Hydrocephalus: Treatment

1. Goals of Treatment

2. Emergency Management

• Address the underlying cause first when possible (e.g., tumour excision causing obstruction).
• Temporary ventricular drainage is needed when waiting for definitive surgery or in a deteriorating patient.

3. Temporary CSF Diversion

External Ventricular Drain (EVD)

• Inserted through a burr hole at Kocher's point (right of midline, anterior to the coronal suture), perpendicular to the brain surface.
• Catheter tip rests adjacent to the foramen of Monro in the lateral ventricle.
• Can also be used to deliver intrathecal antibiotics.
• Lumbar drains are an alternative for communicating hydrocephalus.

Medical (Temporary)

• Acetazolamide (carbonic anhydrase inhibitor) + furosemide: reduces the rate of CSF production; used for slowly progressive hydrocephalus as a bridge to surgery, particularly in infants/neonates. Clinical effects are limited and not sustained long term.
• Not appropriate as definitive management.

4. Definitive Surgical Treatment

A. Ventriculoperitoneal (VP) Shunt - MAINSTAY

1. Proximal (ventricular) catheter - inserted into the lateral ventricle
2. Shunt valve - regulates flow by opening at a preset pressure; usually incorporates a CSF reservoir for percutaneous sampling
3. Distal catheter - tunnelled subcutaneously to the peritoneal cavity
4. Optional: anti-siphon device to prevent overdrainage in the upright position
5. Programmable valves - allow the opening pressure to be adjusted non-invasively using an external magnetic device

VP shunt valve examples - Bailey & Love's Short Practice of Surgery, 28th Ed.

• Ventriculoatrial (VA) shunt
• Ventriculopleural shunt
• Lumboperitoneal shunt (for communicating hydrocephalus)

B. Endoscopic Third Ventriculostomy (ETV)

• Especially useful for obstructive hydrocephalus (e.g., aqueduct stenosis).
• A neuroendoscope is passed into the frontal horn of the lateral ventricle, then through the foramen of Monro into the third ventricle.
• The floor of the third ventricle is opened between the mamillary bodies and the pituitary recess.
• Creates a bypass from the third ventricle to the basal subarachnoid cisterns - no implanted hardware, so no infection risk from tubing.
• ETV + Choroid Plexus Cauterization (ETV-CPC): combined procedure shown to be effective in >70% of patients with myelomeningocele, avoiding the need for VP shunting with similar neurocognitive outcomes. - Campbell-Walsh-Wein Urology
• Limitations: reblockage is common; many patients subsequently require a shunt. Long-term results have been disappointing as a primary approach in some series. - Adams and Victor's Principles of Neurology, 12th Ed.

5. Shunt Complications (15-20% require revision within 3 years)

6. Special Scenarios

Normal Pressure Hydrocephalus (NPH)

• Classic triad: gait disturbance, urinary incontinence, cognitive decline.
• Treated with VP shunting - ventricular size can normalize within 1-2 weeks.
• Gait disturbance improves more slowly than cognitive symptoms.
• Acetazolamide has minimal clinical effect in NPH, despite some imaging improvement. - Adams and Victor's, 12th Ed.

Idiopathic Intracranial Hypertension (IIH)

• Managed with weight loss, acetazolamide, serial lumbar puncture.
• CSF diversion (VP shunt or lumboperitoneal shunt) is a last resort. - Bailey and Love's, 28th Ed.

Infantile/Congenital Hydrocephalus

• VP shunting is standard; ETV-CPC is an effective shunt-sparing alternative in many cases.
• Risk of slit ventricle syndrome is higher in young children with early shunting.
• Whether to shunt all hydrocephalic infants at birth is controversial given the relatively low proportion surviving with normal mental function in several series. - Adams and Victor's, 12th Ed.

7. Summary

• [Bailey and Love's Short Practice of Surgery, 28th Ed.](Bailey and Love's Short Practice of Surgery, 28th Ed., p. 727)
• [Adams and Victor's Principles of Neurology, 12th Ed.](Adams and Victor's Principles of Neurology, 12th Ed., p. 640)
• [The Harriet Lane Handbook, 23rd Ed.](The Harriet Lane Handbook, 23rd Ed.)
• [Campbell-Walsh-Wein Urology](Campbell-Walsh-Wein Urology, 3-Volume Set)
• Recent PubMed: PMID 41525875 - 2026 systematic review on low/negative pressure hydrocephalus management; PMID 40825578 - 2025 systematic review on fetal hydrocephalus surgical interventions.