Acquired Hydrocephalus

Acquired Hydrocephalus

Article
Brain & Nerve
Diseases & Conditions
+3
Contributed byMaulik P. Purohit MD MPHMar 25, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Acquired Aqueductal Stenosis
  • Post-Traumatic Hydrocephalus
  • Water in the Brain

What is Acquired Hydrocephalus? (Definition/Background Information)

  • Hydrocephalus is a buildup of excess fluid in the brain. There are 2 types of hydrocephalus:
    • Acquired Hydrocephalous
    • Congenital Hydrocephalous
  • Acquired Hydrocephalus is the abnormal buildup of cerebrospinal fluid (CSF) inside several cavities of the brain. This is caused either by certain developmental irregularities and disorders, or due to injuries to the head or brain
  • The uncharacteristic buildup of CSF in the brain may injure the organ, resulting in physical and mental abnormalities. Early detection and treatment is vital to prevent adverse effects of this abnormality
  • The prognosis is dependent on a variety of factors; however, the key factor that determines the outcome is the cause of Acquired Hydrocephalus

Who gets Acquired Hydrocephalus? (Age and Sex Distribution)

  • Any individual (a child, or a young or old adult) may develop hydrocephalus; it is chiefly dependent upon the risk factors, the underlying disorders, or morphological abnormalities (if any present)
  • However, it has been observed that the two key ‘stages of life’ for hydrocephalus presentation is early infancy (congenital) and adulthood
  • Generally, both male and female sexes are equally prone to the condition, with the exception of Bickers-Adam syndrome (where only male babies are affected by the congenital type)
  • Information on racial or ethnic preference (if any) is unavailable

What are the Risk Factors for Acquired Hydrocephalus? (Predisposing Factors)

Risk factors for Acquired Hydrocephalus include:

  • Excess production of cerebrospinal fluid (CSF), or inability of the brain to absorb sufficient CSF
  • Any obstruction to the free flow of CSF within the central spinal cord
  • Brain hemorrhages, tumors or cysts (in the brain or spinal cord)
  • Preterm delivery causing bleeding in the brain
  • Infections that affect the brain or central nervous system - like bacterial meningitis, encephalitis, mumps, and cysticercosis
  • Head or brain injury (stroke), any external trauma affecting these regions
  • Achondroplasia or venous thrombosis, which results in an increased cranial pressure
  • Certain surgical procedures (like removal of a tumor) may cause a blockage of ‘CSF flow regular pathways’

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Acquired Hydrocephalus? (Etiology)

  • The cerebrospinal fluid has an important role to play in effective functioning of the brain. It regulates the flow of blood within the brain and also acts as a protective buffer around the brain, by absorbing any external force or shock, and by providing an immunological barrier against disease or any infection
  • Hydrocephalus occurs, when there is an excess of CSF in the brain cavities, which cannot be drained or absorbed. In some cases there may also be an overproduction of the cerebrospinal fluid. These factors build up pressure in the head to disproportionate levels, and has a potential of causing serious long-term harm, including death
  • Any trauma, disease, or infection, affecting the brain can bring about Acquired Hydrocephalus. The acquired causes of hydrocephalus in adults and infants are not significantly different
  • Not all cases of hydrocephalus have identifiable causes. Some are due to unknown factors, such as genetic factors, morphological and anatomical defects. In such cases, it is called idiopathic hydrocephalus

What are the Signs and Symptoms of Acquired Hydrocephalus?

The signs and symptoms of Hydrocephalus (Acquired) differ for infants, children, and adults. It is influenced by the individual’s age, causal factor, onset conditions, location, and duration.

In infants, the signs and symptoms of hydrocephalus include:

  • Lethargy, sleepiness
  • Irritability
  • Seizures (convulsions)
  • Drooping, downward deviating eyes, termed as ‘sunsetting’
  • Feeding troubles, frequent vomiting
  • Developmental delays
  • Infants usually have a large head (considering the body proportion)

In children, the signs and symptoms of hydrocephalus include:

  • Lethargy, sleepiness, drowsiness
  • Irritability, headaches
  • Blurred vision, swollen optic nerve, ‘sunsetting’ of the eyes
  • Vomiting (particularly in the mornings) and nausea
  • Unsteady walking posture, loss of balance
  • Dementia, memory loss, cognition and coordination problems, speech impairment
  • Seizures
  • Urinary incontinence
  • Growth delays, pubertal problems (delayed or early)

In adults, the signs and symptoms of hydrocephalus include:

  • Dementia, progressive deterioration of the senses, memory loss
  • Vision or ophthalmological problems, such as double vision, blurred vision
  • Headaches, neck pain
  • Difficulty walking, maintaining balance
  • Vomiting, nausea, and drowsiness
  • Urinary incontinence, involuntary bowel movements (loss of control)

How is Acquired Hydrocephalus Diagnosed?

The main diagnostic tools for Acquired Hydrocephalus include:

  • Physical examination with a complete evaluation of medical history
  • Neurological assessment that include checking reflexes, muscle strength and tone, sensory perceptions (such as, sight, hearing, touch), mental health, coordination and balance
  • Imaging studies performed could include: MRI scan of the central nervous system (brain and spine), CT scan of the head and neck, pressure tests to detect pressure of CSF in the brain, ultrasound scans (for babies)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Acquired Hydrocephalus?

Complications due to Acquired Hydrocephalus may include the following:

  • Severe hydrocephalus can pose a serious threat to normal physical and mental growth of the baby
  • A sudden increase in pressure in the skull or on the brain, can lead to an emergency medical situation
  • Significant loss of motor and neurological skills
  • Vision defects
  • Many diseases and conditions could cause fluid pressure buildup in the brain. The presence of these conditions could create additional medical complications
  • In treating hydrocephalus, a shunt system is surgically inserted into the skull. This may cause infection, bleeding, obstruction, over-drainage, or the shunt device itself may fail. In many cases multiple shunt systems may be required to treat the condition, due to repeat malfunctions

How is Acquired Hydrocephalus Treated?

Following are the treatment measures for Acquired Hydrocephalus:

Very mild cases of excess fluid in the brain and associated symptomatic features may be treated with medications. However, surgical interventions are required to drain excess fluid, relieve the pressure buildup, and treat hydrocephalus in most cases. In moderate to severe cases, the surgery is performed when the individual is ‘medically fit’ to undergo such a procedure.

There are two main invasive procedures - the shunt system placement and endoscopic third ventriculostomy (ETV) procedure.

Shunt system placement:

  • A mechanical device consisting of a valve and two catheters (small, flexible tubes), known as a shunt, is normally used to treat hydrocephalus. This is the standard and most preferred option used
  • One tube is surgically inserted into the head, the valve is placed around the ear, and any excess CSF is drained from the brain, by directing the other tube beneath the skin, usually into the stomach (sometimes into the lung or heart)
  • The shunt is left in place, until the condition has improved and the individual is discharged
  • Instructions are provided to the parents, on how to take care of the individual, at home (if the individual is a child)

Endoscopic third ventriculostomy (ETV) procedure:

  • A tiny hole is surgically made in the wall of the brain cavity. Using endoscopes, the CSF is routed to another location of the body, for it to be reabsorbed
  • The rerouting bypasses the obstruction causing the fluid buildup in the brain (such as a tumor, hemorrhage, or cyst)
  • ETV helps in normalizing the pressure by avoiding a shunt system - it is an alternative treatment choice, but this method has to be compatible with the patient’s medical condition

How can Acquired Hydrocephalus be Prevented?

  • There are many causes for Acquired Hydrocephalus - some causal factors are preventable or avoidable, while other factors may not be so. For example, a trauma-related hydrocephalus can be avoided
  • Genetic counseling with genetic testing should be considered by individuals having a family history of the condition, and planning for a child

What is the Prognosis of Acquired Hydrocephalus? (Outcomes/Resolutions)

The prognosis for an individual with Acquired Hydrocephalus is based on a combination of many factors, which include:

  • Degree of severity of hydrocephalus
  • Causative factor of the condition
  • Age of the infant, child, or adult
  • Health and medical history of the individual
  • Tolerance level of the individual, when subjected to the procedures and medications; a very young child, or an older adult may have lower tolerance levels
  • Success of the surgical procedures performed
  • Complications that may develop, if any due to Acquired Hydrocephalus

In some cases, life-long management of the condition is necessary, since the brain and other vital organs may be permanently affected; there could also be abnormal developments (both physical and mental).

  • An early detection and aggressive treatment of Acquired Hydrocephalus, with regular follow-up health checkup is essential, in all cases
  • Untreated cases of the condition have mortality rates of 60%; others who survive, may have permanent debilitating physical and mental disorders
  • Causative factor of this brain disorder plays an important role in determining prognosis of Acquired Hydrocephalus; individuals with infections and tumors do the worst

Additional and Relevant Useful Information for Acquired Hydrocephalus:

  • Congenital Hydrocephalus is a birth defect, caused either by genetic defects, or fetal growth and development anomalies
  • The success rate of a first time shunt is around 1 in 3. Most patients would require multiple shunts to manage their condition
  • Those living in geographical locations with limited access to healthcare, due to economic hardships cannot afford the high cost of a shunt system placement procedure. As a result, hydrocephalus mortality rates in such regions are significantly much higher
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Maulik P. Purohit MD MPH picture
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Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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