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Hydrocephalus
1. HYDROCEPHALUS
P R E S E N T E D BY
A N I T H A M A R Y M AT H E W
&
J E S N A J O Y
F R O M G R O U P N O : - 7
FA C I L I T Y O F M E D I C I N E 5 T H Y E A R 2 N D S E M E S T E R
2. INTRODUCTION
• Hydrocephalus can be defined broadly as a disturbance of
cerebrospinal fluid (CSF) formation, flow, or absorption,
leading to an increase in volume occupied by this fluid in the
central nervous system (CNS).
• This condition could also be termed a hydrodynamic CSF
disorder
• In hydrocephalus, the build-up of CSF can raise pressure
inside the skull, which squashes surrounding brain tissue.
• Cerebrospinal fluid normally flows through the ventricles and
bathes the brain and spinal column.
• In some cases, this can cause the head to steadily grow in
size, convulsions, and brain damage.
• Hydrocephalus can be fatal if left untreated.
3. EPIDEMIOLOGY
• The incidence of congenital hydrocephalus is 3 per 1,000 live births
• Generally, incidence is equal in males and females.
• The exception is Bickers-Adams syndrome (X-linked hydrocephalus with stenosis of
aqueduct of Sylvius), transmitted by females and manifested in males.
• NPH has a slight male preponderance.
• Incidence of human hydrocephalus presents a bimodal age curve. One peak occurs in
infancy and is related to the various forms of congenital malformations and premature
birth . Another peak occurs in adulthood, mostly resulting from NPH.
• Adult hydrocephalus represents approximately 40% of total cases of hydrocephalus.
• In untreated hydrocephalus, death may occur by tonsillar herniation secondary to
raised ICP with compression of the brain stem and subsequent respiratory arrest.
4. CLASSIFICATION
Congenital hydrocephalush
Approximately 1 in every 500 American babies are born with hydrocephalus. It
may be caused by an infection in the mother during pregnancy, such as rubella or
mumps, or a birth defect, such as spina bifida. It is one of the most common
developmental disabilities, more common than Down syndrome or deafness.
Acquired hydrocephalus
This develops after birth, usually after a stroke, brain tumor, meningitis, or as a
result of a serious head injury.
Communicating hydrocephalus
This type of hydrocephalus occurs when the CSF becomes blocked after leaving
the ventricles. It is called “communicating” because CSF can still flow between
the brain’s ventricles.
5. Non-communicating hydrocephalus
Also called obstructive hydrocephalus, non-communicating hydrocephalus occurs when
the thin connections between the ventricles become blocked.
Normal pressure hydrocephalus
This only affects people aged 50 or older. It may develop after stroke, injury, infection,
surgery, or hemorrhage. However, in many cases, doctors do not know why it occurred.
An estimated 375,000 older adults in America have normal pressure hydrocephalus.
Hydrocephalus ex-vacuo
This type occurs after stroke, traumatic brain injury, or degenerative diseases. As brain
tissue shrinks, the ventricles of the brain become larger.
6. ETIOLOGY
Too much CSF is produced.
One of the ventricles in the brain is blocked or narrowed, stopping or restricting
the flow of CSF, so that it cannot leave the brain.
CSF cannot filter into the bloodstream
Causes of congenital hydrocephalus (present at birth)
The baby is born with a blockage in the cerebral aqueduct
The choroid plexus produces too much CSF.
Infections during pregnancy – these can affect the development of the baby’s brain
Dandy-Walker malformation.
Arnold-Chiari malformation type 1 and type 2.
Bickers-Adams syndrome: This is an X-linked hydrocephalus
7. Causes of acquired hydrocephalus
Brain hemorrhage
Brain lesions
Brain tumors
Meningitis
Stroke
Causes of normal pressure hydrocephalus
There are two theories:
• CSF is not reabsorbed into the bloodstream properly. Because of this, the brain starts
to produce less new CSF, resulting in a gradual rise in pressure over a long period.
The gradual rise in pressure may cause progressive brain damage.
• An underlying condition, such as heart disease, high blood cholesterol level, or
diabetes affects normal blood flow, which may lead to a softening of brain tissue. The
softened brain tissue results in increasing pressure
8. PATHOPHYSIOLOGY
• CSF can accumulate within the ventricles, this condition is called
internal hydrocephalus and may result in increased CSF pressure.
• The production of CSF continues, even when the passages that
normally allow it to exit the brain are blocked.
• Consequently, fluid builds inside the brain, causing pressure that
dilates the ventricles and compresses the nervous tissue.
• Compression of the nervous tissue usually results in irreversible
brain damage
• If the skull bones are not completely ossified when the
hydrocephalus occurs, the pressure may also severely enlarge the
head.
• The cerebral aqueduct may be blocked at the time of birth or may
become blocked later in life because of a tumor growing in
the brainstem
9. SIGNS AND SYMPTOMS
Common signs and symptoms of hydrocephalus in infants include:
• 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
• Deficits in muscle tone and strength
10. Toddlers and older children
• 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 incontinvence
• Behavioral and cognitive changes
• Stunted growth and sexual maturation from third ventricle dilatation: This can lead to obesity and to
precocious puberty or delayed onset of puberty.
11. Young and middle-aged adults
• 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
• 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
12. Symptoms of NPH
• Gait disturbance is usually the first symptom
• Dementia should be a late finding in pure (shunt-responsive) NPH.
• Urinary incontinence may present as urgency, frequency, or a diminished awareness of
the need to urinate.
• Other symptoms that can occur include personality changes and Parkinsonism.
13. PHYSICAL EXAMINATION
• Head enlargement
• Disjunction/splaying of sutures
• Dilated scalp veins
• Tense/bulging fontanelle
• Setting-sun sign: Characteristic of increased intracranial pressure.
• Macewen sign: A "cracked pot" sound is noted on percussion of the head.
• Frontal release signs such as sucking and grasping reflexes appear in late stages of
NPH
• Increased limb tone
• Papilledema
• Failure of upward gaze
• Unsteady gait
• Unilateral or bilateral sixth nerve palsy (secondary to increased ICP), secondary to
increased ICP
14. DIAGNOSIS
Imaging studies
Computed tomography (CT) scanning: To assess size of ventricles and other
structures
Magnetic resonance imaging (MRI): To assess for Chiari malformation or cerebellar
or periaqueductal tumors
Ultrasonography through anterior fontanelle in infants: To assess for
subependymal and intraventricular hemorrhage
Skull radiography: To detect erosion of sella turcica
MRI cine: To measure CSF stroke volume
Diffusion tensor imaging (DTI)
Radionuclide cisternography
18. TREATMENT
Treatments for congenital and acquired hydrocephalus
• Both types of hydrocephalus require urgent treatment to reduce the pressure on the
brain.
• A shunt is the surgical insertion of a drainage system. A catheter (a thin tube with a
valve) is placed in the brain to drain away excess fluid from the brain into another part
of the body, such as the abdomen, the chest cavity, or a chamber of the heart. Usually,
this is all that is needed, and no further treatment is required.
• Patients with hydrocephalus will usually need to have a shunt system in place for the
rest of their lives. If the shunt is placed in a child, additional surgeries may be needed
to insert longer tubing as they grow.
• Ventriculostomy – the surgeon makes a hole in the bottom of a ventricle so that the
excess fluid flows towards the base of the brain. Normal absorption occurs at the base
of the brain. This procedure is sometimes performed when the flow of fluids between
ventricles is obstructed.
19. Treatment for normal pressure hydrocephalus
• Shunts may also be used for normal pressure hydrocephalus. However, shunts may
not be suitable for some patients. Other procedures may be carried out to check
suitability:
• Lumbar puncture – some of the cerebrospinal fluid is removed from the base of the
spine. If this improves the patient’s gait or mental abilities, fitting a shunt will probably
help.
• Lumbar infusion test – a needle is inserted through the skin of the lower back into the
spine. Measurements are taken of CSF pressure as fluid is injected into the spine.
Patients usually benefit from having a shunt fitted if their CSF pressure is over a
certain limit.
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22. COMPLICATIONS
Related to progression of hydrocephalus
• Visual changes
• Cognitive dysfunction
• Incontinence
• Gait changes
Related to medical treatment
• Electrolyte imbalance
• Metabolic acidosis
23. • Related to surgical treatment
• VP shunt complications include:- peritonitis, inguinal hernia, perforation of
abdominal organs, intestinal obstruction, volvulus, and CSF ascites.
• Ventriculoatrial (VA) shunt complications include:- septicemia, shunt embolus,
endocarditis, and pulmonary hypertension.
• Lumboperitoneal shunt complications include:- radiculopathy and arachnoiditis.
