A Contemporary Definition and Classification of Hydrocephalus

This review focuses on the problems related to defining hydrocephalus and on the development of a consensus on the classification of this common problem. Such a consensus is needed so that diverse research efforts and plans of treatment can be understood in the same context. The literature was searched to determine the definition of hydrocephalus and to identify previously proposed classification schemes. The historic perspective, purpose, and result of these classifications are reviewed and analyzed. The concept of the hydrodynamics of cerebrospinal fluid (CSF) as a hydraulic circuit is presented to serve as a template for a contemporary classification scheme. Finally, a definition and classification that include all clinical causes and forms of hydrocephalus are suggested. The currently accepted classification of hydrocephalus into “communicating” and “noncommunicating” varieties is almost 90 years old and has not been modified despite major advances in neuroimaging, neurosciences, and treatment outcomes. Despite a thorough search of the literature using computerized search engines and bibliographies from review articles and book chapters, I identified only 6 previous attempts to define and classify different forms of hydrocephalus. This review proposes the following definition for hydrocephalus: hydrocephalus is an active distension of the ventricular system of the brain related to inadequate passage of CSF from its point of production within the ventricular system to its point of absorption into the systemic circulation. Based on this definition (potential points of flow restriction) and on the view of the CSF system as a hydraulic circuit, a classification system is proposed. The acceptance of this proposed definition and classification schema would allow clinicians and basic scientists to communicate effectively, to share information and results, and to develop testable hypotheses.