More on Hydrocephalus

Common Causes of Hydrocephalus

Although rare, hydrocephalus can be inherited genetically or may be associated with developmental disorders, including spina bifida (congenital defect of the spine) and encephalocele (hernia of the brain). Other causes can include bleeding within the brain, brain tumors, head injuries, complications of premature birth such as hemorrhage, or diseases such as meningitis or other infections. In some cases, normal flow of CSF within the brain is blocked, resulting in fluid build-up.

Symptoms of hydrocephalus vary greatly from person to person. According to the Hydrocephalus Association, some of the most common symptoms are listed below as a reference.

Symptoms of Hydrocephalus in infants Abnormal enlargement of the head; soft spot (fontanel) is tense and bulging; scalp can appear thin; bones separated in baby's head; prominent scalp veins; vomiting; drowsiness; irritability; downward deviation of baby's eyes; seizures; or poor appetite.

Symptoms of Hydrocephalus in toddlers/children
Abnormal enlargement of baby's head; headache; nausea; vomiting; fever; blurred or double vision; unstable balance; irritability; sleepiness; delayed progress in walking or talking; poor coordination; change in personality; inability to concentrate; loss of sensory motor functions; seizures; or poor appetite. Older children may experience difficulty in remaining awake or waking up.

Symptoms of Hydrocephalus in young and middle-aged adults
Headache; difficulty in remaining awake or waking up; loss of coordination or balance; bladder control problems; impaired vision and cognitive skills that may affect job performance and personal skills.

Symptoms of Hydrocephalus in older adults
Loss of coordination or balance; shuffling gait, memory loss; headache; or bladder control problems. Hydrocephalus is often categorized for age groups as either congenital or normal pressure hydrocephalus. Congenital hydrocephalus refers to conditions that are caused by conditions existing at birth. Primary symptoms include headache, nausea, vomiting and drowsiness. Normal pressure hydrocephalus (NPH) is the accumulation of cerebrospinal fluid that causes the ventricles in the brain to become enlarged, with little or no increase in pressure. Adult-onset NPH mainly occurs in adults age 60 and older. Patients with NPH often get misdiagnosed with Alzheimer's disease or dementia, as some of the symptoms mimic these two conditions.

Diagnosing Hydrocephalus

Before your doctor can recommend a course of treatment, he or she will:

• Review your medical history, and perform a physical examination
• Perform a complete neurological examination including diagnostic testing if needed
• Ask specific questions to determine if symptoms are caused by hydrocephalus

The neurological examination will also help to determine the severity of your condition. Further tests such as an ultrasound (if the patient is an infant), computed tomography (CT or CAT scan), or magnetic resonance imaging (MRI) may be ordered. The tests may reveal useful information about the severity of the condition and its likely cause.

When Surgery is Necessary

Hydrocephalus can be treated in a variety of ways. The problem area may be treated directly (by removing the cause of CSF obstruction), or indirectly (by diverting the fluid to somewhere else; typically to another body cavity). Indirect treatment is performed by implanting a device known as a shunt to divert the excess CSF away from the brain. The body cavity in which the CSF is diverted is usually the peritoneal cavity (the area surrounding the abdominal organs).

In some cases, two procedures are performed, one to divert the CSF, and another at a later stage to remove the cause of obstruction (e.g., a brain tumor). Once inserted, the shunt system usually remains in place for the duration of a patient's life (although additional operations to revise the shunt system are sometimes needed). The shunt system continuously performs its function of diverting the CSF away from the brain, thereby keeping the intracranial pressure within normal limits.

An alternative operation called endoscopic third ventriculostomy utilizes a tiny camera to look inside the ventricles, allowing the surgeon to create a new pathway through which CSF can flow.

Recovery

Your neurological function will be evaluated post surgery. If any neurological problems persist, rehabilitation may be required to further your improvement. However, recovery may be limited by the extent of the damage already caused by the hydrocephalus and by your brain's ability to heal.

Because hydrocephalus is an ongoing condition, long-term follow-up by a doctor is required. Follow-up diagnostic tests including CT scans, MRIs and x-rays, are helpful in determining if the shunt is working properly. Do not hesitate to contact your physician if you experience any of the following postoperative symptoms:

• Redness, tenderness, pain or swelling of the skin along the length of the tube or incision
• Irritability or drowsiness
• Nausea, vomiting, headache or double vision
• Fever
• Abdominal pain
• Return of preoperative neurological symptoms

Prognosis

The prognosis for hydrocephalus depends on the cause, the extent of symptoms, and the timeliness of diagnosis and treatment. Some patients show a dramatic improvement with treatment while others do not. In some instances of NPH, dementia can be reversed by shunt placement. Other symptoms such as headaches may disappear almost immediately if the symptoms are related to elevated pressure.

In general, the earlier hydrocephalus is diagnosed, the better the chance for successful treatment. The longer the symptoms have been present, the less likely it is that treatment will be successful. Unfortunately, there is no way to accurately predict how successful surgery will be for each individual. Some patients will improve dramatically while others will reach a plateau or decline after a few months.

Shunt malfunction or failure may occur. The valve can become clogged or the pressure in the shunt may not match the needs of the patient, requiring additional surgery. In the event of an infection, antibiotic therapy may be needed. A shunt malfunction may be indicated by headaches, vision problems, irritability, fatigue, personality change, loss of coordination, difficulty in waking up or staying awake, a return of walking difficulties, mild dementia or incontinence. Fortunately, most complications can be dealt with successfully.

Source: NeurosurgeryToday.Org

What is shunt?

A shunt is a surgically implanted device that diverts cerebrospinal fluid (CSF) in a controlled manner from fluid compartments in the brain or near the spine to another part of the body, such as the abdomen or the heart. For nearly half a century, hydrocephalus has been treated by surgical placement of a (CSF) diverting shunt.
Shunts typically consist of three components:
1. An inflow (proximal) catheter, which drains CSF from the ventricles or the lumbar subarachnoid space, to a valve;
2. A valve mechanism, which regulates differential pressure or controls flow through the shunt tubing;
3. An outflow (distal) catheter, which directs CSF from the valve to the peritoneum, heart or other suitable drainage site.

Other shunt components may include reservoirs and/or antechambers for sampling or injecting medications; or dyes, on/off devices, anti-siphon or other flow-compensating devices, auxiliary catheters, etc., to modify performance or adapt the basic system to the patient’s specialized needs. In selected cases (such as when extraventricular fluid collections are drained), a shunt may not contain a valve.

Hydrocephalus

From Hydrocephalus Association:

Hydrocephalus comes from Greek words: hydro means water, cephalus means head. Hydrocephalus is an abnormal accumulation of fluid—cerebrospinal fluid, or CSF—within cavities called ventricles inside the brain. This condition may occur at any age. CSF is produced in the ventricles, circulates through the ventricular system in the brain and is absorbed into the bloodstream. CSF is in constant circulation and has many functions. It surrounds the brain and spinal cord and acts as a protective cushion against injury. CSF contains nutrients and proteins that are needed for the nourishment and normal function of the brain. It carries waste products away from surrounding tissues. Hydrocephalus occurs when there is an imbalance between the amount of CSF that is produced and the rate at which it is absorbed. As the CSF builds up, it causes the ventricles to enlarge and the pressure inside the head to increase.

While there's no known cure for hydrocephalus, the future holds promise. Recent research gives great hope that we may one day find a cure. In the meantime, gradual advances in technology as well as diagnostic and treatment protocols are helping more and more people with hydrocephalus to lead full and active lives.

Although hydrocephalus (hi-dro-sef-a-lus) is not yet a household word, it is NOT uncommon and you are not alone. Over a million people of all ages currently live with hydrocephalus in the United States, though it occurs all over the world. Hydrocephalus knows no socioeconomic, gender or ethnic boundaries. Of every 1,000 babies born in this country, one or two will have hydrocephalus. It is the most common reason for brain surgery in children, but it can also be diagnosed in any decade of life. Experts estimate that hundreds of thousands of older adults have normal pressure hydrocephalus (NPH), which often goes undetected or is misdiagnosed as untreatable dementia, Alzheimer’s or Parkinson’s.

How is Hydrocephalus treated?
There is no known way to prevent or cure hydrocephalus. To date, the most common treatment is surgical insertion of a shunt. A shunt is a flexible tube placed into the ventricular system of the brain which diverts the flow of CSF into another region of the body, most often the abdominal cavity, where it can be absorbed. A valve within the shunt regulates the flow of CSF.

The shunt procedure is performed by a neurosurgeon. Shunts can become clogged or malfunction and surgical revisions are often required. Endoscopic Third Ventriculostomy (ETV) is a procedure in which a small perforation is made in the thinned floor of the third ventricle, allowing movement of CSF out of the blocked ventricular system. It is a promising technique that can treat hydrocephalus without a shunt. ETV is not a cure for hydrocephalus and it is not an appropriate choice of treatment for many types of hydrocephalus. Each case must be evaluated individually by experienced medical professionals.


The association provides a family book about Hydrocephalus. You can get it here.


Useful Links

Hydrocephalus Association

Pediatric Neurosurgery - Hydrocephalus

Hydrocephalus Fact Sheets