Review of the Treatment & Management of Hydrocephalus

Abstract and Introduction

Abstract

In hydrocephalus, a disease or anatomical defect causes an increase in the amount of cerebrospinal fluid (CSF) present in the cranium, which most commonly results in increased pressure against the brain tissue. Hydrocephalus is difficult to diagnose because of its varied signs and symptoms, which commonly overlap with those of other medical conditions. There are generally two approaches to the treatment of hydrocephalus: 1) shunt placement and 2) endoscopic third ventriculostomy (surgical creation of an opening in the floor of the third ventricle to enable the passage of CSF). The treatment of hydrocephalus often requires long-term care and lifelong follow-up, especially in children and neonates in whom there is a congenital cause.

Introduction

Hydrocephalus is a condition in which a disease state or defect causes an increase in the amount of cerebrospinal fluid (CSF) present in the cranium, most commonly resulting in increased pressure against the brain tissue.^[1] The word hydrocephalus translates to "water on the brain," but the condition is caused by a variety of disorders and disease states, thus making it complex to define and diagnose.^[1] Most CSF is produced by the choroid plexus in the third and fourth ventricles in the brain.^[2] It fills the subarachnoid spaces, protecting and cushioning the brain.^[2] As more CSF is produced, it runs from the brain to the spinal cord and is removed from circulation via the arachnoid villi and the vertebral venous plexus.^[2] When hydrocephalus occurs, part of this process is blocked, although different parts are blocked depending upon the type of hydrocephalus. Causes range from obstructions to congenital diseases (Table 1).^[1,3–5]

Table 1. Causes of Hydrocephalus
Inherited	Acquired
Myelodysplasia	Tumor
Atresia of foramen of Monro	Meningitis
Neural tube defects:	Infection
Encephalocele	Hemorrhage
Spina bifida	Arachnoid cyst
Genetic abnormalities:	Posterior fossa cyst
Aqueductal stenosis	Traumatic brain injury
Dandy-Walker complex	Idiopathic
Other cerebral and spinal malformations

Source: References 1, 3–5.

Table 2. Differentiating Between Hydrocephalus and Diseases With Similar Presentation
Disease State	Symptoms Shared With Hydrocephalus	Symptoms Not Shared With Hydrocephalus
Alzheimer's disease	Decreased memory function Personality changes Incontinence Cognitive impairment Possible gait disturbances	Social withdrawal Usually begins in one's 60s Aphasia and apraxia Difficulty sleeping
Parkinson's disease	Gait disturbances Confusion Cognitive impairment	Gait disturbances responsive to levodopa Cogwheel rigidity Sialorrhea
Creutzfeldt-Jakob disease	Confusion Gait disturbances Decreased coordination Muscle spasms Extreme sleepiness Personality changes	No loss of bladder control Usually begins in one's late 50s Hallucinations
Vascular dementia	Confusion Cognitive impairment Incontinence	Emotional instability Possible signs of stroke on MRI Difficulty sleeping
Source: Reference 8.

Table 3. Hydrocephalus Treatment Options
Intervention	Indication	Outcome	Complications
Medication (furosemide, acetazolamide)	Decrease production of CSF due to increased fluid excretion	Temporary relief of increased CSF until surgical intervention is possible	No direct evidence of effectiveness versus waiting until surgical intervention is possible; potential increased risk of complications
Lumbar puncture	Remove excess CSF through spine to reduce pressure	Temporary relief of increased CSF until surgical intervention is possible	Possible increased risk of infection from multiple perforations
Shunt placement	All classifications of hydro- cephalus in which patient is able to undergo surgery	Relief through drainage of excess CSF	Shunt collapse, infection, shunt failure, possible need for surgical adjustment or replacement
ETV	Obstructive hydrocephalus, shunt failure	Relief through drainage of excess fluid	Occlusion of puncture site, difficulty performing procedure, infection, hemorrhage, nerve damage

CSF: cerebrospinal fluid; ETV: endoscopic third ventriculostomy.
Source: References 3, 4, 6, 8, 10–14.

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Review of the Treatment & Management of Hydrocephalus

Abstract and Introduction

Abstract

Introduction

Tables

Tables

Tables

References

Authors and Disclosures

Authors and Disclosures

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