions into the pathophysiology of the disease. A better understanding of those missing points might help create novel therapeutic strategies that can reverse or even prevent the ultimate neurological impairment that affects this population and improve long-term clinical outcome.

中文翻译：

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先天性脑积水对胎儿脑部的损害。

背景

先天性脑积水（HCP）是一种发展性脑部疾病，其特征是脑室内脑脊液的异常积聚。它是由遗传和后天因素引起的，这些因素始于早期胚胎发生，并破坏了神经胚区。可以预见的是，早发性脑积水会改变大脑发育过程，导致无法弥补的神经功能缺损。室管膜/神经干细胞的主要改变（影响囊泡运输和异常的细胞连接）导致其丢失或剥落，并导致神经祖细胞（NPC）和神经干细胞（NSC）移位到脑脊液（CSF）中。在这些异常条件下，星形胶质反应概念的基础是形态和功能过程，试图恢复脑室周围区域的稳态。这种星形胶质细胞反应包括星形胶质细胞肥大，增生，以及新层的发展，该新层具有类似于室管膜的重组功能特征。尽管进行了数十年的研究，但仍缺乏有关与HCP相关的大脑异常的生物学基础的信息。

讨论区

目前的文献综述讨论了先天性脑积水发作后妊娠过程中的神经病理学改变以及该病的病理生理学尚未解答的问题。对那些缺失点的更好理解可能有助于创建新颖的治疗策略，从而可以逆转甚至预防影响该人群的最终神经功能障碍并改善长期临床疗效。