Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

suitable for surgery

1st line – ventriculoperitoneal shunting or endoscopic third ventriculostomy

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suitable for surgery
1st line – 

ventriculoperitoneal shunting or endoscopic third ventriculostomy

Surgery should be considered in patients with a good response to any of the diagnostic cerebrospinal fluid (CSF) procedures, after an assessment of risks and benefits.

A catheter is placed into the lateral ventricle under general anesthesia, usually via the nondominant cerebral hemisphere, and the distal end placed in the peritoneal cavity via a line tunneled under the skin. The valve is usually positioned behind the ear. A programmable shunt is used.

After surgery, the shunt valve and subcutaneous catheter should be inspected for signs of infection or breakage.

Pumping the valve should elicit prompt refilling of the reservoir after compression. If this does not occur, a proximal obstruction is likely. Difficulty compressing the valve implies distal blockage.

Common shunt complications include subdural hematoma, mechanical obstruction, and infection, and occur in about 22% of patients.[32] Shunt obstruction may cause a sudden change in walking function, but this may also be due to stroke or subdural hematoma. Obstruction more commonly causes a gradual return of symptoms present before surgery.

The adjustment of shunt valve pressure is contentious. In one double-blind randomized controlled trial, gradual lowering of the valve setting to pressures below 12 cm water was associated with significantly better outcomes (but increased risk of overdrainage) compared with fixed valve setting at a mean of 13 cm water.[32] However, another double-blind RCT failed to find a significant difference in clinical improvement between gradual reduction of the valve setting compared with a fixed valve setting of 12 cm water.[33]

Underdrainage of CSF may produce recurrence of symptoms and hearing loss. Chronic overdrainage of CSF can cause subdural hematoma, headache, and tinnitus. One study found that gravitational valves avoid one additional overdrainage complication in approximately every third patient, compared with other valve types.[34] One Cochrane review concluded that the use of flow-regulated or differential pressure-regulated valves did not influence the outcome of shunting in this condition.[35]

Shunt malfunction can often be managed by external adjustment of the valve settings; however, shunt revision is usually required, and approximately 75% of these patients will respond positively.[31]

Endoscopic third ventriculostomy (ETV) is an alternative option to shunting. Unlike shunting, ETV does not require prosthesis implantation. However, preliminary results from an open label randomized trial found that shunting is superior to ETV, as it is associated with better functional neurologic outcomes 12 months after surgery.[36][37]

Consider – control of cardiovascular risk factors

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ONGOING
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suitable for surgery
Consider – 

control of cardiovascular risk factors

Treatment recommended for SOME patients in selected patient group

All patients should be offered treatment aimed at controlling their vascular risk factors, such as blood pressure control with antihypertensive drugs, smoking cessation, and cholesterol-lowering drugs. See Essential hypertension (Management approach), Smoking cessation (Management approach), and Hypercholesterolemia (Management approach).

not suitable for surgery

1st line – control of cardiovascular risk factors

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ONGOING
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not suitable for surgery
1st line – 

control of cardiovascular risk factors

Patients who refuse surgery, or those who are unsuitable for surgery, should be offered treatment aimed at controlling their vascular risk factors, such as blood pressure control with antihypertensive drugs, smoking cessation, and cholesterol-lowering drugs. See Essential hypertension (Management approach), Smoking cessation (Management approach), and Hypercholesterolemia (Management approach).

Consider – repeated large-volume cerebrospinal fluid (CSF) taps

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ONGOING
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not suitable for surgery
Consider – 

repeated large-volume cerebrospinal fluid (CSF) taps

Treatment recommended for SOME patients in selected patient group

Some patients who refuse surgery, or those who are unsuitable for surgery, may respond to repeated large-volume CSF taps, with apparent improvement sustained for weeks or months.

This involves the removal of 30 to 60 mL of CSF via lumbar puncture. The procedure improves cerebral blood flow temporarily, possibly by reducing the turgor of the subarachnoid space and relieving obstruction to CSF flow at the aqueduct.[21]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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