Chronic negative-pressure hydrocephalus: management of prolonged subatmospheric drainage failure and treatment approach in persistent ventriculomegaly. Illustrative case.

Background: Chronic negative-pressure hydrocephalus (cNegPH) is a rare condition, with only a few cases reported worldwide. Improvement of symptoms correlates with a reduction in ventricular size, which has been well documented in low-pressure hydrocephalus after prolonged subatmospheric cerebrospinal fluid (CSF) drainage and is considered the standard of care in such cases. However, the management of treatment failure and manifestation of cNegPH is still challenging, as well-documented treatment concepts are lacking.

Methods: Ventriculomegaly in a 36-year-old posthemorrhage, posttrauma patient with hydrocephalus remained refractory to low-gradient ventriculoperitoneal shunting, neck wrapping, endoscopic third ventriculostomy, and prolonged subatmospheric CSF drainage. Extended clinical care with multiple surgical and diagnostic interventions such as repeated replacements of the external ventricular drainage catheter, ventriculoperitoneal shunt revisions, and multiple cranial computed tomography scans was required in the course of the diagnosis. Complications including shunt malfunction, infection, meningitis, and recurrent clinical deteriorations were observed.

Conclusions: Diagnosing cNegPH can be challenging, and the failure of common treatment approaches has to be accepted after a certain point in time. At such a point, alternative options must be considered to provide outpatient care and reduce the risk of treatment complications. Intermittent shunt reservoir pressing proved to be an adequate long-term treatment option with a favorable outcome at the 6-month follow-up. https://thejns.org/doi/10.3171/CASE24672.