Hydrocephalus an abnormal increase in the amount of cerebrospinal fluid within the cranial cavity
that is accompanied by expansion of the cerebral ventricles, enlargement of the skull and atrophy of
the brain. Brain cells do not regenerate or replace themselves, thus any damage brought by the Hydrocephalus is permanent.

Hydrocephalus can lead to physical incapacity, mental retardation, blindness and ultimately death. It is estimated that one out of 200 babies born are affected as well as a large number of adults last year alone we received thousands of phone calls for help, we sent out thousands of packets to parents and friends that know someone who has been afflicted with this dreaded birth defect. We also connect the parents of children afflicted with Hydrocephalus to qualified physicians from all over the world.

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What Types of Hydrocephalus Are There?

Although Hydrocephalus (“water on the brain”) is a brain disease that affects the flow of cerebrospinal fluid. There are multiple causes and types of Hydrocephalus here we present a simplified explanation
Causes: Congenital Hydrocephalus is present at birth, although it may not be detected until later in life. It forms because of abnormal development of the brain and surrounding structures. The exact cause is usually unknown, but contributing factors may include a genetic predisposition and certain infections during pregnancy.
Acquired hydrocephalus results from injuries or illnesses that occur at birth or later. It can develop as a complication of infections in the brain and spinal column (meningitis), bleeding of blood vessels in the brain, severe head injury, or brain tumors or cysts. Hydrocephalus also can occur when there is no known injury or illness to cause it.
Types: Obstructive (non-communicating) hydrocephalus results from an obstruction within the system of ventricles in the brain. The obstruction prevents the cerebrospinal fluid from flowing (or “communicating”) with the area that surrounds the brain and spinal cord. Obstructions could be present at birth or occur later. Example: Aqueductal Stenosis.
Non-obstructive (communicating) hydrocephalus results from problems with the production or absorption of cerebrospinal fluid. Also this type can be present at birth or can occur later.
Normal Pressure Hydrocephalus is an acquired type of communicating hydrocephalus in which the ventricles are enlarged but not under high pressure. This is seen mainly in older adults. It is primarily the result of injury or illness, but in some cases the cause is unknown.

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 Prenatal hydrocephalus describes hydrocephalus that is diagnosed in utero (before birth).
Hydrocephalus can be detected in a fetus as early as the latter part of the first trimester of pregnancy. Around 20 to 24 weeks of gestation, abnormal dilation of the fetus's ventricles is more clearly detectable.
Prenatal ultrasound, performed by a radiologist or perinatologist skilled in obstetric ultrasound, is highly reliable and accurate. It will establish that there is an abnormal cerebrospinal fluid (CSF) collection but it may not show the precise site of flow obstruction. The more important question is what caused the hydrocephalus and to what degree are there other malformations within the central nervous system. In general, early (first trimester) development of significant hydrocephalus can be a bad prognostic sign for infant mortality and developmental progress. In some cases, mild ventricular dilation identified by ultrasound has resolved itself by the third trimester.
Prenatal hydrocephalus is generally not treated until after birth, when a shunt may be inserted into the baby's brain to divert CSF fluid. While some neurosurgeons have attempted in utero placement of a shunt, existing studies indicate that there are few benefits of doing so and, in fact, the potential complications outweigh the benefits.
There are many unknowns surrounding a prenatal diagnosis of hydrocephalus. This can be very frightening, distressing and sad for expectant parents, and extremely frustrating for the medical specialists who provide care. Although the outlook and success rate for infants diagnosed at or after birth is generally very high, there are limited studies available on the long-term prognosis of those with prenatal-onset hydrocephalus. Much seems to depend on the extent of associated brain and systemic abnormalities, structural changes in the brain and the extent of the hydrocephalus. It is very difficult to accurately predict future function based on morphology or imaging. The more significant factor in the future development of children with prenatal-onset hydrocephalus is the presence of continuing care and the absence of complications such as infection or hemorrhage
 

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A computerized axial tomography scan is more commonly known by its abbreviated name, CAT scan or CT scan. It is an x-ray procedure which generates cross-sectional views and, if needed, three-dimensional images of the internal organs and structures of the body. A CAT scan is used to define normal and abnormal structures in the body and/or assist in procedures by helping to accurately guide the placement of instruments or treatments.
CAT scans are performed to analyze the internal structures of various parts of the body. This includes the head, where traumatic injuries, (such as blood clots or skull fractures), tumors, and infections can be identified.
Occasionally, contrast material (an x-ray dye) is placed into the spinal fluid to further enhance the scan and the various structural relationships of the spine, the spinal cord, and its nerves. The technique is painless and can provide extremely accurate images of body structures in addition to guiding the radiologist in performing certain procedures, such as biopsies of suspected cancers, removal of internal body fluids for various tests, and the draining of abscesses which are deep in the body. Many of these procedures are minimally invasive and have markedly decreased the need to perform surgery to accomplish the same goal.
The amount of radiation a person receives during a CAT scan is minimal. In men and non-pregnant women, it has not been shown to produce any adverse effects. If a woman is pregnant, there may be a potential risk to the fetus, especially in the first trimester of the pregnancy. If a woman is pregnant, she should inform her doctor of her condition and discuss other potential methods of testing, such as an ultrasound, which are not harmful to the fetus.
 

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The image and resolution produced by MRI is quite detailed and can detect tiny changes of structures within the body. An MRI scan can be used as an extremely accurate method of disease detection throughout the body. In the head, trauma to the brain can be seen as bleeding or swelling. Other abnormalities often found include brain aneurysms, stroke, tumors of the brain, as well as tumors or inflammation of the spine. Often, surgery can be deferred or more accurately directed after knowing the results of an MRI scan.
An MRI scan is a painless radiology technique which has the advantage of avoiding x-ray radiation exposure. There are no known side effects of an MRI scan. The benefits of an MRI scan relate to its precise accuracy in detecting structural abnormalities of the body. During the MRI scan, patients lie in a closed area inside the magnetic tube. Some patients can experience a claustrophobic sensation during the procedure.
After the MRI scanning is completed, the computer generates visual images of the area of the body that was scanned and these images are transferred to film (hard copy). This film is given to a radiologist, a physician who is specially trained to interpret images of the body reproduced on film. The interpretation is transmitted in the form of a report to the practitioner who requested the MRI scan. The practitioner can then discuss the results with the patient and/or family.
 

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A lumbar puncture (an LP) is the insertion of a needle into the fluid within the spinal canal. It is termed a "lumbar puncture" because the needle goes into the lumbar portion (the "small") of the back. An LP is most commonly peformed to diagnose a disease, namely to obtain a sample of the fluid in the spinal canal (the cerebrospinal fluid) for examination. Spinal fluid obtained from the lumbar puncture can be used to diagnose many important diseases such as bleeding around the brain; increased pressure from hydrocephalus; inflammation of the brain, spinal cord, or adjacent tissues (encephalitis, meningitis); tumors of brain or spinal cord, etc.
Spinal fluid is sometimes removed by LP for the purpose of decreasing spinal fluid pressure in patients with uncommon conditions (such as, for examples, normal-pressure hydrocephalus and benign intracranial hypertension).
The patient is typically lying down sideways for the procedure. LPs in infants are often done upright.
When spinal fluid is removed during an LP, the risks include headache, brain herniation, bleeding, and infection. Each of these complications are uncommon with the exception of headache, which can appear from hours to up to a day after LP. Headaches occur less frequently when the patient remains lying flat 1-3 hours after the procedure.

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In the United States, a little over 1 in 1000 births are affected by hydrocephalus.
Hydrocephalus is one of the most common "birth defects" and afflicts in excess of 10,000 babies each year.
Studies by the World Health Organization show that one birth in every 2,000 result in hydrocephalus.
There are 70,000 discharges a year from hospitals in the United States with a diagnosis of hydrocephalus.
More than 50% of hydrocephalus cases are congenital.
As many as 75% of children with hydrocephalus will have some form of motor disability.
Over the past 25 years, death rates associated with hydrocephalus have decreased from 54% to 5%; intellectual disability has decreased from 62% to 30%.
 

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 A shunt complication usually requires another operation to make a surgical revision of the shunt. Depending on the cause of the complication, some or all of the components of the shunt will be replaced. In the event of infection, the child is given a course of antibiotic therapy and usually the entire shunt is replaced.
Although there are exceptional cases in which children receive a shunt and have no further need for revisions or replacements, these cases are rare. Experience shows us that some children undergo several revisions throughout their lives. Whether or not other complications will arise depends on your child’s particular medical problems and bodily reactions to the surgical procedure and the shunt.
Hydrocephalus, left untreated, may cause severe brain damage with physical and mental retardation. We wish there were simple answers about when permanent damage can result —but much depends upon the timeliness and effectiveness of the treatment or the occurrence and severity of complications. The best way to prevent the possibility of your child’s having brain damage is early detection of problems, should they occur. This is why it is so important that you learn the signs and symptoms of shunt malfunction and infection and have your child evaluated regularly by the pediatrician, neurosurgeon, and neurologist.
It is important to develop a strong relationship with the healthcare team and to share information as well. Your pediatrician or family practice physician will provide your child’s primary health care and will consult your neurosurgeon if a problem with the shunt is suspected. Your neurosurgeon will monitor your child’s ventricles and will take care of problems associated with the shunt. A neurologist may follow your child’s neurological status, as well as growth and development. You are an integral part of your child’s health-care team, with your own knowledge of your child’s health and history. Together with the medical professionals, you have the combined skills to provide excellent care for your child.
Some families find peace of mind—and a sense of control—in having their child carry or wear a medical identification bracelet or necklace. Both methods of identification provide valuable medical information such as the names, addresses and telephone numbers of doctors to be contacted in an emergency; shunt type, manufacturer and pressure setting (if appropriate); and any additional information regarding medical conditions or allergies. Whether or not an identification device important to you, it is crucial to keep your child’s medical reports up-to-date and easily accessible

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Dandy Walker is known by many names: Dandy Walker Syndrome, Dandy Walker Malformation, Dandy Walker Cyst, Dandy Walker Variant.
By definition, Dandy Walker is a congenital brain malformation typically characterized by incomplete formation of the cerebellar vermis, dilation of the fourth ventricle, and enlargement of the posterior fossa. In layman's terms, Dandy Walker is a cyst in the cerebellum (typically symmetrical) that is involved with the fourth ventricle. This may interfere with the body's ability to drain cerebrospinal fluid from the brain, resulting in hydrocephalus.
Dandy Walker cysts are formed during early embryonic development, while the brain forms. The cyst in the cerebellum typically has several blood vessels running through it connecting to the brain, thereby prohibiting surgical removal. In most cases the cause of Dandy Walker is not known, though there are a few known cases resulting from autosomal recessive genes.
Dandy Walker is generally discovered before birth, by ultrasound. Symptoms in the newborn may include a bulge or lump on the back of the head ("bulging occiput"); a "hair collar", or patch of long hair just above this lump; red pigmented skin in the area of the lump (similar to a "stork bite" birthmark); increased head circumference (if hydrocephalus is present); a downward gaze (if hydrocephalus is present). Other symptoms of the syndrome can also include congenital heart problems; malformations of the face, fingers, or toes; other central nervous system structural anomalies (often agenesis of the corpus callosum); abnormal breathing patterns; motor delays; hypotonia; learning problems; mental retardation; high pain tolerance; seizures; reflux; nystagmus (jerky eyes); ataxia; and other complications.
There is an extreme range of severity with Dandy Walker. Some people have it their whole lives without any symptoms ever presenting; some infants may have it in association with other syndromes resulting in severe complications or death.
It is difficult to find information about Dandy Walker on the internet. The list that follows includes sources we have found, along with the names and addresses of hydrocephalus organizations that may be able to offer more information

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NPH stands for Normal Pressure Hydrocephalus, and it’s a potentially reversible form of dementia. Although it denotes progressive short-term memory loss, other symptoms—trouble when starting to walk (feet seem magnetized to the floor) and urinary urgency/ inconsistence—make it distinct from Alzheimer’s disease, Parkinson’s disease/ dementia or multi-infarct dementia.
With NPH, fluid that surrounds brain tissue builds up with too much pressure, distorting and injuring nerve tissue and possibly compromising blood flow.
The cause of this excessive brain fluid pressure remains elusive in at least half the people with NPH. Known causes for NPH include a history of ruptured brain aneurysm, brain trauma and meningitis.
There is no single test to identify NPH. Often, the symptoms of NPH are attributed to other conditions. A careful history and examination by a neurologist when memory loss occurs is a good first step.
An MRI study may very well show enlargement of the brain cavities, ventricles, due to a buildup of excess cerebrospinal fluid. A neurologist may perform a spinal tap to remove fluid and see whether symptoms improve. If they do, the patient very likely has treatable NPH.

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Today, the best and most effective treatment for hydrocephalus known is a surgical procedure in which a flexible tube called a shunt is placed into the child’s CSF (cerebrospinal fluid) system.
The shunt diverts the flow of CSF from the ventricles into another region of the body, most often the abdominal cavity or a chamber of the heart called the atrium. The shunt tube is about 1/8” in diameter and is made of a soft and pliable plastic (usually Silastic®) that is well-tolerated by our body tissues. Shunt systems come in a variety of models but have similar functional components. Catheters (tubing) and a flow-control mechanism (one-way valve) are components common to all shunts.
The parts of a shunt are named according to where they are placed in the body. The portion of the tube which is inserted into the ventricles is called the ventricular catheter. The peritoneal catheter is the portion of the tube which passes the CSF into the abdomen (peritoneal cavity). If the tube is place d into the right atrium of the heart it is called the atrial catheter. The valve regulates the pressure of the CSF flow and prevents backward flow of spinal fluid toward the ventricles. Valves are designed to operate at low, medium, or high pressure. Depending on the particular nature of your child’s hydrocephalus, your neurosurgeon will select an appropriate valve pressure to accommodate your child’s needs. Some valve models have an on/off control device.
Many shunt systems also have a flexible flushing chamber (reservoir) which may be housed within the same unit as the valve or may be a separate unit along the shunt, depending in the design of the shunt system. This chamber serves several important purposes. It permits your physician to remove samples of CSF from the shunt with a needle and syringe, and to inject the chamber for testing shunt function and for treatment. The chamber also allows the shunt to be “flushed” or pumped. This is accomplished simply by pressing on the skin overlying the flushing chamber. Depending on the design of the shunt and the manner in which the chamber is pressed, fluid can be forced toward or away from the ventricles. Flushing the chamber sometimes allows your doctor or nurse to determine whether the shunt is functioning properly. Because serious problems may result from too frequent or improper flushing of the chamber, parents and children should never try to manipulate the shunt system on their own unless they are explicitly instructed to do so by their physician.
To get a better idea of what a shunt looks like, ask your neurosurgeon or nurse to show you samples of the shunts they use. Have them write down for you the name and model of the shunt your child has. This is important information for you to know and can be helpful to any nurse or neurosurgeon who checks your child’s shunt. It is especially important to know if the shunt has an on/off control valve because it is possible to shut the valve off accidentally. If the valve were shut off, it would result in increased pressure in the head that could cause the child to go into a coma.

 

Common components of a shunt	Ventriculo-peritoneal (VP) shunt	Ventiriculo-atrial shunt

 

Ventriculo-pleural (VP) shunt	Lumbo-peritoneal shunt

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The surgical procedure is carried out under sterile conditions in the operating room. Although the operation is relatively short, careful preparation for the surgery adds extra time. In order to help prevent infection, some of the hair on the head may need to be shaved. The head and body are washed with special soap. Sterile linen is used to cover the patient and to maintain the sterile environment throughout the surgery.
A small incision (cut) is made in the scalp (the skin covering the head). A small hole is then made in the skull. A tiny opening is made in the dura, a protective covering of the brain. These openings are made to accommodate the ventricular catheter (proximal catheter) being placed into the lateral ventricle. The neurosurgeon then makes two or three small incisions in order to place the shunt valve (usually above or behind the ear). The peritoneal or atrial catheter (distal catheter) is tunneled under the skin to the abdominal or neck incision. Finally, the end of the catheter is carefully placed either in the peritoneal cavity or in a vein of the neck leading to the atrium of the heart. Following the operation, small sterile bandages are applied to each incision.
Immediately after surgery, the patient will go to the post-anesthesia care unit. The patient will remain there for close observation for about an hour and then go to his/her room. The length of hospitalization varies from patient to patient. Most patients leave the hospital within two to seven days, depending on their clinical progress.
Although this is the usual procedure when a shunt is placed, each patient may have a slightly different experience based upon their neurosurgeon, hospital, and the need to individualize the care for the patient.
 

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