HYDROCEPHALUS

Alka Sara Saju

II year MSc Nursing
Overview
• Review of anatomy
• Definition of hydrocephalus
• Incidence
• Etiology
• Classification‘
• Pathophysiology
• Signs and symptoms
• Management
• Nursing care
• Quiz
Anatomy
• Bony skull forms the strongest covering & provides
primary protection to brain

• It is an expansible structure in infants and small

children due to incomplete ossification of skull
bones

• Rigid in older children and adolescents

Meninges
 Brain
Parts of brain

1. The cerebrum
cerebral cortex - outermost layer of brain cells. (Thinking
and voluntary movements.)

2. The brain stem is between the spinal cord and the rest of
the brain. Basic functions like breathing and sleep are
controlled here.

•Medulla: sensory & motor tracts ,breathing &

cardiovascular centers, regulate consciousness & arousal
•Pons: sensory & motor tracts,
•Midbrain : sensory & motor tracts, movements of head
,eyes & trunk, response to visual stimuli.
3. The cerebellum is at the base and the back of the
brain. The cerebellum is responsible for coordination
and balance.

4. Diencephalon :
• Thalamus: helps regulate motor fuction &
maintenance of consciousness
• Hypothalamus: releasing hormones & control of
ANS
• Epithalamus: pineal gland (secrete melatonin)
Functional organization of cerebral cortex
• Frontal lobes are responsible for problem
solving and judgment and motor function.
• Parietal lobes manage sensation,
handwriting, and body position.
• Temporal lobes are involved with memory
and hearing.
• Occipital lobes contain the brain's visual
processing system
 Blood supply
• Internal carotid artery
– branches into various
brain segments
• Volume of blood to
brain constitute 17% of
cardiac output and
supplies brain with
20% oxygen
Cranial nerves - 12
• The olfactory nerve (I) - Smell

• The optic nerve (II) - Vision

• Oculomotor nerve (III) - Eye movement

• Trochlear nerve (IV) - Eye movement

• Trigeminal nerve (V) - Combined, these nerves provide

sensation to the skin of the face and also controls the
muscles of mastication (chewing).
• Abducens nerve (VI) _ Eye movement

• Facial nerve (VII) - Facial expression

• Vestibulocochlear nerve (VIII) - Hearing and balance

• glossopharyngeal nerve (IX) - Oral sensation, taste, and

salivation

• Vagus nerve (X) - swallowing

• Accessory nerve(xi) - Shoulder elevation and head-

turning

• Hypoglossal nerve (XII) - Tongue movement

 Cerebrospinal fluid
Origin of CSF:

• CSF is an ultrafiltrate of plasma that enters the basal side of

the choroid epithelium and by active metabolism it is
transformed into CSF and secreted at the apical or
ventricular side of the epithelium

• Most CSF is formed in the ventricular system. Possible sites

of origin include the choroid plexus (~ 80%), the
parenchyma (~20%), and the ependyma (negligible).
Function of CSF
• Clear colourless fluid
• Protects brain & spinal cord from physical &
chemical injuries
• Carries oxygen & glucose from blood to neurons
• The formation rate of CSF to be about 20 ml/hour
or 500 ml/day in adults and children.

• The total CSF volume in the ventricles and

subarachnoid space is age-dependent but reaches
the adult volume of 150 ml by age 5 years

• Intracranial pressure
• Infants : 5 – 10 mm Hg
• Children : 15 mm Hg
CSF CIRCULATION
Source :http://adameducation.com/adam_images
sourcehttp://adameducation.com/adam_images
Hydrocephalus
Hydrocephalus

• Greek
• “Hydro” – water
• “Cephalus” - head

A Condition resulting from imbalance in

CSF production and absorption
Incidence
• 3 / 1000 live births

• Occurs in 80-85% children born with

meningomyelocele
• Incidence is equal in males and females
 Etiology
• Hydrocephalus is a condition of obstructed CSF
circulation or impaired absorption

Congenital causes Acquired conditions

• Myelomeningocele • Intraventricular
• Intrauterine infections haemorrhage
(CMV, Toxoplasmosis) • Tumor
• Aqueduct stenosis • Trauma
• Chiari malformation • CSF infection/ intracranial
infection
• Developmental causes

• Type II Chiari malformation : Herniation of cerebrum,

medulla, pons and 4th ventricle into cervical spinal canal
through enlarged foramen magnum resulting in
obstruction of CSF flow. Commonly associated with
meningomyelocele
• Dandy walker malformation: cystic expansion of
4th ventricle, partial / complete absence of cerebral
vermis and subsequent obstruction of CSF flow.
 Classification
• Communicating hydrocephalus/ Non obstructive
• occurs when the CSF flows out of the ventricles and into the
spinal canal, but it is not reabsorbed normally by the tissue
surrounding the brain and spinal cord. Sometimes this type of
hydrocephalus corrects itself.

• Non communicating hydrocephalus/ Obstructive

• occurs when the CSF does not flow properly between or out of
the brain ventricles because of an obstruction, such as from a
malformation or narrowing.
• Congenital hydrocephalus
• Congenital hydrocephalus, which is present at birth. Congenital
hydrocephalus may be caused by physical problems with how
CSF flows or is made or absorbed, by infections or trauma
during fetal development, or by teratogens.
• It may be linked with other birth defects that affect the spine,
especially open neural tube defects.

• Acquired hydrocephalus

• Acquired hydrocephalus, which develops at the time of birth or

later.
• It can be caused by infections such as meningitis, bleeding,
injury, or a tumor.
• Ex vacuo hydrocephalus
• Refers to a condition of brain volume loss. This may be
present at birth.
• It may be the result of failure in fetal development of
brain (eg; schizencephaly)
• Brain also undergo destruction or atrophy from infection
or poor nutrition .
• Ventricles become large to fill the space where there is
absence of brain tissue .

Source:http://adameducation.com/adam_images
Pathophysiology
CSF fluid imbalance?
1.Impaired absorption
2.Obstruction of flow through ventricular system

• Increased accumulation of CSF

• Dilatation of ventricles & cortex can atrophy

• Increased CSF in ventricular system

• Compress brain against cranium

• If this occur before fusion of cranial sutures there
can be:
• Enlargement of skull
• dilatation of ventricles

If sutures are previously closed

sagittal suture may become opened

Pathophysiology
Normal CSF flow Obstruction of CSF flow

• Source http://adameducation.com/adam_images
Clinical manifestations
• Infants – early
• Abnormally rapid head growth

• Bulging fontanels (tense , non pulsatile)

• Dilated scalp veins

• Separated sutures

• Macewen sign ( cracked pot sound on percussion of skull near the

junction of the frontal, temporal and parietal bones.)

• Thinning of skull bones

• Poor head control

Later in infancy

• Frontal enlargement

• Depressed eyes

• When the third ventricle dilates, the child

can present with Parinaud syndrome
(upgaze palsy with a normal vertical Doll
response) or

• The setting sun sign (Parinaud syndrome

with lid retraction and increased tonic
downgaze).

• Pupils sluggish

• Unequal pupillary response to light

Other symptoms
• Irritability, high pitched cry
• Lethargy
• Cries when picked up/ rocked & quiet when
allowed to lie still
• Change in level of consciousness
• Ophisthotonos
• Lower extremity spasticity
• vomiting

Source:http://diseasespictures.com/hydrocephalus/
Childhood
• Headache on awakening
• Blurred vision due to Papilledema
• Strabismus (Abducent nerve palsy)
• Irritability
• Lethargy
• Confusion
• Vomiting
• Horizontal Diplopia due to
Lateral rectal nerve palsy
 Diagnosis
• History collection

• Physical findings in infants include the following:

Vital signs:

• Bradycardia due to pressure on brain stem

• Systemic hypertension
• Altered respiration & sometimes apnea
Head
• Head enlargement ( HC > 2 cm per month in first 6
months)
• Dysjunction of sutures
• Dilated scalp veins
• Tense fontanelle, anterior fontanelle bulging
In older children
• Large head: Sutures are closed, but chronic
increased ICP will lead to progressive
macrocephaly.
• Macewen sign: A "cracked pot" sound is
noted on percussion of the head (open
fontanels).
• As long as the anterior fontanel is open i.e.
up to 18 months, the test will be positive.

• Transillumination
• Transluscency extending beyond 2.5 cm in frontal
area or over 2cm in occipital area is abnormal
Eyes
• Setting-sun sign: In infants, it is characteristic of
increased intracranial pressure (ICP). Ocular globes
are deviated downward, the upper lids are
retracted, and the white sclerae may be visible
above the iris.
• Papilledema: If the raised ICP is not treated, this
can lead to optic atrophy and vision loss.

• Failure of upward gaze

• Unilateral or bilateral sixth nerve palsy (abducens)

is secondary to increased ICP. (convergent
strabismus)
 Upper and lower limbs
In infants
• Increased limb tone: Spasticity preferentially affects the
lower limbs. The cause is stretching of the periventricular
pyramidal tract fibers by hydrocephalus.

In children
• Unsteady gait: This is related to spasticity in the lower
extremities.
• Pyramidal signs in lower limbs due to stretching of
pyramidal tract fibres.
• Exaggerated plantar reflex and deep tendon reflexes of
lower limbs along with increased tone of lower limb
 Other diagnostic tests
• Ultrasound

Hydrocephalus
• Magnetic Resonance Imaging (MRI)
• Computerized tomography (CT)
Medical Management
• The goal of treatment in clients with hydrocephalus is to
reduce or prevent brain damage by improving the flow of
CSF

• The following medications are used to treat hydrocephalus.

• Diuretics
• Acetazolamide - dose may be as high as 100mg/kg.
• furosemide 1mg/kg (treat posthemorrhagic hydrocephalus to
decrease secretion of CSF at the level of the choroid plexus).
• Anticonvulsants
• Interfere with impulse transmission of cerebral cortex and prevent
seizures.
• Antibiotics
• Culture and sensitivity dependent for shunt infections such
as septicemia, ventriculitis, meningitis, or given as a prophylactic
treatment
 EVD - External ventricular drain
• EVDs are a short term solution to hydrocephalus

• The purpose of external ventricular drainage is to

divert fluid from the ventricles of the brain and
allow for monitoring of intracranial pressure
 Shunt
• The most common treatment for hydrocephalus is the surgical
insertion of a drainage system, called a shunt

Types of shunts
• Ventriculoperitoneal ( VP) shunt

• Ventriculoatrial (VA) shunt

• Lumboperitoneal (LP) shunts (Koutoukidis et al.

2016).

• Ventriculopleural shunts ( E Küpeli et.al. 2010)

• Shunts will generally consist of three components:

1. An inflow catheter – This drains the CSF from the

ventricles. It leaves the brain through a small hole in
the skull, which then runs under the skin.

2. A valve mechanism – This regulates the pressure

control through the shunt tubing. It is connected to
the catheter and lies between the skin and the skull,
usually on top of the head or behind the ear.

3. An outflow catheter – This runs under the skin and

moves the CSF from the valve to the peritoneal cavity,
heart or other drainage site.
(Hydrocephalus Association 2017)
• One end of the tubing is usually placed in one of
the brain's ventricles.

• The tubing is then tunneled under the skin to

another part of the body where the excess
cerebrospinal fluid can be more easily absorbed —
such as the abdomen or a chamber in the heart.

• People who have hydrocephalus usually need a

shunt system for the rest of their lives, and regular
monitoring is required.
VP shunt procedure
Complications

• Infection - Most serious complication

• Septicaemia
• Bacterial endocarditis
• Wound infection
• Meningitis
• ventriculitis
• Malfunction
• All shunts are subject to mechanical difficulties like
kinking, plugging, separation or migration of tubing
• Endoscopic third ventriculostomy

• In this procedure, an endoscope is used to

make a small opening in the bottom/ floor
of 3rd ventricle to enable cerebrospinal fluid
to flow freely through the previously blocked
ventricle.
Complications
• Intraventricular haemorrhage
• Meningitis
• Septostomy failure
Nursing care
Assessment
History taking.
• Head circumference – Occipitofrontal circumference
• Neurologic and vital signs.
• Check the fontanelles. If the fontanelles are not
closed, carefully observe them for any signs of bulging
tenseness & separation
• Assess for irritability, lethargy, seizures, feeding
behaviour & vital signs.
• Monitor increase in intracranial pressure (LOC).
 SIGNS OF INCREASED ICP
•Shallow breathing
•Headache
•Nausea
•Vomiting
•Increased blood pressure
•Decreased mental abilities
•Confusion about time,
location and people
•Double vision
•Pupils that don’t respond to
changes in light
•Seizures
•Loss of consciousness
•Coma
GCS ASSESSMENT FOR CHILDREN
Preoperative care
• Maintain nutrition
• Prepare child for diagnostic tests – CT, ventricular
tap
• Preoperative preparation
• Explanation to child and family
• Skin preparation
• Safety
• Psychological support
Post operative care
• Continuous monitoring Q1 hourly
• Position on unoperated site to prevent pressure on
shunt valve & pressure areas
• Child is kept flat to avoid complication resulting from
rapid reduction of ICP.

( when ventricular size reduces too rapidly the cerebral

cortex may pull away from dura & tear the small
interlacing veins resulting in subdural hematoma)
• In case of Increase in ICP, surgeon may prescribe head end
elevation or allow child to sit up to enhance gravity flow through
shunt

• Continue assessment – GCS , vital signs, pupillary dilatation,

hypoxia

• Observe for abdominal distension – (peritonitis , post op paralytic

ileus)

• Monitor intake output

• Nutrition (iv fluids / oral feeds after determining bowel sounds)

• Skincare

• Family support
Nursing Diagnosis
• Risk for Injury related to increased ICP.

• Risk for Impaired Skin Integrity related to pressure from

physical immobility.

• Risk for Infection related to the presence of a shunt.

• Risk for Delayed Growth and Development related to

impaired ability to achieve developmental tasks.

• Anxiety related to the family caregiver’s fear of the surgical

outcome.

• Deficient Knowledge related to the family’s understanding

of the child’s condition and home care.
Task …
• Identify signs of hydrocephalus from the following
case study

• No group answers…
Case 1

• Name : Ms Yuvarani Age :15 years Sex :female

• 1st born to her parents ( 3rd degree
consanguineous marriage.)
• Admitted with h/o:
• Fever x 15 days ( high grade, intermittent)
• Vomiting x 2 days
• No cold/cough/joint swelling/ skin rash
• Txd in local hospital – TB meningitis
• After 10 days developed holocranial headache (throbbing)
• Vomiting – non projectile , non bilious
• Referred to cmch on 20/11/17
• Gcs 15/15
• After admission on day 2
• Drooping of eye lids & blurred vision in left eye x 1 day

•Physical examination : anterior fontanelle 1x1 cm, flat,

pulsatile
•Drooping eye lids
•Visual disturbance in left eye
•Otherwise nil significant findngs
• TB XPERT PCR – MTB
• CSF : occasional pus cells
• CT: features of early cerebral edema
• Started cefotaxime 150mg/kg
• Day 8 seizures: phenytoin
• Repeat CT :obstructive hydrocephalus
• GCS 13/15
• Seizures, GCS dropped to 7/15
• Intubated
• EVD inserted
• 2nd line ATT started
• Pupils were dilated
• Bp fluctuating & hypotension : ionotropes and crystalloids
• On 2/12/17 – death declared
Case 2
• Master Abdus
• 3months old
• Congenital hydrocephalus
• c/o progressive increase in head size
• Feeding difficulty
• Fast breathing , RR – 36/min
• Irritability
• No h/o fever , vomiting, loose stool, seizure or
cyanosis
• Antenatal h/o

• Ist trimester uneventful, 2 TT doses, iron & folate

suppliments taken
• 2nd trimester: USG showed dilated lateral ventricles
• 3rd trimester: no fever/ GDM/PIH
• Natal : born at term, NVD, birth wt : 3.5 kg
• Postnatal: NICU X 10 days with HIE, sepsis

• Diet : on formula feeds ( not sucking well)

• Immunization: upto date

• Development : social smile 1 month , not attained head control

Physical examination
• Vital signs
• HR- 168/Min
• RR – 168/min
• Spo2 – 93%
• Macrocephaly
• Anterior fontanelle 6 x 4 cm, flat , pulsatile
Quiz
• Define hydrocephalus

• Types of hydrocephalus

• Any 2 Signs that can be elicited in a child with

hydrocephalus

• Surgical procedures for Management of hydrocephalus