HYDROCEPHALUS (H) &

SPINA BIFIDA (SP BI)

SAM LAZUARDI

SPINA BIFIDA (SP BI)

Spina bifida (split spine) : incomplete closure of
embryonic neural tube, overlying vertebrae unfused & open abn portion of sp cord stick out
through opening in bones
Sp bi 4 categories : sp bi occulta, sp bi cystica
(myelomeningocele), meningocele, lipomeningocele
Location : lumbar & sacral
Myelomeningocele disability
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SPINA BIFIDA (SP BI)

Sp bi can be closed surgically, but damage is
permanent
Sp bi incidence can be 70 %, by daily folic acid
intake before conception
Sp bi lumbar symptoms hip, leg, knee, foot
muscles & scoliosis
Sacral foot muscles, bowel- bladder problems,
erection
Often hydrocephalus, rarely intellectual problems
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SPINA BIFIDA (SP BI)

Tethered sp cord sy (TSC) as a result from scar
tissue following surgery to close open defect sp bi
myelomeningocele early D/ & Th / before
permanent damage
TSC symptoms : lower body pain, leg weakness,
incontinence, scoliosis, numbness
73 % Sp bi allergy to latex (gloves, catheter etc)

SPINA BIFIDA (SP BI)

Sp bi occulta (hidden) : no opening, but outer part
of vertebra not completely closed skin normal /
some hair growing, dimple in skin, lipoma or
dermal sinus, birthmark
May suffer TSC sy from diastematomyelia or
lipoma in lipomyelomeningocele sp cord
trapped below affected level of growing spine
legs & bladder symptoms
Back pain worse with sp bi occulta
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SPINA BIFIDA (SP BI)

Meningocele or meningeal cyst (least common)
Sp bi cystica most common & serious
Unfused portion of sp collumn allows sp cord
protrude through opening in overlying vertebr
Meningeal membrane covering sp cord form a sac
enclosing spinal elements
Sp bi + myeloschizis most severe : plate-like mass
of nervous tissue with no overlying skin prone
to infections
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SPINA BIFIDA (SP BI)

Depending of locations deformities of hip
knees, feet loss of muscle tone, some degree
of paralysis, loss of sensation below level of
spinal defect, bladder bowel control
problems
Associated abnormalities : Arnold chiari II
(cerebellum malformation), myelomeningocele, hydrocephalus
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SPINA BIFIDA (SP BI)

Pathophysiology :neural tube closure 28
days postfertilization
Cause : multiple gene & environmental
factors (anticonvulsant, antidiabetic drugs)
Women with risk factor high dose folic
acid (4 mgr/d) 3 mo before conception - 12
wks during pregnancy (normal 0.4 mgr / d)
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SPINA BIFIDA (SP BI)

Folic acid in leaf veggies, whole grains, fruits
& beans
Pregnancy screening : AFP(alfa fetopro- tein
for Down sy, SP BI, Neural Tube Defect), USG,
amniocentesis, genetic counseling.
Incidence 1 2 per 1000 births
Treatment : surgery (sp cord & nerve roots put
back into spinal canal & covered with
meninges) & shunt for hydrocephalus
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OBESE WOMEN
Obese women significantly more likely to
have children with birth defect such as
spina bifida, heart defects, cleft lip than
slim mothers
An estimated one in six pregnant women in
Britain is overweight (10 Febr 2009)

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Vitamin B12 Deficiency Can Lead

To Childhood Brain Damage
Women with low levels of Vitamin B12 just before
or after conception are more likely to give birth to
children with neural tube defects (Pediatrics,
February 2009)
The study authors wrote that it is not known how
B12 and folate might interact to influence the
formation of the neural tube
many fish and shellfish are among the top sources
of vitamin B12
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HYDROCEPHALUS (H)
Hydrocephalus = water head
Abn CSF accumulation in ventricles IC
pressure in skull, progressive head enlargement, convulsions & mental disability
Normally CSF circulates through brain,
ventricles, spinal cord & drains into
circulatory system
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HYDROCEPHALUS (H)
H caused by overproduction of CSF, congen malf,
blocking, complication of trauma capitis or
inflammation blockage in ventricles
Any cause of CSF accum in ventricles brain
compression convulsion & retardation
especially older infants
Younger infants abn large head & downward
gaze
Th/ shunting excess CSF to peritoneum
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HYDROCEPHALUS (H)
Internal H : 4th foramina or cerebral
aqueduct blocked CSF accum in ventricles (brain stem tu) IC press
irreversible brain damage
External H : CSF accumulation in subarach
space
H cause : CSF flow obstruction, reabsorption, excessive production
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HYDROCEPHALUS (H)
1.Obstruction (most common) : aqueduct
stenosis, foramen Monro stenosis due to tu,
inflammation, hemorrhage, congenital
malformation
2.Overproduction of CSF : choroid plexus
papilloma
H : 1.communicating
2. Non-communicating (obstructive)
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HYDROCEPHALUS (H)
1.Communicating H : impaired CSF reabsorption
functional impairment of arachnoidal
granulations in sup sagital sinus by subarachn /
IVH, meningitis, chiari malf, congen absence of
Pachionis granulations Normal Pressure H
(NPH), H exvacuo (enlargement of ventricles &
subarach space by brain atrophy in dementia,
schizophrenia)
NPH : intermittent ICP D / continuous intraventricular pressure recording
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NORMAL PRESSURE
HYDROCEPHALUS NPH
NPH : Hakim triad gait instability,
urinary incontinence, dementia
Focal signs : n.6 palsy, gaze palsy (Parinaud
sy) due to compression of quadrigeminal
plate
The increased volume of CSF press down &
damage brain tissue
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RISK FACTORS OF NPH

Subarachnoidal hemorrhage
Closed head ijury
Meningitis
Surgery of the brain (craniotomy)
NPH account for 5 % of all dementias
Symptoms begin slowly
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3 SYMPTOMS OF NPH
1.Walking difficulties (gait apraxia), feet
held wider apart, shuffling & unsteadiness
2.Slowing of mental function :
forgetfulness, difficulty paying attention &
apathy
3.Urinary incontinence & sometimes fecal
incontinence
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EXAMS AND TESTS OF NPH

Sudden falls without a loss of consciousness
or drops attacks may occur early in the illness
Walking changes & increased deep tendon
reflexes in the lower legs
Lumbar puncture with careful testing of
walking before & after spinal tap
Head CT-scan or MRI of the head
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Treatment of NPH
* Ventricoperitoneal shunt
* Some improve, but many do not
Without treatment, symptoms often get worse and
could lead to death
People with minimal symptoms have the best
outcome
Possible complications : infection, bleeding,
dementia that become worse, injury form falls,
shortened life span
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HYDROCEPHALUS (H)
Noncomm H / obstructive H caused by CSF
obstruction :
1.Foramen Monro obstruction (colloid cyst)
2.Aqueduct Sylvvi ( atresia, tu, hemorrh, inflam)
3.4th ventricle obstruction
4. Foramen Luschka & Magendi obstr (Dandy
Walker malf)
5.Subarchn space surround brainstem obstruction
(hemorrhage, inflammation)
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HYDROCEPHALUS (H)
Congenital H : IVH in premature infants, inflam,
type 2 Arnold Chairi malf, Dandy Walker malf,
aqueduct atresia
Symptoms : poor feeding, vomiting, apatis,
downward eyes, visual reduction, weak
movements
80 - 90 % infant with spina bifida associated with
meningocele, myelomeningocele & develop H
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CHAIRI MALFORMATIONCM
The indented bony space at the lower rear of
the skull is smaller than normal -> cerebel-lum
& brainstem is pushed downward. The resulting
pressure on the cerebellum can block the flow
of csf & cause dizziness, muscle weakness,
numbness, vision problems, headache, and
problems with balance and coordination. There
are three primary types of CM.
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CM
Most common is Type I -> no symptoms &
found by accident during examination for
another condition. Type II (Arnold-Chiari malf)
is accompanied by myelomeningo-cele-a form
of Sp Bi -> partial or complete paralysis below
the spinal opening. Type III -most serious form
-> severe neurological defects. Often associated
with hydrocepha-lus, syringomyelia, and spinal
curvature.
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Arnold-Chiari malformation, blocking

fluid flow between the ventricles and ...
www2.warwick.ac.uk

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DANDY-WALKER SY
Dandy-Walker Syndrome is a congenital brain
malf. involving the cerebellum and the fluidfilled spaces around it -> enlargement of the
fourth ventricle, a partial or complete absence of
the area of the brain between the two cerebellar
hemispheres (cerebellar vermis), and cyst
formation near the internal base of the skull.
Increase size of the fluid spaces surrounding the
brain & increase in pressure -> hydrocephalus.
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Dandy Walker is
known by many
names: Dandy
Walker Syndrome,
Dandy Walker
Malformation,
Dandy Walker
Cyst, Dandy
Walker Variant

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A sagittal T1-weighted magnetic resonance imaging (MRI) scan in a 5-year-old girl (also
see the following 2 images) shows a large posterior fossa cyst elevating the torcular
herophili and sinus rectus (short arrow). The hypoplastic vermis is everted over the
posterior fossa cyst (long arrow). The cerebellar hemispheres and brainstem (b) are
hypoplastic. Thinned occipital squama is seen (arrowheads).

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An axial T2-weighted MRI scan

that shows hydrocephalus, a large
cerebrospinal fluid cyst in the
posterior fossa, thinned occipital
bone (arrows), and hypoplastic
cerebellar hemispheres with a
winged appearance (c).

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HYDROCEPHALUS (H)
Acquired H caused by CNS inflam,brain tu,
meningitis, trauma capitis, IC hemorrh
Symptoms of IC press : Hache, nausea,
vomit, papiledema, sleepiness, coma
IC press uncal / cerebellar herniation
brain stem compression life
threatening
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HYDROCEPHALUS (H)
H symptoms : learning disability, visual problems,
clumpsy, precocious puberty, epilepsy
D/ CT-scan
Th / ventricular cath into ventricles drain into
body cavities (peritoneum, right atrium, pleura)
Shunt complication: malfunction, inflammation,
failure hache, nausea, vomit, photophobia,
seizures
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