What is the appropriate management of hydrocephalus?

Management of Hydrocephalus

Acute symptomatic hydrocephalus requires urgent cerebrospinal fluid (CSF) diversion via external ventricular drain (EVD) or lumbar drainage to prevent neurological deterioration and improve outcomes, while chronic symptomatic hydrocephalus necessitates permanent CSF diversion with ventriculoperitoneal shunt placement. 1, 2

Acute Hydrocephalus Management

Immediate CSF Diversion

• Perform urgent EVD placement or lumbar drainage for all patients with acute symptomatic hydrocephalus to improve neurological outcomes (Class I recommendation). 1, 2
• EVD is generally preferred as it allows continuous intracranial pressure monitoring and controlled CSF drainage. 2
• Lumbar drainage serves as an alternative when ventricular access is challenging or contraindicated, particularly in communicating hydrocephalus. 1, 2

EVD Protocol Implementation

• Implement a standardized EVD bundled protocol covering insertion technique, management, education, and monitoring to reduce infection rates from as high as 45% to <1%. 1, 2
• Key protocol elements include: aseptic insertion technique, proper skin preparation, appropriate catheter selection (consider antibiotic-impregnated catheters), standardized dressing type and change frequency, defined CSF sampling protocols, healthcare professional training requirements, and systematic tracking of catheter days and infection rates. 1

Troubleshooting EVD Failure

• When hydrocephalus worsens despite EVD in place, immediately verify catheter patency and positioning, optimize drainage parameters, and obtain urgent neuroimaging. 2
• Assess for catheter obstruction by blood clot or debris, particularly in intraventricular hemorrhage. 2
• For patients with intraventricular hemorrhage and Glasgow Coma Scale >3, add intraventricular thrombolytic therapy (alteplase or urokinase) to EVD irrigation as this significantly reduces mortality compared to EVD with saline alone (Class 2a recommendation). 2

Chronic Hydrocephalus Management

Permanent CSF Diversion

• Place a permanent ventriculoperitoneal shunt for all patients with chronic symptomatic hydrocephalus to improve neurological outcomes (Class I recommendation). 1
• Chronic shunt-dependent hydrocephalus occurs in 8.9% to 48% of patients following subarachnoid hemorrhage. 1
• Predictors of shunt dependency include: poor admission neurological grade, increased age, acute hydrocephalus requiring EVD, high Fisher grades, intraventricular hemorrhage presence, rebleeding, and prolonged EVD duration. 1

EVD Weaning Strategy

• Do not perform prolonged EVD weaning protocols (>24 hours) as this does not reduce the need for permanent shunt placement and only delays definitive treatment (Class III recommendation). 1, 2

Special Population: Posthemorrhagic Hydrocephalus in Premature Infants

Temporizing Measures

• Use ventricular access devices (VADs), ventriculosubgaleal (VSG) shunts, or external ventricular drains as temporizing options based on clinical judgment (Level II recommendation). 1, 3
• VSG shunts reduce the need for daily CSF aspiration compared to VADs. 1
• VADs are preferred over EVDs as they reduce morbidity and mortality. 1, 3

What NOT to Do in Premature Infants

• Do not use serial lumbar punctures routinely to reduce shunt placement need or prevent hydrocephalus progression (Level I recommendation with high clinical certainty). 1, 3
• Do not use acetazolamide and furosemide as they do not decrease permanent shunt need and actually increase death and neurological morbidity at 1 year (Level I recommendation). 1, 3
• Do not use intraventricular thrombolytics (tPA, urokinase, or streptokinase) as they do not reduce shunt placement need and may increase secondary intraventricular hemorrhage (Level I recommendation). 1, 3

Timing of Permanent Shunt in Infants

• Delay permanent shunt insertion in premature infants when possible, using temporary CSF diversion first, as early permanent shunt placement results in twice as many revisions at 3-year follow-up. 3
• This approach allows blood products to dissipate from CSF, reducing infection risk and minimizing lifelong shunt complications. 3

Subarachnoid Hemorrhage-Associated Hydrocephalus

Acute Phase Management

• Acute hydrocephalus occurs in 15% to 87% of patients with aneurysmal subarachnoid hemorrhage. 1
• Perform CSF diversion (EVD or lumbar drainage) based on clinical scenario for acute symptomatic hydrocephalus (Class I, Level B evidence). 1
• The risk of aneurysm rebleeding with EVD placement remains controversial, with conflicting retrospective data. 1

Chronic Phase Management

• Do not perform routine fenestration of the lamina terminalis as it does not reduce shunt-dependent hydrocephalus rates. 1

Common Pitfalls to Avoid

• Do not delay imaging when clinical worsening occurs—urgent CT or MRI is essential to guide management decisions. 2
• Do not continue ineffective EVD management—if standard measures fail, escalate to thrombolytic therapy (if intraventricular hemorrhage present) or consider additional drainage options. 2
• Do not attribute all neurological worsening to hydrocephalus alone in subarachnoid hemorrhage patients—consider concurrent vasospasm, rebleeding, and seizures. 2
• Do not use repeated lumbar punctures as definitive treatment as they may contribute to subsequent shunt infection without preventing permanent shunt need. 3