Review of the Treatment & Management of Hydrocephalus

Jacqueline Groat, PharmD Candidate; Joshua J. Neumiller, PharmD, CDE, CGP, FASCP

Disclosures

US Pharmacist. 2013;38(3):HS-8-HS-11. 

In This Article

Treatment Options

Although many causes of hydrocephalus exist, the number of treatments is limited. All successful, long-term treatments are surgical. There is little use for medication in hydrocephalus. In some acquired cases, as with tumors and infections, resolving the underlying condition will resolve the hydrocephalus, but most patients still require surgical intervention.[6]

There are generally two approaches to treating hydrocephalus. The most common treatment is the placement of a shunt.[3] In use since the 1950s, this approach is considered the best treatment option in most cases. The other procedure, endoscopic third ventriculostomy (ETV), involves the surgical creation of an opening in the floor of the third ventricle to enable the passage of CSF.[6,9]

Shunt Placement

As noted above, the standard treatment for hydrocephalus is shunt placement. Shunts are usually placed in the lateral ventricle and can have one of three different drainage points. The most common drainage site is the peritoneum, which is connected to the shunt with subcutaneous tubing.[3,8] This is known as a ventriculoperitoneal shunt. Two other types of shunts, ventriculopleural and ventriculoatrial, terminate in the pleural space and the internal jugular vein, respectively.[3] The last type, the lumboperitoneal shunt, is placed in the lumbar intradural space.[3] Shunt systems include a valve that controls the rate of drainage. The valve may have to be accessed surgically, or it may be placed so that adjustments can be made without further surgery (Figure 1).

Figure 1.

Placement of a Ventriculoperitoneal Shunt.

With current standards and infection control, the postoperative mortality rate for shunt placement is less than 5%.[10] Although shunt placement is considered the best treatment for hydrocephalus, it has several drawbacks. Shunt failure occurs at a rate of approximately 20% and can be caused by overdrainage, obstruction, or shunt collapse.[8,11] Infection, which eventually can lead to sepsis, occurs at a rate of 5% to 15%.[4] Antibiotic-infused shunt catheters appear to greatly reduce the rate of infection.[4]

ETV

As mentioned previously, ETV is an alternative treatment for hydrocephalus. It is indicated in patients with an obstruction that prevents CSF from draining between the third ventricle and the cortical subarachnoid spaces.[9] In this procedure, the floor of the third ventricle is punctured, allowing CSF to flow into the cortical subarachnoid space.[6,9] ETV is currently approved in the treatment of obstructive hydrocephalus and in patients who have had multiple shunt failures and replacements.[6,9] ETV also has been shown to be effective for treating NPH.[9] The procedure is difficult and requires that the third ventricle floor and the surrounding structures have dimensions appropriate for successful completion of the procedure.[6] Imaging must be performed before surgery to determine that the anatomy of these structures is appropriate.[6]

One risk of ETV is piercing an unseen artery on the opposite side of the third ventricle floor, and infection can occur, although the rate is lower than that for shunts. There is also a risk of the drain becoming occluded, which would necessitate additional surgery. ETV may not be effective in neonates. The survival rate is extremely low for infants younger than 3 months, but reaches 64% around age 6 months. ETV generally is not performed in neonates if other options are available.[10]

Lumbar Puncture

A short-term option for the treatment of hydrocephalus is to perform periodic lumbar punctures. This is a temporary approach to reduce the amount of CSF until a long-term treatment can be performed.[12] Lumbar puncture may be used to treat communicating hydrocephalus, although it is sufficient only for patients who are still able to absorb some CSF.[12] In some cases, a drain may be placed so that continuous lumbar tapping is not necessary; however, there is a relatively high rate of infection with this approach compared with serial lumbar punctures.[12] Lumbar puncture may be used in neonates, who have an extremely low surgical success rate with ETV.[13]

Noninvasive Treatments

At present, there are no significant noninvasive treatment alternatives. Some studies have shown that acetazolamide or furosemide may be acceptable for CSF fluid reduction, but these medications currently are used only on a temporary basis.[14] Both drugs act to reduce the production of CSF by the choroid plexus.[14] As with lumbar puncture, these agents are typically used in low-birthweight infants who will have a low success rate with shunt placement or ETV.[13] There is no evidence that either of these medications increases survival rates, and a Cochrane review concluded that therapy with acetazolamide or furosemide is neither effective nor safe for treating posthemorrhagic ventricular dilatation in infants.[14]

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