HYDROCEPHALUS
Definition
-Hydrocephalus is the abnormal rise in cerebrospinal fluid
volume with ventricular dilatation due to imbalance of
cerebrospinal fluid production and absorption.
It is usually accompanied by increased intracranial pressure.
Hydrocephalus is a diverse group of conditions, which result
from;
1) Impaired circulation of CSF
2) Impaired absorption of CSF
3) In the rare circumstance, from increased production by
a choroid plexus papilloma
The most common causes of hydrocephalus in acquired cases
are tumor obstruction, trauma, intracranial hemorrhage, and
infection.
Pathophysiology
Flow of CSF
CSF is produced in the lateral ventricle choroid plexus at
30mL/hr (20mL/hr in infants)
Foramen of Monro ↓
3rd Ventricle
Cerebral aqueduct of sylvius ↓
4th Ventricle
Foramen of Lushka(2-lateral)↓ magendie foramen
Subarachnoid basal cisterns posteriorly over the cerebellum and
cerebral cortex, and anteriorly through the cistern system and
over the convexities of the cerebral hemispheres.
arachnoid granulations ↓
superior sagittal sinus
↓ ventricular system, and fluid within the ventricles
Venous circulation
− These openings lead to a system of interconnecting and
focally enlarged areas of subarachnoid spaces referred to
as cisterns.
− The cisterns in the posterior fossa connect through
pathways that traverse the tentorium to the subarachnoid
spaces over the cerebral convexities.
− The spinal subarachnoid space communicates with the
intracranial subarachnoid space by the basal cisterns
NB. Main CSF production is from the lateral ventricles ,some
from the 4th ventricle. 25% CSF is produced from the ependymal
lining of the ventricles.
Infants –volume of CSF 50mls
Adults – volume of CSF 125-150mls.
Daily production of CSF 30ml/hr up to 720 ml/day
TYPES OF HYDROCEPHALUS
a) Obstructive / Non-communicating hydrocephalus
Hydrocephalus resulting from obstruction within the ventricular
system .Proximal dilatation from obstruction.
− In noncommunicating hydrocephalus the ventricular fluid
does not communicate with CSF in the spinal subarachnoid
spaces or in the basal cisterns.
− This implies a block of CSF flow within the ventricular
system, such as at the foramen of Monro, the aqueduct of
Sylvius, or the fourth ventricle and its outlets.
1. An abnormality of the Aqueduct:
Stenosis; Congenital - inherited as a sex-linked recessive trait
,Acquired in Neurofibromatosis
Gliosis; Intrauterine viral infections, SAH in a premature infant,
Neonatal meningitis,Mumps meningoencephalitis
2.Lesions in the fourth ventricle:
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Chiari malformation
 Type II is characterized by progressive hydrocephalus and
a myelomeningocele which results in elongation of the
fourth ventricle and kinking of the brain stem, with
displacement of the inferior vermis, pons, and medulla into
the cervical canal.
 Produce symptoms during infancy consisting of stridor,
weak cry, and apnea, which may be relieved by shunting
or by posterior fossa decompression.
 Type I produces symptoms during adolescence or adult life
and is usually not associated with hydrocephalus.
 These patients complain of recurrent headache, neck pain,
urinary frequency, and progressive lower extremity
spasticity. The deformity consists of tonsilar herniation.
Dandy-Walker syndrome
 Consists of a cystic expansion of the fourth ventricle in the
posterior fossa, which results from a developmental failure
of the roof of the 4th ventricle during embryogenesis.
 Posterior fossa brain tumors- Meduloblastomas,
hemangiomas, epindymomas.
3.Space occupying lesions may obstruct the flow along
anyway along the ventricular system-Tumors, bleeds etc
b) Non-obstructive / Communicating hydrocephalus -
Hydrocephalus resulting from obliteration of the
subarachnoid cisterns or malfunction of the arachnoid
granulations.
− In communicating hydrocephalus, the block is outside the
communicates with the spinal subarachnoid space and
basal cisterns.
1.Infections;
-Intrauterine infections may destroy the CSF pathways. ----
Pneumococcal and TB meningitis have a propensity to
produce a thick, tenacious exudate that obstructs the basal
cisterns
2.SAH
Blood in the subarachnoid spaces may cause obliteration of the
cisterns or arachnoid villi, and obstruction of CSF flow.
3.Leukemic infiltrates
May seed the subarachnoid space and produce communicating
hydrocephalus
4. Superior saggital sinus thrombosis
This impairs venous drainage and impairs re-absorption through
the arachnoid villi.
5. Overproduction of CSF-choroid plexus papilloma
c) Normal-Pressure Hydrocephalus
Cerebral ventricular dilation with normal lumbar CSF pressure
(5-18cmH2O)
Aetiology – Same as Non-Obstructive or communicating
hydrocephalus above
Presentation- Triad of normal pressure hydrocephalus.
-Dementia - rare cause of dementia in the elderly
-Apraxia of gait - initiation of gait is hesitant - described as a
"slipping clutch" or "feet stuck to the floor" gait - and walking
eventually occurs. Motor weakness and staggering are absent
-Urinary incontinence
Investigations
CSF pressure is normal
CT/MRI - the ventricles are dilated
Mx;
Shunting - Brief improvement after removing about 50mL of
CSF indicates a better prognosis with shunting but the longer
1
the disease has been present, the less likely shunting will be pressure exerted on the hypothalamus by the dilated third
curative ventricle.

d)Ex Vacuo Hydrocephalus DDx

Enlarged ventricles resulting from a loss of brain tissue The head may appear enlarged secondary to a thickened
Other terms cranium resulting from;
-Arrested hydrocephalus-the ventricles dilated but the pressures 1) Familial-autosomal dominant
not increasing. Seen more in children 2) Chronic anemia eg Haemoglobinopathies, SCD,
-Occult hydrocephalus-dilated ventricles but no signs of raised Thalassaemia
ICP or suggestive of hydrocephalus. 3) Rickets
4) Achondroplasia
Composition of CSF-Clear fluid,15-45 mg/ml of protein 5) Osteogenesis imperfecta
-60-80mg/ml of glucose,3 lymphocytes or fewer 6) Epiphyseal dysplasia
7) Neurofibromatosis
Presenting complaints 8) Storage diseases-Gaucher’s disease, Tay sachs
Infants; sutures still open disease.
History
1.Accelerated rate of enlargement of the head is the most Investigations in infants
prominent sign 1.Skull X-ray – Not very revealing but may show
2.Irritability, lethargy, poor appetite, and vomiting -Separation of the sutures
3.Delayed milestones -Erosion of the posterior clinoids in the older child
4-Failure to thrive -Increase in convolutional markings ("beaten-silver
5.Family history appearance") with longstanding increased intracranial pressure.
6.Premature delivery -Flattening of gyri and obliteration of sulci
7.Previous history of treatment for meningitis -Herniations-tentorial and through foramen magnum
8.Maternal infections and drug intake in prenatal period. 2.Ultrasonography
Through the open anterior fontanele.
Physical examination 3.CT-scan
1. Enlarged head for the age. At birth head circumference is Show ventricular dilatation
35cm +/- 2cm.this increases by: 4.MRI
 2cm per month for first 3 months
 1cm per month next 3 months Adults
 ½ cm per month for next 6months Clinical presentation
 ¼ cm per month from 1year to 3 years old. Mainly signs of increased intracranial pressure.
The occipitofrontal head circumference is recorded and kept for Signs /Symptoms of increased ICP
monitoring the head progression. Symptoms
2-Scalp veins are dilated and congested 1) -Severe bursting headache
3-The forehead is broad 2) -Projectile vomiting
4-Anterior fontanel is wide open and bulging 3) -Blurring of vision
5-Percussion of the skull may produce a "cracked-pot" or 4) -Convulsions/seizures
Macewen sign, indicating separation of the sutures 5) –Drowsiness and altered consciousness.
6-Craniotabes Signs
7-A foreshortened occiput suggests the Chiari malformation, 1) -Vital signs-increased BP and decreased pulse rate
and a prominent occiput suggests the Dandy-Walker (cushings reflex)
malformation. 2) -Anisocoria-unequal pupils
8-Dilatation of the 3rd ventricle → dilatation of the suprapineal 3) -Papilloedema on fundoscopy
recess which then impinges on the tectum → eyes deviate 4) -Nerve palsy eg 3rd and 6th cranial nerves
downward - "setting-sun" eye sign. 5) -Irregular breathing/slowed fats
− Upward gaze may be impaired because of pressure on the
midbrain, and the sclera above the iris will be visible. This is MANAGEMENT
known as the setting-sun sign. Aims
Also lid lag 1. Reduce the intracranial pressure-Medical treatment or CSF
9.Long tract signs-due to stretching and compression of cortico- diversion
spinal tract eg brisk tendon reflexes, spasticity, clonus in lower 2.Remove the underlying cause-Definitive surgery
extremities, babinski reflex up going
− Spasticity in the extremities, especially the legs, may Medical treatment
develop as the fibers from the cortical motor areas are - When patients have slowly progressive hydrocephalus with
stretched around the bodies of the dilated ventricles in their few symptoms or signs, and when the condition of the patient
course to the cerebral peduncles. has prohibited surgery.
10. Transillumination of the skull is positive with massive -Medical therapy usually is a temporizing measure. In transient
dilatation of the ventricular system or in the Dandy-Walker conditions, such as sinus occlusion, meningitis, or neonatal
syndrome. intraventricular hemorrhage, medical therapy can be effective.
11.Fundoscopy-Papilloedema is observed in older children but - Acetazolamide (25 mg/kg/d in 3 doses): Careful monitoring of
is rarely present in infants because the cranial sutures separate respiratory status and electrolytes is crucial. Treatment beyond
as a result of the increased pressure 6 months is not recommended.
-In the older child, the cranial sutures are partially closed so that - Furosemide (1 mg/kg/d in 3 doses): Again, electrolyte balance
the signs of hydrocephalus may be more subtle. The signs of and fluid balance need to be monitored carefully.
increased ICP become more prominent. - Lumbar punctures: In neonates recovering from
− Disturbances in growth, accelerated pubertal development, intraventricular hemorrhage, serial lumbar punctures can
and fluid and electrolyte homeostasis may develop from

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resolve hydrocephalus in some cases. If possible, this is the
preferred method of treatment.
- Removal of the underlying cause resolves hydrocephalus in
most cases
SURGERY
CSF DIVERSION
Short-term(temporary)
-Acute intraventricular bleeds, subarachnoid bleeds, infections.
There is high protein concentration in the CSF which would
cause coagulum and block the valve.
-Do external ventricular drains until blood is cleared from the
CSF. And the infection has settled.
-Exteriorization of the shunt can also be used for the
administration of antibodies.
-Depend on the cause of the dilated ventricles and not on the
size of the cortical mantle at the time of the shunting.
 Increased risk of developmental problems
 Mean intelligence quotient is reduced compared to
general population
 Risk of aggressive and delinquent behaviour
 Accelerated pubertal development in patients with
shunted hydrocepahalus or meningomyelocele due to
increased gonadotropins due to increased ICP

Permanent CSF diversion

1.Ventriculoperitoneal shunt
2.Ventriculo-artrial Shunt
3.Ventriculopleaural shunt

Ventriculoperitoneal shunt
A shunt is placed from the lateral ventricle to the peritoneum
Procedure
-Standard pre-operative preparation
-Patient in supine position
-Clean and drape the right parietal, neck, chest and abdomen.
Right side of the head to be away from the speech areas-
Brocas and Wernickes left in most people.
-Do right parietal burr hole
-Right subcostal minilaparatomy
-Subcuticular tunneling
-Insertion of the shunt system.
-Cannulation of lateral ventricle and confirm that the shunt is
functioning
-Standard wound closure.

Complications;
1-Bacterial infection, usually due to Staphylococcus epidermidis
Other organisms found less frequently include S. aureus, enteric
bacteria, diphtheroids, and Streptococcus species. Infection
must be suspected in any child with a shunt who develops an
unusual or persistent febrile illness.
2-Shunt blockade-mechanical kinks, choroid plexus block,
omentum
3-Shunt disconnection-from the drainage of the lateral ventricle.
4-Shunt migration-can migrate downward the peritoneum
5-Subdural bleed due to rapid decompression seen with
patients who have had sutural closure.
Another complication of ventriculoatrial shunts is pulmonary
hypertension owing to chronic microembolism from thrombi
formed on the atrial catheter.

− Recently, third ventriculostomies (Establishment of an

opening in a ventricle, usually through the floor of the third
ventricle to the subarachnoid space to relieve
hydrocephalus) have been used to treat some forms of
hydrocephalus and have been performed instead of shunt
revisions.
− Young infants are often not candidates for this procedure.
− If the shunt system becomes disconnected or the catheters
become obstructed, symptoms will recur if the
hydrocephalus is still active.

DEFINITIVE SURGERY
-Operation for tumors
-Appropriate craniotomy dependent on the location of the tumor.

Prognosis

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