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clinicalstudio.blogspot.com
#What is hydrocephalus ?
 The term hydrocephalus is derived from the Greek
 words "hydro" meaning water and "cephalus" meaning
 head. As the name implies, it is a condition in which
 the primary characteristic is excessive accumulation of
 fluid in the brain. Although hydrocephalus was once
 known as "water on the brain," the "water" is actually
 cerebrospinal fluid (CSF)--a clear fluid that surrounds
 the brain and spinal cord. The excessive accumulation
 of CSF results in an abnormal widening of spaces in
 the brain called ventricles. This widening creates
 potentially harmful pressure on the tissues of the
 brain.
 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).
 The balance between production and absorption of CSF is
  critically important. Because CSF is made continuously,
  medical conditions that block its normal flow or
  absorption will result in an over-accumulation of CSF. The
  resulting pressure of the fluid against brain tissue is what
  causes hydrocephalus.
#Typeses of hydrocephalus

 Hydrocephalus may be congenital or acquired.
 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 type of hydrocephalus
 can affect individuals of all ages and may be caused by
 injury or disease.
 Hydrocephalus may also be communicating or non-
  communicating.
 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.
 Hydrocephalus ex-vacuo occurs when stroke or traumatic
  injury cause damage to the brain. In these cases, brain
  tissue may actually shrink. Normal pressure hydrocephalus
  can happen to people at any age, but it is most common
  among the elderly. It may result from a subarachnoid
  hemorrhage, head trauma, infection, tumor, or
  complications of surgery. However, many people develop
  normal pressure hydrocephalus even when none of these
  factors are present for reasons that are unknown.
Who gets this hydrocephalus?

 The number of people who develop hydrocephalus or
 who are currently living with it is difficult to establish
 since there is no national registry or database of
 people with the condition. However, experts estimate
 that hydrocephalus affects approximately 1 in every
 500 children.
What causes hydrocephalus?

 The causes of hydrocephalus are still not well
 understood. Hydrocephalus may result from inherited
 genetic abnormalities (such as the genetic defect that
 causes aqueductal stenosis) or developmental
 disorders (such as those associated with neural tube
 defects including spina bifida and encephalocele).
 Other possible causes include complications of
 premature birth such as intraventricular hemorrhage,
 diseases such as meningitis, tumors, traumatic head
 injury, or subarachnoid hemorrhage, which block the
 exit of CSF from the ventricles to the cisterns or
 eliminate the passageway for CSF into the cisterns.
#Symptoms of hydrocephalus:
 Symptoms of hydrocephalus vary with age, disease
 progression, and individual differences in tolerance to
 the condition. For example, an infant's ability to
 compensate for increased CSF pressure and
 enlargement of the ventricles differs from an adult's.
 The infant skull can expand to accommodate the
 buildup of CSF because the sutures (the fibrous joints
 that connect the bones of the skull) have not yet
 closed.
 In infancy, the most obvious indication of
 hydrocephalus is often a rapid increase in head
 circumference or an unusually large head size. Other
 symptoms may include vomiting, sleepiness,
 irritability, downward deviation of the eyes (also called
 "sunsetting"), and seizures.
 Older children and adults may experience different
 symptoms because their skulls cannot expand to
 accommodate the buildup of CSF. Symptoms may
 include headache followed by vomiting, nausea,
 papilledema (swelling of the optic disk which is part of
 the optic nerve), blurred or double vision, sunsetting
 of the eyes, problems with balance, poor coordination,
 gait disturbance, urinary incontinence, slowing or loss
 of developmental progress, lethargy, drowsiness,
 irritability, or other changes in personality or cognition
 including memory loss.
 Symptoms of normal pressure hydrocephalus include,
 problems with walking, impaired bladder control
 leading to urinary frequency and/or incontinence, and
 progressive mental impairment and dementia. An
 individual with this type of hydrocephalus may have a
 general slowing of movements or may complain that
 his or her feet feel "stuck." Because some of these
 symptoms may also be experienced in other disorders
 such as Alzheimer's disease, Parkinson's disease,
 and Creutzfeldt-Jakob disease, normal pressure
 hydrocephalus is often incorrectly diagnosed and
 never properly treated
 Doctors may use a variety of tests, including brain
 scans (CT and/or MRI), a spinal tap or lumbar
 catheter, intracranial pressure monitoring, and
 neuropsychological tests, to help them accurately
 diagnose normal pressure hydrocephalus and rule out
 any other conditions.
 The symptoms described in this section account for
 the most typical ways in which progressive
 hydrocephalus manifests itself, but it is important to
 remember that symptoms vary significantly from one
 person to the next.
Diagnosis:
 Hydrocephalus is diagnosed through clinical
 neurological evaluation and by using cranial imaging
 techniques such as ultrasonography, computed
 tomography (CT), magnetic resonance imaging (MRI),
 or pressure-monitoring techniques. A physician selects
 the appropriate diagnostic tool based on an
 individual's age, clinical presentation, and the
 presence of known or suspected abnormalities of the
 brain or spinal cord.
aqueductal stenosis(non communicating type)
Aqueductal Stenosis :
CAT Scan
Communicating hydrocephalus
Dilated ventricles
Treatment:
 Hydrocephalus is most often treated by surgically inserting
  a shunt system. This system diverts the flow of CSF from
  the CNS to another area of the body where it can be
  absorbed as part of the normal circulatory process.
 A shunt is a flexible but sturdy plastic tube. A shunt system
  consists of the shunt, a catheter, and a valve. One end of
  the catheter is placed within a ventricle inside the brain or
  in the CSF outside the spinal cord. The other end of the
  catheter is commonly placed within the abdominal cavity,
  but may also be placed at other sites in the body such as a
  chamber of the heart or areas around the lung where the
  CSF can drain and be absorbed. A valve located along the
  catheter maintains one-way flow and regulates the rate of
  CSF flow.
 A limited number of individuals can be treated with an
 alternative procedure called third ventriculostomy. In
 this procedure, a neuroendoscope — a small camera
 that uses fiber optic technology to visualize small and
 difficult to reach surgical areas — allows a doctor to
 view the ventricular surface. Once the scope is guided
 into position, a small tool makes a tiny hole in the
 floor of the third ventricle, which allows the CSF to
 bypass the obstruction and flow toward the site of
 resorption around the surface of the brain.
third ventriculostomy
complications of a shunt system
 Shunt systems are not perfect devices. Complications may
  include mechanical failure, infections, obstructions, and
  the need to lengthen or replace the catheter. Generally,
  shunt systems require monitoring and regular medical
  follow up. When complications occur, the shunt system
  usually requires some type of revision.
 Some complications can lead to other problems such as
  overdraining or underdraining. Overdraining occurs when
  the shunt allows CSF to drain from the ventricles more
  quickly than it is produced. Overdraining can cause the
  ventricles to collapse, tearing blood vessels and causing
  headache, hemorrhage (subdural hematoma), or slit-like
  ventricles (slit ventricle syndrome).
 Underdraining occurs when CSF is not removed
 quickly enough and the symptoms of hydrocephalus
 recur. In addition to the common symptoms of
 hydrocephalus, infections from a shunt may also
 produce symptoms such as a low-grade fever, soreness
 of the neck or shoulder muscles, and redness or
 tenderness along the shunt tract. When there is reason
 to suspect that a shunt system is not functioning
 properly (for example, if the symptoms of
 hydrocephalus return), medical attention should be
 sought immediately.
Prognosis:
 The prognosis for individuals diagnosed with
 hydrocephalus is difficult to predict, although there is
 some correlation between the specific cause of the
 hydrocephalus and the outcome. Prognosis is further
 complicated by the presence of associated disorders,
 the timeliness of diagnosis, and the success of
 treatment. The degree to which relief of CSF pressure
 following shunt surgery can minimize or reverse
 damage to the brain is not well understood.
 Affected individuals and their families should be aware
 that hydrocephalus poses risks to both cognitive and
 physical development. However, many children
 diagnosed with the disorder benefit from
 rehabilitation therapies and educational interventions
 and go on to lead normal lives with few limitations.
 Treatment by an interdisciplinary team of medical
 professionals, rehabilitation specialists, and
 educational experts is critical to a positive outcome.
 Left untreated, progressive hydrocephalus may be
 fatal.
 The symptoms of normal pressure hydrocephalus
 usually get worse over time if the condition is not
 treated, although some people may experience
 temporary improvements. While the success of
 treatment with shunts varies from person to person,
 some people recover almost completely after treatment
 and have a good quality of life. Early diagnosis and
 treatment improves the chance of a good recovery.
Research:
 The National Institute of Neurological Disorders and
 Stroke (NINDS) and other institutes of the National
 Institutes of Health (NIH) conduct research related to
 hydrocephalus in laboratories and clinics at the NIH
 and support additional research through grants to
 major medical institutions across the country. Much of
 this research focuses on finding better ways to prevent,
 treat, and ultimately cure disorders such as
 hydrocephalus. The NINDS also conducts and
 supports a wide range of fundamental studies that
 explore the complex mechanisms of normal and
 abnormal brain development.
Reference:
http://clinicalstudio.blogspot.com/
Emedicine.medscape.com
medicinenet.com

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Hydrocephalus

  • 2. #What is hydrocephalus ?  The term hydrocephalus is derived from the Greek words "hydro" meaning water and "cephalus" meaning head. As the name implies, it is a condition in which the primary characteristic is excessive accumulation of fluid in the brain. Although hydrocephalus was once known as "water on the brain," the "water" is actually cerebrospinal fluid (CSF)--a clear fluid that surrounds the brain and spinal cord. The excessive accumulation of CSF results in an abnormal widening of spaces in the brain called ventricles. This widening creates potentially harmful pressure on the tissues of the brain.
  • 3.
  • 4.  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.
  • 5.  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).  The balance between production and absorption of CSF is critically important. Because CSF is made continuously, medical conditions that block its normal flow or absorption will result in an over-accumulation of CSF. The resulting pressure of the fluid against brain tissue is what causes hydrocephalus.
  • 6. #Typeses of hydrocephalus  Hydrocephalus may be congenital or acquired. 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 type of hydrocephalus can affect individuals of all ages and may be caused by injury or disease.
  • 7.  Hydrocephalus may also be communicating or non- communicating.  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.
  • 8.  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.  Hydrocephalus ex-vacuo occurs when stroke or traumatic injury cause damage to the brain. In these cases, brain tissue may actually shrink. Normal pressure hydrocephalus can happen to people at any age, but it is most common among the elderly. It may result from a subarachnoid hemorrhage, head trauma, infection, tumor, or complications of surgery. However, many people develop normal pressure hydrocephalus even when none of these factors are present for reasons that are unknown.
  • 9. Who gets this hydrocephalus?  The number of people who develop hydrocephalus or who are currently living with it is difficult to establish since there is no national registry or database of people with the condition. However, experts estimate that hydrocephalus affects approximately 1 in every 500 children.
  • 10. What causes hydrocephalus?  The causes of hydrocephalus are still not well understood. Hydrocephalus may result from inherited genetic abnormalities (such as the genetic defect that causes aqueductal stenosis) or developmental disorders (such as those associated with neural tube defects including spina bifida and encephalocele). Other possible causes include complications of premature birth such as intraventricular hemorrhage, diseases such as meningitis, tumors, traumatic head injury, or subarachnoid hemorrhage, which block the exit of CSF from the ventricles to the cisterns or eliminate the passageway for CSF into the cisterns.
  • 11. #Symptoms of hydrocephalus:  Symptoms of hydrocephalus vary with age, disease progression, and individual differences in tolerance to the condition. For example, an infant's ability to compensate for increased CSF pressure and enlargement of the ventricles differs from an adult's. The infant skull can expand to accommodate the buildup of CSF because the sutures (the fibrous joints that connect the bones of the skull) have not yet closed.
  • 12.  In infancy, the most obvious indication of hydrocephalus is often a rapid increase in head circumference or an unusually large head size. Other symptoms may include vomiting, sleepiness, irritability, downward deviation of the eyes (also called "sunsetting"), and seizures.
  • 13.  Older children and adults may experience different symptoms because their skulls cannot expand to accommodate the buildup of CSF. Symptoms may include headache followed by vomiting, nausea, papilledema (swelling of the optic disk which is part of the optic nerve), blurred or double vision, sunsetting of the eyes, problems with balance, poor coordination, gait disturbance, urinary incontinence, slowing or loss of developmental progress, lethargy, drowsiness, irritability, or other changes in personality or cognition including memory loss.
  • 14.  Symptoms of normal pressure hydrocephalus include, problems with walking, impaired bladder control leading to urinary frequency and/or incontinence, and progressive mental impairment and dementia. An individual with this type of hydrocephalus may have a general slowing of movements or may complain that his or her feet feel "stuck." Because some of these symptoms may also be experienced in other disorders such as Alzheimer's disease, Parkinson's disease, and Creutzfeldt-Jakob disease, normal pressure hydrocephalus is often incorrectly diagnosed and never properly treated
  • 15.  Doctors may use a variety of tests, including brain scans (CT and/or MRI), a spinal tap or lumbar catheter, intracranial pressure monitoring, and neuropsychological tests, to help them accurately diagnose normal pressure hydrocephalus and rule out any other conditions.
  • 16.  The symptoms described in this section account for the most typical ways in which progressive hydrocephalus manifests itself, but it is important to remember that symptoms vary significantly from one person to the next.
  • 17. Diagnosis:  Hydrocephalus is diagnosed through clinical neurological evaluation and by using cranial imaging techniques such as ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), or pressure-monitoring techniques. A physician selects the appropriate diagnostic tool based on an individual's age, clinical presentation, and the presence of known or suspected abnormalities of the brain or spinal cord.
  • 19.
  • 20.
  • 21.
  • 25. Treatment:  Hydrocephalus is most often treated by surgically inserting a shunt system. This system diverts the flow of CSF from the CNS to another area of the body where it can be absorbed as part of the normal circulatory process.  A shunt is a flexible but sturdy plastic tube. A shunt system consists of the shunt, a catheter, and a valve. One end of the catheter is placed within a ventricle inside the brain or in the CSF outside the spinal cord. The other end of the catheter is commonly placed within the abdominal cavity, but may also be placed at other sites in the body such as a chamber of the heart or areas around the lung where the CSF can drain and be absorbed. A valve located along the catheter maintains one-way flow and regulates the rate of CSF flow.
  • 26.
  • 27.  A limited number of individuals can be treated with an alternative procedure called third ventriculostomy. In this procedure, a neuroendoscope — a small camera that uses fiber optic technology to visualize small and difficult to reach surgical areas — allows a doctor to view the ventricular surface. Once the scope is guided into position, a small tool makes a tiny hole in the floor of the third ventricle, which allows the CSF to bypass the obstruction and flow toward the site of resorption around the surface of the brain.
  • 29. complications of a shunt system  Shunt systems are not perfect devices. Complications may include mechanical failure, infections, obstructions, and the need to lengthen or replace the catheter. Generally, shunt systems require monitoring and regular medical follow up. When complications occur, the shunt system usually requires some type of revision.  Some complications can lead to other problems such as overdraining or underdraining. Overdraining occurs when the shunt allows CSF to drain from the ventricles more quickly than it is produced. Overdraining can cause the ventricles to collapse, tearing blood vessels and causing headache, hemorrhage (subdural hematoma), or slit-like ventricles (slit ventricle syndrome).
  • 30.  Underdraining occurs when CSF is not removed quickly enough and the symptoms of hydrocephalus recur. In addition to the common symptoms of hydrocephalus, infections from a shunt may also produce symptoms such as a low-grade fever, soreness of the neck or shoulder muscles, and redness or tenderness along the shunt tract. When there is reason to suspect that a shunt system is not functioning properly (for example, if the symptoms of hydrocephalus return), medical attention should be sought immediately.
  • 31. Prognosis:  The prognosis for individuals diagnosed with hydrocephalus is difficult to predict, although there is some correlation between the specific cause of the hydrocephalus and the outcome. Prognosis is further complicated by the presence of associated disorders, the timeliness of diagnosis, and the success of treatment. The degree to which relief of CSF pressure following shunt surgery can minimize or reverse damage to the brain is not well understood.
  • 32.  Affected individuals and their families should be aware that hydrocephalus poses risks to both cognitive and physical development. However, many children diagnosed with the disorder benefit from rehabilitation therapies and educational interventions and go on to lead normal lives with few limitations. Treatment by an interdisciplinary team of medical professionals, rehabilitation specialists, and educational experts is critical to a positive outcome. Left untreated, progressive hydrocephalus may be fatal.
  • 33.  The symptoms of normal pressure hydrocephalus usually get worse over time if the condition is not treated, although some people may experience temporary improvements. While the success of treatment with shunts varies from person to person, some people recover almost completely after treatment and have a good quality of life. Early diagnosis and treatment improves the chance of a good recovery.
  • 34. Research:  The National Institute of Neurological Disorders and Stroke (NINDS) and other institutes of the National Institutes of Health (NIH) conduct research related to hydrocephalus in laboratories and clinics at the NIH and support additional research through grants to major medical institutions across the country. Much of this research focuses on finding better ways to prevent, treat, and ultimately cure disorders such as hydrocephalus. The NINDS also conducts and supports a wide range of fundamental studies that explore the complex mechanisms of normal and abnormal brain development.