Hydrocephalus

Definition:
Hydrocephalus, also known as “water on 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  and is an excessive amount of fluid that surrounds the brain and spinal cord called cerebrospinal fluid (or CSF).

This may cause increased intracranial pressure inside the skull and progressive enlargement of the head, convulsion, tunnel vision, and mental disability.The excess fluid can compress surrounding, fragile brain tissue, causing brain damage. Left untreated, hydrocephalus can be fatal. Hydrocephalus can also cause death.
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Hydrocephalus is sometimes present at birth, although it may develop later. About 1 out of 500 children is born with the disorder. The outlook if you have hydrocephalus depends on how quickly the condition is diagnosed and whether any underlying disorders are present.

The name derives from the Greek words  hudro means “water”, and kephalos means “head”.

Symptoms:
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 the Hakim 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. For details on other manifestations of increased intracranial pressure:

The triad (Hakim triad) of gait instability, urinary incontinence and dementia is a relatively typical manifestation of the distinct entity normal pressure hydrocephalus (NPH). Focal neurological deficits may also occur, such as abducens nerve palsy and vertical gaze palsy (Parinaud syndrome due to compression of the quadrigeminal plate, where the neural centers coordinating the conjugated vertical eye movement are located).

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 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

Hydrocephalus produces different combinations of these signs and symptoms, depending on its cause, which also varies by age. For example, a condition known as normal pressure hydrocephalus, which mainly affects older people, typically starts with difficulty walking. Urinary incontinence often develops, along with a type of dementia marked by slowness of thinking and information processing.

Causes:
In hydrocephalus, CSF builds up and puts pressure on the brain, squashing the delicate tissues and causing the chambers or ventricles within the brain to swell. Without treatment damage or destruction of the brain tissues may occur.

Our brain is the consistency of gelatin, and it floats in a bath of cerebrospinal fluid. This fluid also fills large open structures, called ventricles, which lie deep inside your brain. The fluid-filled ventricles help keep the brain buoyant and cushioned.

Cerebrospinal fluid flows through the ventricles by way of interconnecting channels. The fluid eventually flows into spaces around the brain, where it’s absorbed into your bloodstream.

Keeping the production, flow and absorption of cerebrospinal fluid in balance is important to maintaining normal pressure inside your skull. Hydrocephalus results when the flow of cerebrospinal fluid is disrupted — for example, when a channel between ventricles becomes narrowed — or when your body doesn’t properly absorb this fluid.

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There are two types of hydrocephalus:
Non-communicating hydrocephalus – the flow of CSF through the brain is blocked.
Communicating hydrocephalus – either too much CSF is produced, or it isn’t reabsorbed back into the tissues as it should be, so volume increases.

There are many causes of hydrocephalus and some of them are:
•Bleeding in the brain (for example if a baby is born very preterm)
•Congenital malformations (structural abnormalities that are present from birth)
•After infections in the brain
•Brain tumours
•Abnormalities in the blood vessels in the brain

Defective absorption of cerebrospinal fluid causes normal pressure hydrocephalus, seen most often in older people. In normal pressure hydrocephalus, excess fluid enlarges the ventricles but does not increase pressure on the brain. Normal pressure hydrocephalus may be the result of injury or illness, but in many cases the cause is unknown.

Risk Factors:
Premature infants have an increased risk of severe bleeding within the ventricles of the brain (intraventricular hemorrhage), which can lead to hydrocephalus.

Certain problems during pregnancy may increase an infant’s risk of developing hydrocephalus, including:
*An infection within the uterus
*Problems in fetal development, such as incomplete closure of the spinal column

Congenital or developmental defects not apparent at birth also can increase older children’s risk of hydrocephalus.

Other factors that increase your risk of hydrocephalus include:
*Lesions or tumors of the brain or spinal cord
*Central nervous system infections
*Bleeding in the brain
*Severe head injury

Complications:
The severity of hydrocephalus depends on the age at which the condition develops and the course it follows. If the condition is well advanced at birth, major brain damage and physical disabilities are likely. In less severe cases, with proper treatment, it’s possible to have a nearly normal life span and intelligence.

Congenital:
The cranial bones fuse by the end of the third year of life. For head enlargement to occur, hydrocephalus must occur before then. The causes are usually genetic but can also be acquired and usually occur within the first few months of life, which include 1) intraventricular matrix hemorrhages in premature infants, 2) infections, 3) type II Arnold-Chiari malformation, 4) aqueduct atresia and stenosis, and 5) Dandy-Walker malformation.

In newborns and toddlers with hydrocephalus, the head circumference is enlarged rapidly and soon surpasses the 97th percentile. Since the skull bones have not yet firmly joined together, bulging, firm anterior and posterior fontanelles may be present even when the patient is in an upright position.

The infant exhibits fretfulness, poor feeding, and frequent vomiting. As the hydrocephalus progresses, torpor sets in, and the infant shows lack of interest in his surroundings. Later on, the upper eyelids become retracted and the eyes are turned downwards (due to hydrocephalic pressure on the mesencephalic tegmentum and paralysis of upward gaze). Movements become weak and the arms may become tremulous. Papilledema is absent but there may be reduction of vision. The head becomes so enlarged that the child may eventually be bedridden.

About 80-90% of fetuses or newborn infants with spina bifida—often associated with meningocele or myelomeningocele—develop hydrocephalus.

Acquired:
This condition is acquired as a consequence of CNS infections, meningitis, brain tumors, head trauma, intracranial hemorrhage (subarachnoid or intraparenchymal) and is usually extremely painful.

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.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://www.bbc.co.uk/health/physical_health/conditions/hydrocephalus2.shtml
http://www.mayoclinic.com/health/hydrocephalus/DS00393
http://en.wikipedia.org/wiki/Hydrocephalus

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