Hydrocephalus is a build-up of fluid inside the skull, which causes brain swelling. Hydrocephalus means "water on the brain."
Alternative NamesWater on the brain
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
Hydrocephalus most often occurs in children, but may also occur in adults and the elderly.
See also: Normal pressure hydrocephalus (NPH)
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
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:
- Vision changes
- Uncontrolled eye movements
- Loss of coordination
- Poor gait (walking pattern)
- Mental aberrations (such as confusion or psychosis)
- Changes in facial appearance and eye spacing
Exams 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 (dilated).
Part or the entire head may be larger than normal. Enlarge 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 (the sclera) may appear above the colored part of the eye (the iris), given the eyes a "setting-sun" appearance. Reflexes may be abnormal.
The following tests may be performed:
This disease may also alter the results of a RHISA scan.
- Transillumination of the head (shining a light against the head)
- Head CT scan (one of the best tests for identifying hydrocephalus)
- Spinal tap and examination of the CSF (rarely done)
- Skull x-rays
- Brain scan using radioisotopes
- Echoencephalogram, an ultrasound of the brain
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.
Alternatively, a shunt may be placed outside the brain, in an area such as the right chamber of the heart or the abdominal peritoneum.
Removing or burning away (cauterizing) the parts of the brain that produce CSF may reduce CSF production.
An important complication of shunt placement is blockage. Symptoms of such a blockage include headache and vomiting. Surgeons may be able to help the shunt open without having to replace it.
Antibiotics are given if there are sign of infection. Severe infections may require the shunt to be removed.
Another option is endoscopic third ventriculostomy (ETV), which relieves pressure without replacing the shunt.
Follow-up examinations generally continue throughout the child's life. These are done to check the child's developmental level and to treat any intellectual, neurologic, or physical problems.
Visiting nurses, social services, support groups, and local agencies can provide emotional support and assist with the care of a child with hydrocephalus who has significant brain damage.
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.
- Problems with the shunt, such as kinking, blockage, or tube separation
- Intellectual impairment
- Neurologic damage (decrease in movement, sensation, function)
- Physical disabilities
- Complications of surgery
When to Contact a Medical Professional
Any symptoms of this disorder should be brought to the health care provider's attention immediately. Call if a child's head is getting larger or there is a change in the appearance of the face, head, or eyes. Also call if the condition deteriorates to the point that the child cannot be cared for in the home.
Go to the emergency room or call 911 if emergency symptoms occur, including difficulties with sucking/feeding, high-pitched cry, fever, lethargy or drowsiness, stiff neck (unwillingness to bend or move the neck or head), problems with breathing, severe headache, seizures, irregular heartbeat, or no heartbeat.
Protect the head of an infant or child from injury. Prompt treatment of infections such as meningitis and other disorders associated with hydrocephalus may reduce the risk of developing the disorder.