Hydrocephalus

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Hydrocephalus Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

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Epidemiology & Demographics

Risk Factors

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Natural History, Complications & Prognosis

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Physical Examination

Electrocardiogram

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Case #1

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Risk calculators and risk factors for Hydrocephalus

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun M.D., PhD.,Kalsang Dolma, MBBS

For patient information click here

Synonyms and keywords: Water on the brain

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Common Causes, Causes by Organ System, Causes In Alphabetical Order


Hydrocephalus Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Epidemiology & Demographics

Risk Factors

Screening

Natural History, Complications & Prognosis

Diagnosis

Diagnostic Study of Choice

History & Symptoms

Physical Examination

Electrocardiogram

X-ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Hydrocephalus On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Hydrocephalus

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

National Guidelines Clearinghouse

NICE Guidance

FDA on Hydrocephalus

CDC on Hydrocephalus

Hydrocephalus in the news

Blogs onHydrocephalus

Directions to Hospitals Treating Hydrocephalus

Risk calculators and risk factors for Hydrocephalus

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]; Associate Editor-In-Chief: Cafer Zorkun M.D., PhD.,Kalsang Dolma, MBBS

Epidemiology and Demographics

Hydrocephalus affects one in every 500 live births, making it one of the most common birth defects, more common than Down syndrome or deafness. There are an estimated 700,000 children and adults living with hydrocephalus, and it is the leading cause of brain surgery for children in the United States.

Overview

(insert disease state here) is a major health problem affecting _____ patients in the United States and ____ patients worldwide.

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(insert disease state here) is a common health problem affecting _____ patients in the United States and ____ patients worldwide.

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(insert disease state here) is a common health problem that tends to more often affect the ____ (elderly for example) and _____ (men for example).

or

(insert disease state here) is a rare disease (defined as a prevalence of < 50 per 100,000 patients or < 200,000 patients affected in the United States) with a prevalence of ______ per 100,000 patients.

Prevalence

Worldwide, the prevalence of (insert disease state here) ranges from a low of _____ per 100,000 patients, to a high of _____ per 100,000 patients with an average prevalence of _____ per 100,000 patients.

Worldwide, the prevalence of (insert disease state here) is _____ per 100,000 patients.

In developed countries, the prevalence of (insert disease state here) ranges from a low of _____ per 100,000 patients, to a high of _____ per 100,000 patients with an average prevalence of _____ per 100,000 patients.

In developed countries, the prevalence of (insert disease state here) is _____ per 100,000 patients.

In developing countries, the prevalence of (insert disease state here) ranges from a low of _____ per 100,000 patients, to a high of _____ per 100,000 patients with an average prevalence of _____ per 100,000 patients.

In developing countries, the prevalence of (insert disease state here) is _____ per 100,000 patients.

In Africa, the prevalence of (insert disease state here) ranges from a low of _____ per 100,000 patients, to a high of _____ per 100,000 patients with an average prevalence of _____ per 100,000 patients.

In Africa, the prevalence of (insert disease state here) is _____ per 100,000 patients.

Incidence

Worldwide, the incidence of (insert disease state here) ranges from a low of _____ per 100,000 patients, to a high of _____ per 100,000 patients with an average incidence of _____ per 100,000 patients.

Worldwide, the incidence of (insert disease state here) is _____ per 100,000 patients.

In developed countries, the incidence of (insert disease state here) ranges from a low of _____ per 100,000 patients, to a high of _____ per 100,000 patients with an average incidence of _____ per 100,000 patients.

In developed countries, the incidence of (insert disease state here) is _____ per 100,000 patients.

In developing countries, the incidence of (insert disease state here) ranges from a low of _____ per 100,000 patients, to a high of _____ per 100,000 patients with an average incidence of _____ per 100,000 patients.

In developing countries, the incidence of (insert disease state here) is _____ per 100,000 patients.

In Africa, the incidence of (insert disease state here) ranges from a low of _____ per 100,000 patients, to a high of _____ per 100,000 patients with an average incidence of _____ per 100,000 patients.

In Africa, the incidence of (insert disease state here) is _____ per 100,000 patients.

Age

The prevalence of (insert disease state here) increases with age.

Gender

(insert disease state here) is more common in males females. The prevalence of (insert disease state here) among men is ____ per 100,000, while it is _____ per 100,000 among women. The incidence of (insert disease state here) among men is ____ per 100,000, while it is _____ per 100,000 among women.

or

Gender is not associated with an increased risk of (insert disease state here)

Race

Race is not associated with an increased risk of (insert disease state here)

References

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Natural History, Complications and Prognosis

One of the most performed treatments for hydrocephalus, the cerebral shunt, has not changed since it was developed in 1960. The shunt must be implanted through neurosurgery into the patient's brain, a procedure which itself may cause brain damage. An estimated 50% of all shunts fail within two years, requiring further surgery to replace the shunts. In the past 25 years, death rates associated with hydrocephalus have decreased from 54% to 5% and the occurrence of intellectual disability has decreased from 62% to 30%.

Because hydrocephalus injures the brain, thought and behavior may be adversely affected. Learning disabilities are common among those with hydrocephalus, who tend to score better on verbal IQ than on performance IQ, which is thought to reflect the distribution of nerve damage to the brain. However, the severity of hydrocephalus differs considerably between individuals and some are of average or above average intelligence. Someone with hydrocephalus may have motivation and visual problems, problems with coordination, and may be clumsy. They may hit puberty earlier than the average child (see precocious puberty). About one in four develops epilepsy.

Elevated intracranial pressure may result in uncal and/or cerebellar tonsill herniation, with resulting life threatening brain stem compression.

Diagnosis

Symptoms

The triad (Hakim triad) of gait instability, urinary incontinence and dementia is a relatively typical manifestation of the distinct entity normal pressure hydrocephalus (NPH). The triad can easily be remembered as "Wacky, Wet, and Wobbly!"

Symptoms of hydrocephalus due to increased intracranial pressure may include:

Physical Examination

HEENT

Neurologic

Imaging Findings

Because the problem resides inside the head, doctors rely heavily upon computer tomography scanning (CT scans), which may be used frequently to evaluate the condition of the disorder throughout the patient's life. Each CT scan exposes the patient to many times the level of x-ray radiation of a chest x-ray. See CT radiation exposure.

Hydrocephalus

Aqueductal Stenosis

Images courtesy of RadsWiki

Treatment

Hydrocephalus treatment is surgical. It involves the placement of a ventricular catheter (a tube made of silastic), into the cerebral ventricles to bypass the flow obstruction/malfunctioning arachnoidal granulations and drain the excess fluid into other body cavities, from where it can be resorbed. Most shunts drain the fluid into the peritoneal cavity (ventriculo-peritoneal shunt), but alternative sites include the right atrium (ventriculo-atrial shunt), pleural cavity (ventriculo-pleural shunt), and gallbladder. A shunt system can also be placed in the lumbar space of the spine and have the CSF redirected to the peritoneal cavity (LP Shunt). An alternative treatment for obstructive hydrocephalus in selected patients is the endoscopic third ventriculostomy (ETV), whereby a surgically created opening in the floor of the third ventricle allows the CSF to flow directly to the basal cisterns, thereby shortcutting any obstruction, as in aqueductal stenosis. This may or may not be appropriate based on individual anatomy. Internal hydrocephalus can be successfully treated by placing a drainage tube (shunt) between the brain ventricles and abdominal cavity to eliminate the high internal pressures. There is some risk of infection being introduced into the brain through these shunts, however, and the shunts must be replaced as the person grows. A subarachnoid hemorrhage may block the return of CSF to the circulation. If CSF accumulates in the subarachnoid space, the condition is called external hydrocephalus. In this condition, pressure is applied to the brain externally, compressing neural tissues and causing brain damage. Thus resulting to a much further damage of the brain tissue and leading to necrotization

Shunt complications

Examples of possible complications include shunt malfunction, shunt failure, and shunt infection. Although a shunt generally works well, it may stop working if it disconnects, becomes blocked, or it is outgrown. If this happens the cerebrospinal fluid will begin to accumulate again and a number of physical symptoms will develop, some extremely serious, like seizures. The shunt failure rate is also relatively high and it is not uncommon for patients to have multiple shunt revisions within their lifetime.

The diagnosis of cerebrospinal fluid buildup is complex and requires specialist expertise.

Another complication can occur when CSF drains more rapidly than it is produced by the choroid plexus, causing symptoms - listlessness, severe headaches, irritability, light sensitivity, sound sensitivity, nausea, vomiting, dizziness, vertigo, migraines, seizures, a change in personality, weakness in the arms or legs, excessive head growth (seen infants, children under age 2), strabismus, and double vision - to appear when the patient is vertical. If the patient lies down, the symptoms usually vanish in a short amount of time. A CT scan may or may not show any change in ventricle size, particularly if the patient has a history of slit-like ventricles. Difficulty in diagnosing overdrainage can make treatment of this complication particularly frustrating for patients and their families.

Resistance to traditional analgesic pharmacological therapy may also be sign of shunt overdrainage or failure. Diagnosis of the particular complication usually depends on when the symptoms appear.

Case Studies

One interesting case involving a person with past hydrocephalus was a 44-year old French man, whose brain had been reduced to little more than a thin sheet of actual brain tissue, due to the buildup of fluid in his skull. The man, who had a shunt inserted into his head to drain away fluid (which was removed when he was 14), went to a hospital after he had been experiencing mild weakness in his left leg.

DWS: All of the black in the middle is water and the brain matter is the rim of white along the outside of the skull. This is a screen shot from a Fox News report.

In July of 2007, Fox News quoted Dr. Lionel Feuillet of Hopital de la Timone in Marseille as saying: "The images were most unusual... the brain was virtually absent."[1] When doctors learned of the man's medical history, they did a computed tomography (CT) scan and another type of scan called magnetic resonance imaging (MRI), and were astonished to see "massive enlargement" of the lateral ventricles in the skull. Intelligence tests showed the man had an IQ of 75, below the average score of 100 but not considered mentally retarded or disabled, either.

Remarkably, the man was a married father of two children, and worked as a civil servant, leading a normal life, despite having little brain tissue. "What I find amazing to this day is how the brain can deal with something which you think should not be compatible with life," commented Dr. Max Muenke, a pediatric brain defect specialist at the National Human Genome Research Institute. "If something happens very slowly over quite some time, maybe over decades, the different parts of the brain take up functions that would normally be done by the part that is pushed to the side."[2]

See also

References

External links

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