Treatment of refractory low-pressure hydrocephalus with an active pumping negative-pressure shunt system

Low-pressure hydrocephalus is a rare type of hydrocephalus characterized by negative intracranial pressure (ICP) and ventriculomegaly. Given the shortcomings of available methods to treat refractory low-pressure hydrocephalus, we set out to develop a new system for evacuation of cerebrospinal fluid (CSF) from the ventricular system where existing shunt systems do not produce the necessary gradient for CSF drainage. We retrospectively reviewed the charts of two patients with the diagnosis of negative-pressure hydrocephalus refractory to traditional treatments. We combined a traditional low-pressure, non-siphoning valve with a pumping chamber placed distal to the valve to create a system that could be actively pumped to remove excess CSF. Treatment of negative-pressure hydrocephalus requires the establishment of a lower ventricular drainage pressure than the drainage pressure in the subarachnoid space. In refractory cases, we propose the use of this active negative-pressure pumping system.