What is the primary treatment for hydrocephalus?

Primary Treatment for Hydrocephalus

The primary treatment for hydrocephalus is cerebrospinal fluid (CSF) diversion through surgical placement of a shunt system or endoscopic third ventriculostomy, depending on the clinical scenario and type of hydrocephalus. 1

Types of Surgical Interventions

Shunt Systems

Ventriculoperitoneal (VP) shunting is the most common surgical treatment for hydrocephalus 2, 3. The procedure involves:

• Placement of a proximal catheter in the ventricle
• Tunneling of the catheter under the skin
• Placement of the distal catheter into the peritoneal cavity

Alternative shunt types include:

• Lumboperitoneal (LP) shunts - suitable for communicating hydrocephalus
• Ventriculopleural shunts - used when the peritoneal cavity is unavailable
• Ventriculoatrial shunts - used in select cases

Endoscopic Procedures

• Endoscopic Third Ventriculostomy (ETV): In centers with neuro-endoscopic expertise, ETV is the preferred procedure for obstructive hydrocephalus 1
• Benefits include avoiding shunt dependency and potential for simultaneous tumor biopsy when applicable
• Lower complication rates compared to shunting based on systematic analysis 1

Management Algorithm

Acute Symptomatic Hydrocephalus

1. Initial Management: External ventricular drainage (EVD) or lumbar drainage, depending on the clinical scenario 1
2. For increased intracranial pressure (ICP): Medical therapy and repeated lumbar punctures as initial management 1
3. For persistent hydrocephalus: Early MRI of the brain and neurosurgical consultation 1

Chronic Symptomatic Hydrocephalus

• Treatment: Permanent CSF diversion with shunt placement 1
• Most patients who develop increased ICP will not resolve without placement of a permanent shunt 1

Advances in Surgical Techniques

Several technical advances have improved outcomes:

• Neuronavigation-guided proximal catheter placement 4
• Laparoscopic or endoscopic assistance for distal catheter placement 3, 4
• Single-incision laparoscopic techniques for pediatric patients 2

Medical Management

While surgical intervention remains the mainstay of treatment, medical management may be considered in specific circumstances 5:

• Carbonic anhydrase inhibitors: Acetazolamide (alone or with furosemide) is the most suitable medical option
• Limitations: Medical therapy alone is generally insufficient for definitive management of hydrocephalus
• Role: May serve as a temporary measure or adjunct to surgical management

Management of Shunt Complications

Shunt Malfunction

• Occurs in approximately 7.3% of VP shunt cases 6
• Requires prompt revision

Shunt Infection

• Occurs in 1.0-5.7% of cases 6
• For infected shunts, the recommendation is to remove the infected shunt and place a replacement at a subsequent time as a second procedure 1

Overdrainage Complications

• Can be mitigated with programmable valves 6

Important Considerations

1. Early diagnosis and treatment are essential to prevent permanent neurological damage
2. Regular follow-up imaging to monitor ventricular size and shunt function
3. Patient education regarding signs of shunt malfunction or infection
4. Multidisciplinary approach involving neurosurgery, neurology, and critical care specialists

The choice between VP shunt and ETV should be based on the etiology of hydrocephalus, patient age, and anatomical considerations. Recent evidence suggests that LP shunts with programmable valves may have lower revision rates than traditional VP shunts in selected patients 6.