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Hydrocephalus

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Hydrocephalus is the buildup of fluid in the cavities (ventricles) deep within the brain. The excess fluid increases the size of the ventricles and puts pressure on the brain. The term hydrocephalus is derived from the Greek words "hydro" meaning water and "cephalus" meaning head.

Although hydrocephalus was once known as "water on the brain," the "water" is actually cerebrospinal fluid (CSF) — a clear fluid that normally surrounds & baths the brain and spinal column. The excessive accumulation of CSF results in an abnormal widening of spaces in the brain called ventricles (fluid-containing cavities). This widening creates potentially harmful pressure on the tissues of the brain and can damage brain tissues, causing a range of impairments in brain function.

  • The ventricular system is made up of four ventricles connected by narrow passages. Normally, CSF flows through the ventricles, exits into cisterns (closed spaces that serve as reservoirs) at the base of the brain, bathes the surfaces of the brain and spinal cord, and then reabsorbs into the bloodstream.

CSF has three important life-sustaining functions: 1) to keep the brain tissue buoyant, acting as a cushion or "shock absorber"; 2) to act as the vehicle for delivering nutrients to the brain and removing waste; and 3) to flow between the cranium and spine and compensate for changes in intracranial blood volume (the amount of blood within the brain).

Hydrocephalus can happen at any age, but it occurs more frequently among infants and adults 60 and over. Surgical treatment for hydrocephalus can restore and maintain normal cerebrospinal fluid levels in the brain. Many different therapies are often required to manage symptoms or functional impairments resulting from hydrocephalus.

What are the different types of hydrocephalus?

  • 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 condition is acquired as a consequence of CNS infections, meningitis, brain tumors, head trauma, toxoplasmosis, intracranial hemorhhage (subarachnoid or intraparenchymal) and is usually painful. This type of hydrocephalus can affect individuals of all ages and may be caused by injury or disease.
  • 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 (NPH).
    • Hydrocephalus ex-vacuo occurs when stroke or traumatic injury cause damage to the brain. In these cases, brain tissue may actually shrink.
    • NPH is an abnormal increase of cerebrospinal fluid in the brain's ventricles that may result from a subarachnoid hemorrhage, head trauma, infection, tumor, or complications of surgery. However, many people develop NPH when none of these factors are present. An estimated 375,000 older Americans have NPH.

Symptoms vary somewhat by age of onset

Infants - common signs and symptoms:

  • Changes in the head
    • An unusually large head
    • A rapid increase in the size of the head
    • A bulging or tense soft spot (fontanel) on the top of the head
  • Physical signs and symptoms
    • Vomiting
    • Sleepiness
    • Irritability
    • Poor feeding
    • Seizures
    • Eyes fixed downward (sunsetting of the eyes)
    • Deficits in muscle tone and strength
    • Poor responsiveness to touch
    • Poor growth

Toddlers and older children signs and symptoms may include:

  • Physical signs and symptoms
    • Headache
    • Blurred or double vision
    • Eyes fixed downward (sunsetting of eyes)
    • Abnormal enlargement of a toddler's head
    • Sleepiness or lethargy
    • Nausea or vomiting
    • Unstable balance
    • Poor coordination
    • Poor appetite
    • Seizures
    • Urinary incontinence
  • Behavioral and cognitive changes
    • Irritability
    • Change in personality
    • Decline in school performance
    • Delays or problems with previously acquired skills, such as walking or talking

Young and middle-aged adults common signs and symptoms:

  • Headache
  • Lethargy
  • Loss of coordination or balance
  • Loss of bladder control or a frequent urge to urinate
  • Impaired vision
  • Decline in memory, concentration and other thinking skills that may affect job performance

Older adults - among adults 60 years of age and older, the more common signs and symptoms include:

  • Loss of bladder control or a frequent urge to urinate
  • Memory loss
  • Progressive loss of other thinking or reasoning skills
  • Difficulty walking, often described as a shuffling gait or the feeling of the feet being stuck
  • Poor coordination or balance

Seek emergency medical care for infants and toddlers experiencing these signs and symptoms:

  • A high-pitched cry
  • Problems with sucking or feeding
  • Unexplained, recurrent vomiting
  • An unwillingness to move the head or lay down
  • Breathing difficulties
  • Seizures
  • Seek prompt medical attention for other signs or symptoms in any age group. Because more than one condition can result in the problems associated with hydrocephalus, it's important to get a timely diagnosis and appropriate care.

Causes

Hydrocephalus is caused by an imbalance between how much cerebrospinal fluid is produced and how much is absorbed into the bloodstream.

Cerebrospinal fluid is produced by tissues lining the ventricles of the brain. It flows through the ventricles by way of interconnecting channels. The fluid eventually flows into spaces around the brain and spinal column. It's absorbed primarily by blood vessels in tissues near the base of the brain.

Cerebrospinal fluid plays an important role in brain function by:

  • Keeping the brain buoyant, allowing the relatively heavy brain to float within the skull
  • Cushioning the brain to prevent injury
  • Removing waste products of the brain's metabolism
  • Flowing back and forth between the brain cavity and spinal column to maintain a constant pressure within the brain — compensating for changes in blood pressure in the brain

Excess cerebrospinal fluid in the ventricles occurs for one of the following reasons:

  • Obstruction. The most common problem is a partial obstruction of the normal flow of cerebrospinal fluid, either from one ventricle to another or from the ventricles to other spaces around the brain.
  • Poor absorption. Less common is a problem with the mechanisms that enable the blood vessels to absorb cerebrospinal fluid. This is often related to inflammation of brain tissues from disease or injury.
  • Overproduction. Rarely, cerebrospinal fluid is created more quickly than it can be absorbed.

Risk factors

In many cases, the exact event leading to hydrocephalus is unknown. However, a number of developmental or medical problems can contribute to or trigger hydrocephalus.

Newborns

  • Hydrocephalus present at birth (congenital) or shortly after birth may occur because of any of the following:
    • Abnormal development of the central nervous system that can obstruct the flow of cerebrospinal fluid
    • Bleeding within the ventricles, a possible complication of premature birth
    • Infection in the uterus during a pregnancy, such as rubella or syphilis, that can cause inflammation in fetal brain tissues
  • Other contributing factors among any age group include:
    • Lesions or tumors of the brain or spinal cord
    • Central nervous system infections, such as bacterial meningitis or mumps
    • Bleeding in the brain from a stroke or head injury
    • Other traumatic injury to the brain
  • Complications:
    • Long-term complications of hydrocephalus can vary widely and are often difficult to predict.
    • If hydrocephalus has progressed by the time of birth, it may result in significant intellectual, developmental and physical disabilities. Less severe cases, when treated appropriately, may have few, if any, serious complications.
    • Adults who have experienced a significant decline in memory or other thinking skills generally have poorer recoveries and persistent symptoms after treatment of hydrocephalus.
  • The severity of complications depends on:
    • Underlying medical or developmental problems
    • Severity of initial symptoms
    • Timeliness of diagnosis and treatment

Prognosis

The prognosis for hydrocephalus depends on the cause, the extent of symptoms and the timeliness of diagnosis and treatment.

  • 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. Most hydrocephalus patients lead long, problem-free lives. However, left untreated, progressive hydrocephalus is generally fatal.
  • Children often have a full life span if hydrocephalus is caught early and treated. Infants who undergo surgical treatment to reduce the excess fluid in the brain and survive to age one will not have a shortened life expectancy due to hydrocephalus. Adults who develop hydrocephalus typically have a shortened life span.
  • There is no medical therapy to treat hydrocephalus. The only effective treatments are surgical.
    • While many people are helped by surgery, many more need further operations to stay well. Of the nearly 40,000 hydrocephalus operations performed annually (one every 15 minutes), only 30% are the patient’s first surgery to treat hydrocephalus.

History

  • References to hydrocephalic skulls can be found in ancient Egyptian medical literature from 2500 BC to 500 AD. Hydrocephalus was described more clearly by the ancient Greek physician Hippocrates in the 4th century BC, while a more accurate description was later given by the Roman physician Galen in the 2nd century AD.
  • The first clinical description of an operative procedure for hydrocephalus appears in the Al-Tasrif (1000 AD) by the Arab surgeon, Abulcasis, who clearly described the evacuation of superficial intracranial fluid in hydrocephalic children. He described it in his chapter on neurosurgical disease, describing infantile hydrocephalus as being caused by mechanical compression.
  • In 1881, a few years after the landmark study of Retzius and Key, Carl Wernicke pioneered sterile ventricular puncture and external drainage of cerebrospinal fluid for the treatment of hydrocephalus.[20] It remained an intractable condition until the 20th century, when shunts and other neurosurgical treatment modalities were developed.
  • It is a lesser-known medical condition; relatively little research is conducted to improve treatment, and there is still no cure. In developing countries, the condition often goes untreated at birth. Before birth, the condition is difficult to diagnose, and there is limited access to medical treatment. However, when head swelling is prominent, children are taken at great expense for treatment. By then, brain tissue is undeveloped and neurosurgery is rare and difficult. Children more commonly live with undeveloped brain tissue and consequential intellectual disabilities and restrictions.
  • See also: Oxford Academic; Paolo Missori, Sergio Paolini, Antonio Currà; From congenital to idiopathic adult hydrocephalus: a historical research, Brain, Volume 133, Issue 6, 1 June 2010, Pages 1836–1849, https://doi.org/10.1093/brain/awq014

Statistics & Mortality

  • Over 1,000,000 people in the United States currently live with hydrocephalus.
  • For every 1,000 babies born in the United States, one to two will have hydrocephalus (occurs in about 1 out of 500 babies), making it as common as Down’s syndrome and more common than spina bifida or brain tumors..
  • Hydrocephalus is the most common reason for brain surgery in children.
  • It is estimated that more than 700,000 Americans have NPH, but less than 20% receive an appropriate diagnosis.
  • Hydrocephalus affects approximately 1 million Americans, in every stage of life, from infants to the elderly. It affects people in all walks of life, from every socioeconomic background.
  • The key to a good prognosis is early detection and treatment, along with preventing infections. Most of the newborns born with hydrocephalus will have a normal lifespan, and approximately 40 to 50 percent will have normal intelligence. Seizure disorders have been diagnosed in about 10 percent of children with hydrocephalus.
  • The mortality rate for infants is approximately 5 percent. 
  • The authors identified 10,406 deaths attributed to childhood hydrocephalus within the 20-year study period. The overall mortality rate was 0.71 per 100,00 person- years. Mortality rates were highest in infants, with 3979 deaths; they were similar between girls and boys. Compared with white infants, black infants had higher relative risk (RR) for death caused by congenital hydrocephalus (RR 1.46, p value < 0.0001) and acquired hydrocephalus (RR 2.58, p value < 0.0001) but not for that caused by hydrocephalus with spina bifida (RR 0.65, p value < 0.0001). From 1979 to 1998, (OMICS - Hydrocephalus)
  • Historically, the outcome of hydrocephalus used to be appalling: Laurence reported in 1962 a 20% survival rate into adulthood, the surviving patients having generally severe sequels. The outcome of hydrocephalic children has undergone a sea change when shunts were introduced in the 1960s, with most patients surviving the initial phase and growing into adulthood. However, the long-term outcome of children treated for hydrocephalus is poorly documented because most studies available were based on surveys, and thus subject to important selection biases, and involving limited numbers of patients, or very detailed studies with a short follow-up. (NCBI - Adult outcome of pediatric hydrocephalus. by: Matthieu Vinchon, Marc Baroncini, & Isabelle Delestret. Childs Nerv Syst. 2012 Jun; 28(6): 847–854. Published online 2012 Feb 19. doi:  10.1007/s00381-012-1723-y)
    • Mortality before 20 years affected 287 patients, making for an actuarial mortality rate of 18.1% at 20 years. Among survivors, 15 (2.85%) died after the age of 20 years, with 5 of these dying of shunt-related causes. The last casualty was recorded at the age of 27 years; the actuarial overall mortality rate after 20 years was 6.3%.
    • Few studies focus on the long-term mortality in pediatric patients treated for hydrocephalus. The mortality rate ranges between 13% after 17 years, 22% at 20 years, and 22% at 10 years, but series are often polluted by intercurrent causes of mortality. Focusing on nontumoral hydrocephalus, Tuli and Casey found similar figures (12.6% and 11% mortality at 10 years, respectively); by contrast, Paulsen found a shunt-related mortality rate of only 2.9% after 20 years.
    • Most children treated for hydrocephalus now reach adult age; however, data on their outcome are still rare and controversial. A life-long neurosurgical follow-up of these patients must be organized and adapted to the specific needs of each patient.

Notable cases

Where can I get more information?

See: National Institute of Neurological Disorders & Stroke - Hydrocephalus Fact Sheet under the above heading for organizations. Address, phone number & online site are listed for each.

References & Additional Reading

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  • This project is found under the “Death by Natural Causes” --> "Neurological Disorders / Diseases" headings.
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