Hydrocephalus

• The term hydrocephalus is derived

from the Greek words "hydro"
meaning water and "cephalus"
meaning head.
• Hydrocephalus results from an
imbalance between production and
absorption of CSF.

• The balance between production and

absorption of CSF is critically
important.
• When production is more than
absorption, CSF accumulates within
the ventricular system producing the
dilation of the spaces in the brain
called ventricles.

• The ventricular system is made up of

four ventricles connected by narrow
pathways.
• 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 is absorbed into the
bloodstream.
• Ideally, the fluid is almost completely
absorbed into the bloodstream as it
circulates; however, there are
circumstances which, when present,
will prevent or disturb the production
or absorption of CSF, or which will
inhibit its normal flow. When this
balance is disturbed, hydrocephalus
is the result.
 Flow of CSF
• lateral ventricles-->
foramen of Monro third
ventricle --> aqueduct
of Sylvius --> fourth
ventricle --> foramina of
Magendie and Luschka
--> subarachnoid space
over brain and spinal
cord --> reabsorption
into venous sinus blood
via arachnoid
granulations.
Imbalance excessive accumulation of CSF

Hydrocephalus

- Excessive production
- Decreased absorbtion
- Obstruction
 Incidence
• Occurs in 3 - 4 cases in every 1000
births.
 Types of hydrocephalus

1.Communicating and Non-communicating

2.Acute and chronic

3.Congenital and acquired

Communicating hydrocephalus
• There is no blockage between the
ventricular system, the basal cisterns
and the spinal subarachnoid space.

• Failure in the absorption system-

unknown cause
• Excessive production of CSF- tumor
or unknown cause
 Obstructive or
Non-Communicating
hydrocephalus
• The block is at any level in the
ventricular system (source of
production and area of its
reabsorption), commonly at the level
of the aqueduct or foramina of
Luschka and Magendie.
1. Acute : Develops within days or few
weeks
- Manifests with rapid progression of
symptoms - Requires early attention and
treatment
hydrocephalus caused by tumor
2. Chronic : Over months (or even years)
- Subtle signs of memory impairment,
walking
difficulty, urinary incontinence
- A classic example is Normal Pressure
Chronic hydrocephalus can present acutely
because of changes in the pathophysiology
of the CSF absorption or flow.
 Con….
1. Congenital : Present at birth or few
weeks/months after birth (1-2/1000 live
births)
 Intrauterine infections ( rubella, cytomegalovirus,
toxoplasmosis),
 Intracranial & intraventricular bleed.
 Midline tumors of the brain
 Congenital malformations
2. Acquired
– Infection (post-meningitis)
– Post – hemorrhagic (SAH,IVH)
– Tumors
• There are two other forms of
hydrocephalus which do not fit
distinctly into the categories
mentioned above and primarily
affect adults:
1. Hydrocephalus ex-vacuo and
2. Normal pressure hydrocephalus.
• Hydrocephalus ex-vacuo:
Hydrocephalus ex-vacuo occurs
when there is damage to the brain
caused by stroke or traumatic injury.
In these cases, there may be actual
shrinkage (atrophy or wasting) of
brain tissue. In cerebral atrophy
ventricles are dilated but pressure is
not raised .
• Normal pressure hydrocephalus.
Normal pressure hydrocephalus
commonly occurs in the elderly and
is characterized by memory loss,
dementia, gait disorder, urinary
incontinence, and a general slowing
of activity.
 Pathophysiology
• The ventricles become greatly
distended

• The increased ventricular pressure

results in thinning of the cerebral
cortex and cranial bones especially
frontal, parietal and temporal areas.
• The floor of the third ventricle commonly
bulges downward, compress the optic
nerves

• The basal ganglia, brain stem and

cerebellum remain relatively normal but
compressed

• The choroid plexus is usually atrophied to

some degree.
CLINICAL MANIFESTATIONS
Due to increased ICP and dilation of ventricles,
causing compression of the adjacent brain
In neonatal period
• Skull - thin and relatively non rigid allows for an
overall cranial expansion
• Craniofacial disproportion
• Irritable Fussy
• May not accept feeds Vomiting
• Poor head control Lethargy
• Drowsiness
• In extreme cases, lapse into a comatose state
Cont….

- Fontanel full , bulging and wide

- Thin and glistening scalp with enlargement
and engorgement of scalp veins
- Macewen's sign ( cracked pot sound on head
percussion)
- Sixth nerve (abducens) palsy
- Setting sun sign - upward gaze palsy
- Hyperactive reflexes.
- Irregular respiration.
- Separation of cranial sutures (sutures
diastasis)
 In older children and adults

The enlarging ventricles result in raised ICP and

compression of the adjacent brain

2 common modes of presentation

a) rapidly progressive hydrocephalus
b) chronic hydrocephalus.
Rapidly progressive hydrocephalus
Increased ICP - new-onset headache and vomiting

If untreated, these symptoms worsen and blurring of vision

often occurs.

In patients with long-standing raised pressure, papilledema

can result

If still untreated, drowsiness and progression to coma

follow.
Chronic hydrocephalus

- CSF accumulates more slowly - gradually

compressing the brain

- Predominantly seen in older adults

- The patient becomes progressively dull,

apathetic, and uninvolved with the
surroundings.

- Memory impairment for recent events is

commonly seen, but usually the remote
memory is well preserved.
- Short stepped gait with a wide stance and unsteadiness

- Urinary incontinence

- No significant headache

Cerebellar fits or hydrocephalic attacks:

(severe headache, patient lapses into sudden
unconsciousness associated with a decerebrate
or decorticate response, downward deviation of
the eyeballs, and respiratory distress)
Medical emergency
 Diagnostic evaluation
• Physical examination infant’s head
transilluminates, Macewen’s sign
• Ophthalmoscopy
• CT scan/ MRI of the head
• Skull x-ray
• Ultrasonography
• Lumber puncture
 Management
The ultimate goal is to reverse the neurologic
damage caused by the raised ICP

Medical treatment - not proved to be useful

Used as a temporary measure and in
conjunction with surgical management.

Acetazolamide - Commonly used - reduce CSF

production.
However, benefits are minimal and high doses
of the drug, which cause metabolic acidosis,
are required to achieve the effect.
Surgical

 Involves diversion of the accumulated CSF

(1) by reopening the obstruction to allow the CSF
to flow into its natural pathway

(2) by creating a diversion before the obstruction

to allow the CSF to drain into the intracranial
pathways distal to the block

(3) by diversion of the CSF into another cavity so

it becomes absorbed into the bloodstream.

 Removal of obstruction like posterior fossa tumor

Although shunts have been the mainstay of
treatment for several decades, endoscopic
procedures have now become more popular.

These include:
i) Endoscopic third ventriculostomy- into the

basal cisterns
ii) Endoscopic aqueductoplasty – 3 forgarty
catheter
iii) Endoscopic aqueductal stenting.
CSF diversion procedure :
- Children <5 years : difficult to assess intellectual
development
- Protects against the effects of persistent ventriculomegaly

and ensures an optimal environment for future intellectual

development

- >5 years and adults with asymptomatic ventriculomegaly

often are closely watched, with frequent assessment
of intellectual development, before considering a shunt
insertion.
Cerebrospinal Fluid Shunts

-Ventriculo-peritoneal(VP) - most common

-Ventriculo-atrial(VA)
-Torkildsen shunt (in aqueduct stenosis by passing a
catheter from the lateral ventricles into the cisternal
space)
-Lumbo-peritonial shunt.
-External drainage – temporary
 Ventriculoperitoneal (VP)
Shunt

Journal of NeurosurgeryPediatrics
Mayo Foundation for Medical Education and Research
 Signs & Symptoms of
Shunt Malfunction
• Same as hydrocephalus
• But also signs of infection
– Fever
– Swelling
– Redness
– Drainage
 Preventative Medicine
• Education
• Routine Clinic Follow-up
• Surveillance Imaging
– Ultrasound
– CT scan
– MRI
 Complications
• 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 do occur and due to
growth of child, usually the shunt system
will require some type of revision.
• Child with V-A shunt may experience
endocardial contusion, clotting,
leading to bacterial endocarditis,
bacteremia, thromboembolism.
 Nursing diagnosis
• Altered cerebral tissue perfusion r/t
raised ICP
• Altered nutrition ( less ) r/t reduced
oral intake and vomiting
• Risk of impaired skin integrity r/t
alteration in level of conciousness
and enlarged head
• Anxiety r/t prognosis,surgery
• Risk for fluid volume deficit r/t CSF
drainage, decreased intake
postoperatively
• Risk for injury r/t malfunctioning
shunt, infection
• Ineffective family coping r/t
diagnosis and surgery