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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