eMedicine Specialties > Neurology > Behavioral Neurology and Dementia
Hydrocephalus: Treatment & Medication
Updated: Aug 20, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Medical treatment in hydrocephalus is used to delay surgical intervention. It may be tried in premature infants with posthemorrhagic hydrocephalus (in the absence of acute hydrocephalus). Normal CSF absorption may resume spontaneously during this interim period.
- Medical treatment is not effective in long-term treatment of chronic hydrocephalus. It may induce metabolic consequences and thus should be used only as a temporizing measure.
- Medications affect CSF dynamics by the following mechanisms:
- Decreasing CSF secretion by the choroid plexus - Acetazolamide and furosemide
- Increasing CSF reabsorption - Isosorbide (effectiveness is questionable)
Surgical Care
- Surgical treatment is the preferred therapeutic option.13
- Repeat lumbar punctures (LPs) can be performed for cases of hydrocephalus after intraventricular hemorrhage, since this condition can resolve spontaneously. If reabsorption does not resume when the protein content of cerebrospinal fluid (CSF) is less than 100 mg/dL, spontaneous resorption is unlikely to occur. LPs can be performed only in cases of communicating hydrocephalus.
- Alternatives to shunting include the following:
- Choroid plexectomy or choroid plexus coagulation may be effective.
- Opening of a stenosed aqueduct has a higher morbidity rate and a lower success rate than shunting, except in the case of tumors. However, lately cerebral aqueductoplasty has gained popularity as an effective treatment for membranous and short-segment stenoses of the sylvian aqueduct. It can be performed through a coronal approach or endoscopically through suboccipital foramen magnum trans-fourth ventricle approach.
- In these cases, tumor removal cures the hydrocephalus in 80%.
- Endoscopic fenestration of the floor of the third ventricle establishes an alternative route for CSF toward the subarachnoid space. It is contraindicated in communicating hydrocephalus.
- Shunts eventually are performed in most patients. Only about 25% of patients with hydrocephalus are treated successfully without shunt placement. The principle of shunting is to establish a communication between the CSF (ventricular or lumbar) and a drainage cavity (peritoneum, right atrium, pleura). Remember that shunts are not perfect and that all alternatives to shunting should be considered first.
- A ventriculoperitoneal (VP) shunt is used most commonly. The lateral ventricle is the usual proximal location. The advantage of this shunt is that the need to lengthen the catheter with growth may be obviated by using a long peritoneal catheter.
- A ventriculoatrial (VA) shunt also is called a "vascular shunt." It shunts the cerebral ventricles through the jugular vein and superior vena cava into the right cardiac atrium. It is used when the patient has abdominal abnormalities (eg, peritonitis, morbid obesity, or after extensive abdominal surgery). This shunt requires repeated lengthening in a growing child.
- A lumboperitoneal shunt is used only for communicating hydrocephalus, CSF fistula, or pseudotumor cerebri.
- A Torkildsen shunt is used rarely. It shunts the ventricle to cisternal space and is effective only in acquired obstructive hydrocephalus.
- A ventriculopleural shunt is considered second line. It is used if other shunt types are contraindicated.
- Rapid-onset hydrocephalus with increased intracranial pressure (ICP) is an emergency. The following can be done, depending on each specific case:
- Ventricular tap in infants
- Open ventricular drainage in children and adults
- LP in posthemorrhagic and postmeningitic hydrocephalus
- VP or VA shunt
Consultations
- Neurosurgeon
- Neurologist
- Neurorehabilitation specialist
- Ophthalmologist
Diet
- Regular, as tolerated
Activity
- Most surgeons agree that, with the use of antisiphon devices, no special positioning is required after shunting. However, some surgeons used to leave patients in whom a standard shunt had been placed in a recumbent position for 1-2 days after surgery to minimize risk of subdural hematoma.
- In treatment of normal pressure hydrocephalus (NPH), gradual postoperative mobilization is recommended.
Medication
Acetazolamide (ACZ) and furosemide (FUR) treat posthemorrhagic hydrocephalus in neonates. Both are diuretics that also appear to decrease secretion of CSF at the level of the choroid plexus. ACZ can be used alone or in conjunction with FUR. The combination enhances efficacy of ACZ in decreasing CSF secretion by the choroid plexus. If ACZ is used alone, it appears to lower risk of nephrocalcinosis significantly.
Medication as treatment for hydrocephalus is controversial. It should be used only as a temporary measure for posthemorrhagic hydrocephalus in neonates.
Carbonic anhydrase inhibitors
These agents inhibit an enzyme found in many tissues of the body that catalyzes a reversible reaction in which carbon dioxide becomes hydrated and carbonic acid dehydrated. These changes may result in a decrease in CSF production by the choroid plexus.
Acetazolamide (Diamox)
Noncompetitive reversible inhibitor of enzyme carbonic anhydrase, which catalyzes the reaction between water and carbon dioxide, resulting in protons and carbonate. This contributes to decreasing CSF secretion by choroid plexus.
Adult
Pediatric
25 mg/kg/d PO tid; not to exceed 100 mg/kg/d
Alkalizes urine and may decrease excretion of amphetamines, procainamide, quinidine, flecainide, anticholinergics, and mecamylamine; may increase excretion and lower plasma levels of salicylate, phenobarbital, and lithium; can increase cyclosporine levels and decrease primidone levels; concurrent salicylates may increase accumulation and toxicity, including CNS depression and metabolic acidosis
Documented hypersensitivity; hepatic insufficiency, hyponatremia, hypokalemia, hyperchloremic acidosis, severe renal insufficiency, nephrocalcinosis, adrenal gland failure
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Can cause hyperglycemia in diabetics; concurrent digoxin can increase susceptibility to ACZ-induced hypokalemia; in patients taking other diuretics, ACZ can aggravate hypokalemia; can aggravate preexisting acidosis, which can be prevented by initiating prophylactic electrolyte replacement; this may consist of sodium citrate starting at 8 mEq/kg/d titrated, keeping serum bicarbonate levels >18 mEq/L and sodium and potassium within reference ranges
Obtain baseline CBC prior to initiating therapy; recheck regularly during therapy
Loop diuretics
These agents increase excretion of water by interfering with the chloride-binding cotransport system, which results from inhibition of reabsorption of sodium and chloride in the ascending loop of Henle and distal renal tubule.
Furosemide (Lasix)
Mechanisms proposed for lowering ICP include lowering cerebral sodium uptake, affecting water transport into astroglial cells by inhibiting cellular membrane cation-chloride pump, and decreasing CSF production by inhibiting carbonic anhydrase. Used as adjunctive therapy with ACZ in temporary treatment of posthemorrhagic hydrocephalus in neonates.
Adult
Pediatric
1 mg/kg/d IV
May increase ototoxic potential of aminoglycoside antibiotics; may increase salicylate toxicity if given with salicylate; may decrease arterial response to norepinephrine
Documented hypersensitivity to drug or sulfonylureas, hepatic coma, anuria, severe electrolyte depletion, concurrent ethacrynic acid (may cause ototoxicity), or lithium (may cause lithium toxicity)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Excessive use can cause dehydration and circulatory collapse; can cause electrolyte imbalance as hypokalemia, hyponatremia, hypochloremic alkalosis, hypomagnesemia, and hypocalcemia; therefore, monitor serum electrolytes; may increase blood glucose in patients with diabetes; may cause photosensitivity
More on Hydrocephalus |
| Overview: Hydrocephalus |
| Differential Diagnoses & Workup: Hydrocephalus |
 Treatment & Medication: Hydrocephalus |
| Follow-up: Hydrocephalus |
| Multimedia: Hydrocephalus |
| References |
| Further Reading |
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Keywords
normal pressure hydrocephalus, communicating hydrocephalus, noncommunicating hydrocephalus, obstructive hydrocephalus, arrested hydrocephalus, acute hydrocephalus, gait apraxia, incontinence, dementia, Arnold-Chiari malformation, papilledema, precocious puberty, Dandy–Walker malformation, obesity, delayed onset of puberty, urinary incontinence, Parkinsonism, seizures, toxoplasmosis, Bickers-Adams syndrome, mental retardation, medulloblastoma, astrocytoma, prematurity, achondroplasia, cysticercosis, treatment, diagnosis
