HYDROCEPHALUS

W & W 495-503
Hydrocephalus
A syndrome, or sign, resulting from
disturbances in the dynamics of
cerebrospinal fluid (CSF), which may
be caused by several diseases.
Incidence
Occurs in 3-4 of every 1000 births.
Cause may be congenital or acquired.
Congenital- may be due to
maldevelopment or intrauterine
infection
Acquired- may be due to infection,
neoplasm or hemorrhage.
Pathophysiology
CSF is formed by two mechanisms:
Secretion by the choroid plexus,
Lymphatic-like drainage by the
extracellular fluid in brain.

CSF circulates thru ventricular system
and is absorbed within subarachnoid
spaces by unknown mechanism.
Mechanisms of Fluid
Imbalance
Hydrocephalus results from:
1. Impaired absorption of CSF within
the subarachnoid space
(communicating hydrocephalus), or
2. Obstruction to the flow of CSF
through the ventricular system (non-
communicating hydrocephalus)
Mechanisms of fluid
imbalance
Both lead to increase accumulation of
CSF in the ventricles!
Ventricles become dilated and
compress the brain.
When this happens before cranial
sutures are closed, skull enlarges.
In children <10-12, previously closed
sutures may open.
Hydrocephalus
Most cases of non-communicating
(obstructive) hydrocephalus are a result of
developmental malformations.
Other causes: neoplasms, intrauterine
infections, trauma.
Developmental defects account for most
causes of hydrocephalus from birth to 2
years of age. (Table 11-3, page 497- sites
and types of hydrocephalus)

Common Defects
Arnold-Chiari Malformation (ACM)
Type 2 malformation of brain seen most
exclusively with myelomeningocele, is
characterized by herniation of a small
cerebellum, medulla, pons, and fourth
ventricle into the cervical spinal canal
through an enlarged foramen magnum.

Clinical manifestations
Clinical picture depends on acuity of
onset and presence of preexisting
structural lesions.

Infancy
Head grows at alarming rate with
hydrocephalus.
First signs- bulging of fontanels without
head enlargement.
Tense, bulging, non-pulsatile anterior
fontanel
Dilated scalp veins, esp. when crying
Thin skull bones with separated sutures
(cracked pot sounds on percussion)
Infancy
Protruding forehead or bossing.
Depressed eyes or setting-sun eyes (eyes
rotating or downward with sclera visible
above pupil)
Pupils sluggish with unequal response to
light
Irritability, lethargy, feeds poorly,
changes in LOC, arching of back
(opisthotonos), lower extremity spasticity.
May cry when picked up or rocked; quiets
when allowed to lay still.

Infancy
Swallowing difficulties, stridor,
apnea, aspiration, respiratory
difficulties and arm weakness may
indicate brain stem compression.
If hydrocephalus progresses,
difficulty sucking and feeding, and a
high-pitched shrill cry results. (lower
brain stem dysfunction)
Infancy
Emesis, somnolence, seizures, and
cardiopulmonary distress ensues and
hydrocephalus progresses.
Severely affected infants may not
survive neonatal period.
Childhood
Signs and symptoms caused by
increased ICP.
Manifestations caused by posterior
neoplasms and aqueduct stenosis,
manifestations associated with
space-occupying lesions.
Childhood

Headache on awakening with improvement
following emesis or sitting up.
Papilledema (swelling of optic disc DT
obstruction), strabismus, and
extrapyramidal tract signs such as ataxia
Irritability, lethargy, apathy, confusion,
and often incoherent

Childhood
Dandy-Walker syndrome- congenital
defect-late onset.
Obstruction of foramen of Lushka and
Magendie
Bulging occiput, nystagmus, ataxia,
cranial nerve palsies
Female predominance (3:1)
Absence or occlusion of ventricles
Diagnostic Evaluation
Antenatal- fetal ultrasound as early as 14
weeks
Infancy- based on head circumference
crosses one or more grid lines on the
infant growth chart within a 4 week period
and there are progressive neuro signs.
CT and MRI to localize site of obstruction;
reveal large ventricles
Therapeutic management
Goals:
Relieve hydrocephaly
Treat complications
Manage problem resulting from
effects of disorder on psychomotor
development
USUALLY SURGICAL!
Surgical Treatment
Therapy of choice!
Direct removal of source of
obstruction (neoplasm, cyst, or
hematoma)
Most require shunt procedure to
drain CSF from ventricles to
extracranial area; usually
peritoneum(VP shunt), or right atrium
(VA shunt) for absorption.
VP shunt
Used in neonates and young infants
Greater allowance for excess tubing;
which minimizes number of revisions
needed as child grows
VA shunt
Reserved for older children who have
attained most of somatic growth, or
children with abdominal pathology.
Contraindicated in children with
cardiopulmonary disease or with
elevated CSF protein.
Major Complications
Shunt infection is most serious
complication!
Period of greatest risk is 1 to 2
months following placement.
Staph and strep most common
organisms
Complications
Mechanical difficulties
kinking, plugging, migration of
tubing.
Malfunction is most often by
mechanical obstruction!
Look for signs of increased ICP;
fever, inflammation and abdominal
pain.
Post-op care
In addition to routine post-op care:
1. Place on unoperated side to prevent
pressure on shunt valve
2. Keep HOB flat; rapid decrease in IC
fluid may cause subdural hematoma due
to small vein rupture in cerebral cortex.
3. Do not pump shunt without specific
direction from doctor (too many
different pump devices)
Post-op care
4. Observe for signs of Increased ICP!
May indicate obstruction of shunt!
Assess pupil size; as pressure on oculomotor
nerve may cause dilation on same side as
pressure.
Blood pressure may be variable due to hypoxia
to brainstem
Abdominal distention- due to CSF peritonitis or
post-op ileus due to catheter placement.
Post-op
5. Monitor I and O- may be on fluid
restriction or NPO for 24 hours to
prevent fluid overload.
6. Monitor VS- increased temp may
indicate infection.
7. Give good skin care to prevent
tissue damage, etc.
Family support
Fear
Communication of procedures
Prepare for discharge.
SPINA BIFIDA
Neural Tube
defects are largest
group of congenital
anomalies.
Failure of neural
tube to close
produces defects
of either entire
neural tube or
small areas.
Etiology
Anacephaly and spina bifida occur
together very often.
Higher in females than males
50% occur due to nutritional
deficiency (folic acid)

Spina Bifida
Defined as midline defects involving failure of the
bony spine to close.
Spina bifida occulta- defect not visible externally.
Occurs most often in lumbosacral area.
Not apparent unless there are gait disturbances, foot
deformities, sphincter dysfunction or other
neuromuscular manifestations.
Many people with occulta will never have any deficits and
may not know they have it.
Spina Bifida
Spina Bifida cystica- visible defect
with external saclike protrusion.
A. meningocele- encases meninges and
spinal fluid, but no neurological deficits.
B. meningomyelocele-contains meninges,
spinal fluid, and nerves. Neuromotor
deficits depend on anatomic level of
protrusion and nerves involved.
Meningomyelocele
AKA spina bifida
Develops during first 28 days of
pregnancy when neural tube fails to
close and fuse.
90% of spinal cord lesions, and may
occur at any point along spine.
Sac usually enclosed in fine
membrane that is prone to tears.
meningomyelocele
Largest number in lumbar or lumbosacral
area
90-95% of children have hydrocephalus
Careful monitoring of head size important
Chiari malformation may be present:
observe infant for stridor, hoarse cry
from vocal cord paralysis; feeding
difficulties, deteriorating upper extremity
function.
Clinical manifestations
S/S vary according to degree of spinal
defect.
Readily apparent on inspection!
Loss of sensation below lesion
Poor urinary and bladder control
Joint deformities in lower extremities
Scoliosis or kyphosis
Hip dislocations
Diagnostic evaluation
Examination of meningeal sac and clinical
manifestations
MRI, CT to assess condition of brain and spinal
cord. Other defects may be present.
Prenatal- fetal ultrasound or amniotic fluid sample
for (alpha-fetal protein (AFP).
Test should be done between 16 and 18 weeks of
gestation. Afterwards AFP level drops, making
detection of SB difficult. Also, therapeutic
abortion not option after this time.
Therapeutic management
Multi-disciplinary approach
Neurology, neuro-surgery, pediatrics,
urology, orthopedics, rehabilitation, PT,
OT, social services, intensive nursing in
many areas.
Goals
1. prevent infection
2. early closure of lesion, within 72
hours (prevents infection and trauma
to exposed tissues, and prevents
further motor impairment). Goal is
satisfactory skin coverage of lesion!
3. PT for specific deformity

Goals
Physical therapy to prevent joint
contractures.
Correct deformity, prevent skin
breakdown, obtain best ambulatory
functioning
Goals
Management of genitourinary
function:
Mylemeningocele is a common cause of
neurogenic bladder which leads to
urinary system distress (frequent UTIs,
ureterohydronephrosis, vesicoureteral
reflux, renal insufficiency.
Urinary incontinence is common
Goals
Clean, intermittent catherization as a
conservative treatment.
Vesicostomy (anterior wall of bladder
brought through abdominal wall to
create stoma) may be done for
bladder control.
Meticulous skin care is needed!
Nursing care
Pre-op
Positioning to keep off sac (use diaper
rolls, pads or sandbags)
Keep in prone position
Can be challenging!
More difficult to keep clean, pressure
areas a threat, feeding a problem
Nursing care
Pre-op
Turn head on side for feeding
Diapering may be contraindicated until
repair and healing has taken place.
Constant stooling due to affected bowel
sphincter (not diarrhea).
Keep skin clean.
Post-op care
Prone position until healing takes
place!
May allow side-lying- depends on
doctor.
Feeding resumed after anesthesia
wears off
Comfort measure/vital signs/I & O
Latex allergy
70% of children and adolescents with
SB are sensitive to latex.
Cause unknown-probably continued
exposure to latex.

Avoid latex, balloons, condoms, catheters,
or anything with latex!
Post-op
Use touch for stimulation, since cant
hold
Observe for increased ICP, such as
bulging fontanels
Assess for infection:
Increased or decreased temperature,
irritability, nuchal rigidity,
Home Care
Involve parents in care
Positioning/feeding/skin care/range of
motion exercises/ clean catheterization
when prescribed, complications.
Help with assistive devices (if child is
paraplegic use hands/arms, etc.)
Long range planning
Spina Bifida Association of America