Hydrocephalus
also known as "water in the brain," is a medical condition in which
there is an abnormal accumulation of cerebrospinal fluid (CSF) in the
ventricles, or cavities, of the brain. This may cause increased
intracranial pressure inside the skull and progressive enlargement of
the head, convulsion, tunnel vision, and mental disability.
Hydrocephalus can also cause death. The clinical presentation of
hydrocephalus varies with chronicity. Acute dilatation of the
ventricular system is more likely to manifest with the nonspecific signs
and symptoms of increased intracranial pressure. By contrast chronic
dilatation (especially in the elderly population) may have a more
insidious onset presenting, for instance, with Hakim's triad (Adams
triad).
Symptoms
of increased intracranial pressure may include headaches, vomiting,
nausea, papilledema, sleepiness or coma. Elevated intracranial pressure
may result in uncal and/or cerebellar tonsill herniation, with resulting
life threatening brain stem compression.
In
infants with hydrocephalus, CSF builds up in the central nervous system,
causing the fontanelle (soft spot) to bulge and the head to be larger
than expected. Early symptoms may also include:
- Eyes that appear to gaze downward (Sundowning)
- Irritability
- Seizures
- Separated sutures
- Sleepiness
- Vomiting
Symptoms that may occur in older children can include
- Brief, shrill, high-pitched cry
- Changes in personality, memory, or the ability to reason or think
- Changes in facial appearance and eye spacing
- Crossed eyes or uncontrolled eye movements
- Difficulty feeding
- Excessive sleepiness
- Headache
- Irritability, poor temper control
- Loss of bladder control (urinary incontinence)
- Loss of coordination and trouble walking
- Muscle spasticity (spasm)
- Slow growth (child 0–5 years)
- Slow or restricted movement
- Vomiting
Signs and Tests
When
a health care provider taps fingertips on the skull, there may be
abnormal sounds that indicated thinning and separation of skull bones.
Scalp veins may appear stretched or enlarged.
Part
or the entire head may be larger than normal. Enlargement is most
commonly seen in the front part of the head. Head circumference
measurements, repeated over time, may show that the head is getting
bigger.
The
eyes may look "sunken in." The white part of the eye may appear above
the colored part of the eye, given the eyes a "setting-sun" appearance.
Reflexes may be abnormal.
A head CT scan is one of the best tests for identifying hydrocephalus. Other tests that may be done include:
- Arteriography
- Brain scan using radioisotopes
- Cranial ultrasound (an ultrasound of the brain)
- Lumbar puncture and examination of the cerebrospinal fluid (rarely done)
- Skull x-rays
Effects
Because hydrocephalus can injure the brain, thought and behavior may be
adversely affected. Learning disabilities including short-term memory
loss 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 can differ considerably between individuals and some are
of average or above-average intelligence. Someone with hydrocephalus may
have motion and visual problems, problems with coordination, or may be
clumsy. They may reach puberty earlier than the average child (see
precocious puberty). About one in four develops epilepsy.
Treatment
Hydrocephalus treatment is surgical, generally creating various types
of cerebral shunts. 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
(Lumbar-peritoneal 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. This is the treatment.
Shunt complications
Examples
of possible complications include shunt malfunction, shunt failure, and
shunt infection, along with infection of the shunt tract following
surgery (the most common reason for shunt failure is infection of the
shunt tract). Although a shunt generally works well, it may stop working
if it disconnects, becomes blocked (clogged), infected, or it is
outgrown. If this happens the cerebrospinal fluid will begin to
accumulate again and a number of physical symptoms will develop
(headaches, nausea, vomiting, photophobia/light sensitivity), some
extremely serious, like seizures. The shunt failure rate is also
relatively high (of the 40,000 surgeries performed annually to treat
hydrocephalus, only 30% are a patient's first surgery) 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.
What are the different types of hydrocephalus?
Hydrocephalus
may be congenital or acquired. Congenital hydrocephalus is present at
birth and may be caused by either events or influences that occur during
fetal development, or genetic abnormalities. Acquired hydrocephalus
develops at the time of birth or at some point afterward. This type of
hydrocephalus can affect individuals of all ages and may be caused by
injury or disease.
Hydrocephalus
may also be communicating or non-communicating. Communicating
hydrocephalus occurs when the flow of CSF is blocked after it exits the
ventricles. This form is called communicating because the CSF can still
flow between the ventricles, which remain open. Non-communicating
hydrocephalus - also called "obstructive" hydrocephalus - occurs when
the flow of CSF is blocked along one or more of the narrow passages
connecting the ventricles. One of the most common causes of
hydrocephalus is "aqueductal stenosis." In this case, hydrocephalus
results from a narrowing of the aqueduct of Sylvius, a small passage
between the third and fourth ventricles in the middle of the brain.
There
are two other forms of hydrocephalus which do not fit exactly into the
categories mentioned above and primarily affect adults: hydrocephalus
ex-vacuo and normal pressure hydrocephalus.
Hydrocephalus ex-vacuo occurs when stroke or traumatic injury cause
damage to the brain. In these cases, brain tissue may actually shrink.
Normal pressure hydrocephalus can happen to people at any age, but it is
most common among the elderly. It may result from a subarachnoid
hemorrhage, head trauma, infection, tumor, or complications of surgery.
However, many people develop normal pressure hydrocephalus even when
none of these factors are present for reasons that are unknown.
Who gets this hydrocephalus?
The
number of people who develop hydrocephalus or who are currently living
with it is difficult to establish since there is no national registry or
database of people with the condition. However, experts estimate that
hydrocephalus affects approximately 1 in every 500 children.
What causes hydrocephalus?
The
causes of hydrocephalus are still not well understood. Hydrocephalus may
result from inherited genetic abnormalities (such as the genetic defect
that causes aqueductal stenosis) or developmental disorders (such as
those associated with neural tube defects including spina bifida and
encephalocele). Other possible causes include complications of premature
birth such as intraventricular hemorrhage, diseases such as meningitis,
tumors, traumatic head injury, or subarachnoid hemorrhage, which block
the exit of CSF from the ventricles to the cisterns or eliminate the
passageway for CSF into the cisterns.
Causes, incidence, and risk factors
Hydrocephalus
is due to a problem with the flow of cerebrospinal fluid (CSF), the
liquid that surrounds the brain and spinal cord.
CSF
moves through pathways of the brain called ventricles. It also flows
around the outside of the brain and through the spinal canal.
Higher-than-normal amounts of CSF can occur in the brain if the flow or
absorption of CSF is blocked, or if too much CSF is produced. The build
up of fluid puts pressure on the brain, pushing the brain up against the
skull and damaging or destroying brain tissues.
The
symptoms depend on the cause of the blockage, the person's age, and how
much brain tissue has been damaged by the swelling. In infants with
hydrocephalus, CSF fluid builds up in the central nervous system,
causing the fontanelle (soft spot) to bulge and the head to expand.
Myelomeningocele, a disorder involving incomplete closure of the spinal
column, is strongly associated with hydrocephalus.
In young children, hydrocephalus may also be associated with the following conditions:
- Infections caught before birth
- Infections that affect the central nervous system (such as meningitis or encephalitis)
- Injury before, during, or after childbirth, including subarachnoid hemorrhage
- Congenital defects
- Tumors of the central nervous system
In older children, risks for hydrocephalus include:
- History of congenital or developmental defects
- Lesions or tumors of the brain or spinal cord
- Central nervous system infections
- Bleeding anywhere in the brain
- Injury
Hydrocephalus most often occurs in children, but may also occur in adults and the elderly.
Symptoms
Early symptoms in infants:
- Enlarged head (increased head circumference)
- Bulging fontanelles (soft spots of the head) with or without enlargement of the head size
- Separated sutures
- Vomiting
Symptoms of continued hydrocephalus:
- Irritability, poor temper control
- Muscle spasticity (spasm)
Symptoms that occur later in the disease:
- Decreased mental function
- Delayed development
- Slow or restricted movement
- Difficulty feeding
- Lethargy, excessive sleepiness
- Urinary incontinence (loss of control over bladder)
- Brief, shrill, high-pitched cry
- Slow growth (child 0-5 years)
Symptoms in older babies and children:
- Headache
- Vomiting
- Vision changes
- Crossed eyes
- Uncontrolled eye movements
- Loss of coordination
- Poor gait (walking pattern)
- Mental aberrations (such as confusion or psychosis)
- Changes in facial appearance and eye spacing
Expectations (prognosis)
Untreated
hydrocephalus has a 50-60% death rate, with the survivors having
varying degrees of intellectual, physical, and neurologic disabilities.
The
outlook for treated hydrocephalus depends on the cause. Hydrocephalus
that is caused by disorders not associated with infection has the best
outlook. Persons with hydrocephalus caused by tumors usually do very
poorly.
More
than 80% of children with hydrocephalus that survive for 1 year will
have a fairly normal life span. Approximately a third will have normal
intellectual function, but neurological difficulties may persist.
Hydrocephalus- An Introduction:
The
term hydrocephalus is derived from the Greek words "hydro" meaning water
and "cephalus" meaning head. As the name suggests, it is a condition in
which excessive accumulation of fluid in the brain. The excess fluid
causes the spaces in the brain, called ventricles, to expand which
results in potentially harmful pressure on the tender tissues of the
brain. Hydrocephalus can be fatal if not treated on time. Once known as
"water on the brain," the "water" is actually cerebrospinal fluid (CSF),
a clear fluid surrounding the brain and spinal cord. Hydrocephalus
affects a wide range of people from infants and older children to young,
middle-aged and older adults.
Symptoms of Hydrocephalus:
The signs and symptoms of hydrocephalus vary by age group and disease progression.
In infants, common signs and symptoms of hydrocephalus include:
- An unusually large head
- A rapid increase in the size of the head
- A bulging "soft spot" on the top of the head
- Vomiting
- Sleepiness
- Irritability
- Seizures
- Eyes fixed downward (sun setting of the eyes)
- Developmental delay
In older children and adults, common signs and symptoms of hydrocephalus include:
- Headache followed by vomiting
- Nausea
- Blurred or double vision
- Eyes fixed downward (sun setting of the eyes)
- Problems with balance, coordination or gait
- Sluggishness or lack of energy
- Slowing or regression of development
- Memory loss
- Confusion
- Urinary incontinence
- Irritability
- Changes in personality
- Impaired performance in school or work
Types of Hydrocephalus
- Congenital Hydrocephalus - Congenital hydrocephalus can cause by genetic factors or it can cause by other environmental influences during fetal development. This type of hydrocephalus can occur before during or soon after birth.
- Acquired Hydrocephalus - This type of hydrocephalus occurs later in the life by some injuries or disease. It can affect people of all ages.
- Communicating Hydrocephalus - In this state cerebrospinal fluid (CSF) cannot circulate properly. Although it flows normally between the four ventricles but is blocked after it exits the ventricles.
- Non-Communicating Hydrocephalus - This is also called Obstructive Hydrocephalus that develops when the flow of CSF is blocked along one or more of the narrow pathways connecting the ventricles and CSF cannot circulate properly.
- Normal Pressure Hydrocephalus (NPH) - There are two types of NPH –
- Diopathic NPH - In Idiopathic NPH as name suggests it happens because of unknown cause.
- PH of Known Cause - Other NPH can happen because of head trauma infections tumour and surgery.
Hydrocephalus
is sometimes present at birth (Congenital hydrocephalus), although it
may develop later at any age (Acquired hydrocephalus).
Causes of Hydrocephalus:
The
causes of hydrocephalus are not entirely clear to scientists. The
condition may result from genetic inheritance (this is called aqueductal
stenosis -- the narrowing of a specific pathway in the brain) or
developmental disorders, such as those associated with neural tube
defects like spinal bifida and encephalocele.
Other possible hydrocephalus causes include:
- Complications associated with premature birth, such as intraventricular hemorrhage
- Conditions such as meningitis, tumors, or traumatic head injury
- Subarachnoid hemorrhage blocking the exit from the ventricles to the cisterns and eliminating the cisterns themselves.
Diagnosis of Hydrocephalus
To
diagnose Hydrocephalus, a physician considers the patient's medical
history, symptoms, results of the physical exam, and certain imaging
tests or procedures. Tests used to diagnose hydrocephalus can include:
- Ultrasound
- Computed tomography (CT scan)
- Magnetic resonance imaging (MRI)
- Pressure-monitoring techniques.
The
tests that the doctor recommends are based on the patient's age,
clinical presentation, and the presence of known or suspected problems
of the brain or spinal cord.
Hydrocephalus Treatment
The goal of treatment is to reduce or prevent brain damage by improving the flow of CSF.
Surgery
is the main treatment. The blockage may be surgically removed, if
possible. If the blockage cannot be removed, a shunt may be placed
within the brain to allow CSF to flow around the blocked area
Types of Hydrocephalus Surgery
- Shunt Placement: The most common treatment for hydrocephalus is the surgical insertion of a drainage system, called a shunt. It consists of a long flexible tube with a valve that keeps fluid from the brain flowing in the right direction and at the proper rate. One end of the tubing is usually placed in one of the brain's ventricles. The tubing is then tunneled under the skin to another part of the body where the excess cerebrospinal fluid can be more easily absorbed — such as the abdomen or a chamber in the heart. People who have hydrocephalus usually need a shunt system for the rest of their lives, so additional surgeries may be needed to insert longer tubing to match a child's growth. Revisions to the shunt also may be needed if the tubing becomes blocked or infected.
- Ventriculostomy: This surgical procedure is sometimes used when there is an obstruction of flow between ventricles. In the procedure, your surgeon makes a hole in the bottom of one of the ventricles, to allow the cerebrospinal fluid to flow toward the base of the brain, where normal absorption occurs.
- Endoscopic Third Ventriculostomy: In some cases of adult hydrocephalus we are able to determine that there is a CSF blockage that can be potentially bypassed using an endoscopic surgery. In this type of shunt surgery, an endoscopic camera and microinstruments are manipulated through a minimally invasive, computer guided approach into the ventricle. A small hole is made in the floor of the third ventricle, allowing CSF to flow out of the brain. When successful, this type of surgery corrects the hydrocephalus without requiring a shunt implantation.
Benefits of Hydrocephalus Surgery and Radiotherapy
The
success of shunts in treatment of hydrocephalus varies from person to
person; some people recover almost completely after treatment and have a
good quality of life. Early diagnosis and treatment improve the chance
of a good recovery. In the last 50 years, there have been incremental
advancements in the diagnosis and treatment of hydrocephalus, however,
often repeated neurosurgical operations are necessary to treat
hydrocephalus. A shunt is designed to control the amount of flow and
pressure of CSF out of the brain ventricles and this surgery has shown
great results. Despite the problems associated with shunts, at present
they are still the best option for most children. Promising new
technology includes shunts with valves that can be externally programmed
postoperatively and a procedure that uses an endoscope to visualize the
blockage and open it, making a shunt unnecessary. Improvements reported
by the patient and the patient’s family, shows good results of
treatment of Hydrocephalus. Most children with hydrocephalus that
survive for 1 year will have a fairly normal life span. Approximately a
third will have normal intellectual function, but neurological
difficulties may persist.
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