Jessica Potts is a 32 year old woman who is
pregnant for the first time. She is working
but she and her
are just making
ends meet with
the expenses for
Jessica is an average height and weight
woman as is Susan. When they decided
to have a child it was
fibroids and can not
therefore Jessica will
be carrying the child.
Treatment of infertility
Usually does not fully prevent conception,
especially in mild to moderate cases
Infertility is more common in women with
severe forms of disease
Treatments are varied
Surgical treatments are superior to hormonal
or medical treatments when goal is
Assisted reproduction may be used
the couple opt
for in vitro
fertilization, Jessica is successful
with IVF therapy on the first
In vitro fertilization
Successfully used in 1978
IVF: Who May Benefit?
Women who have a blocked or
damaged fallopian tube
Mild problem with male
No cause identified for inability
Patient’s who have tried IUI or
ovulation induction with no
IVF Procedure Cont’d
Eggs are mixed with sperm in dish and
cultured in incubator
Dish checked in 2 days to see if eggs
have been fertilized
Those kept are kept for a couple more
days and checked again
Fertilized eggs form ball of cells-embryo
Healthiest embryo is inserted into uterus
Taking progesterone all along to thicken
IVF Procedure Cont’d
Progesterone given by injection, pessary (gel)
Endometrium too thin IVF cycle abandoned
Two embryos transferred
with thin catheter through cervix into
the uterus (via ultrasound)
No more then three embryos
can be legally transferred
Number of embryos
transferred depends on your
age and chances of success
If successful able to
take a pregnancy test in 2
IVF treatment takes 4-6 weeks to complete
Success rates vary
gives women with blocked, damaged, or missing
fallopian tubes a chance to have a baby
increased chances for multiple births, increase
risk for miscarriage and other complications,
hormones not closely monitored lead to ovarian
hyperstimulation syndrome, ectopic pregnancy
The couple live in a small town
about half an hour from the city.
The medical clinic arranges for
Jessica’s prenatal care and any
tests and assessments she may
need for the duration of her
pregnancy and delivery.
Jessica and Susan are so happy to finally
be pregnant, they refuse most prenatal
They’re just so happy to have a baby in
their lives, they are not too concerned
about genetics and anomalies with the
They due, however, agree to have an
blood flow in different partsof
your baby'sbody — such asthe
umbilical cord, brain, liver, and
heart — to help your caregiver
assessyour baby'shealth. It can
ultrasound and usesthesame
Student presentation for more
Mrs. Pottsgoesinto spontaneouslabor at 3am at 39 weeks
gestation. Her labor and delivery areunremarkableand Jessica
and Susan welcomebaby Isabelleat 5:56am. Thecouplebecome
concerned when thenursesstart whispering about thebaby.
Jessicakeepsasking them what theproblem is, but getsno
immediateresponse. Onenursefinally tellsSusan and Jessicathat
thedoctor will bein soon to talk to them. Eventually, thedoctor
deliversthenewsthat their preciouslittlebaby girl infact has
Down Syndrome. Jessicaand Susan react with anger. They are
dissappointed that they didn’t go through with theserum
screening testshowever, they must moveforward now.
Isthemost frequently occurring
Isuniversal acrossraceand gender
Iscaused by an error in cell division
Why it occursisunknown
John Langdon Down (thefather of down
1866: published an accuratedescription of aperson
with down syndrome
Identified down syndromeasachromosomal
In CanadaDown Syndromeoccursin approximately 1
in 800 livebirths
Increasesto 1 in 100 in second birth if your first child
had Down Syndrome
In theUSmorethan 350 000 peoplehaveDown
Incidenceof Down Syndrome
Maternal Age Incidence of
20 1 in 2000
25 1 in 1200
30 1 in 900
35 1 in 350
40 1 in 100
45 1 in 30
49 1 in 10
Thepresenceof 47 chromosomesinstead of 46
Morespecifically it isthepresenceof extra
genetic material associated with the21st
Caused by an error in cell division
Theprocessof cell division involved in all cell
growth, differentiation, and repair
Thechromosomesof each cell duplicate
Two daughter cellsareproduced
They arediploid (contain 46 chromosomesin 23
Occursin all cellsexcept for theoocytesand
Thereisareduction in thenumber of chromosomes
occurs(end up with 23 chromosomes)
Oocytesand sperm arereferred to asbeing haploid
(contain asinglecopy of each chromosome)
Thepaired chromosomescometogether in preparation
for cell division, portionscrossover and genetic
recombination createsgreater diversity in oocytesand sperm
Accountsfor approximately 95% of down syndromecases
A pair of chromosomesmay fail to separatecompletely creating asperm or
oocytethat containseither 2 copiesor no copiesof aparticular chromosome.
Trisomy: when thereare2 copies
Monosomy: when thereareno copies
Prior to or at conception, apair of 21st chromosomesin either thesperm or
theegg failsto separate. Astheembryo develops, theextrachromosomeis
replicated in every cell of thebody
Down syndromeisaform of trisomy
thereisextragenetic material on the
Trisomy can occur on any
chromosomebut theonly formsthat
arefrequently seen in livebirthsare
on the13, 18, and 21 chromosome
Mosaicism occurswhen nondisjunction of
chromosome21 takesplacein oneof theinitial cell
divisionsafter fertilization causing aperson to have46
chromosomesin someof their cellsand 47 in others
Thisistheleast common form of Down syndrome
accountsfor only 1 to 2 percent of all cases
Occurswhen part of chromosome21 breaksoff during cell division and
attachesto another chromosome, usually chromosome14. Whilethetotal
number of chromosomesin thecellsremains46, thepresenceof an extra
part of chromosome21 causesthecharacteristicsof Down syndrome
Maternal ageisnot linked to thechanceof having ababy with translocation.
Most casesaresporadic, chanceevents, but in about onethird of
translocation cases, oneparent isacarrier of atranslocated chromosome.
For thisreason, thechanceof translocation in asecond pregnancy ishigher
than that seen in nondisjunction.
Accountsfor 3 to 4 percent
low nasal bridge
low set ears
singlecreaseacrosspalm of hand
slightly flattened facial profile
3/4 fetusesarespontaneously aborted
20% die beforetheageof 10 r/t complications
haveand IQ ranging from 25-50
1-3 to1-2 have congenital heart defects
most common arean atrioventricular septal
defect, persistant ductusarteriosus, and
tetraology of fallot
decreased ability to fight respiratory infections
increased susceptibility to leukemia
usually develop Alzheimer symptomsby the
increased risk for thyroid and vision problems
usually accompanied by somelevel of mental
averagelife expectancy is55
Down syndromeisnot treated
It isimportant to encourageindividualswith down syndrometo
develop their giftsand talents
Early intervention programscan beinitiated
Many individualswith down syndromego to school
(elementary, secondary, and post-secondary) and someadults
arecapableof working in thecommunity.
With proper careindividualswith down syndromecan lead
Prenatal screening (usually diagnosed here,
but not in Jessica’scase)
maternal serum screening
ultrasound (sonogram) screening
chorionic villussampling (CVS)
percutaneousumbilical blood sampling (PUBS)
Diagnosisismadeat thebirth of baby Isabelle
Along with the diagnosis of
The doctor informs Susan and Jessica that
he would like to run some tests on baby
Isabelle as it appears her head is
Jessica and Susan are panicked at this
point and the nurse tries to comfort the
The doctor refers to a neurologist who
examines baby Isabelle
The neurologist is a stoic man in his late
60’s and doesn’t believe in same-sex
couples having children
He bluntly tells Susan and Jessica the his
diagnosis of baby Isabelle and refers them
to community services for follow up
A Lateral View An Anterior View
Each cerebral hemispherecontainsalargelateral ventricle: Right and Left ventricle or
First and Second ventricle
Theseptum pellucidum separatesthetwo lateral ventricles
Located in thediencephalons
Two lateral ventriclesarenot directly connected to each other
communicateswith thethird ventriclethrough an interventricularforamen(foramen of
Third and Fourth ventricleareconnected through aslender canal known asthe
mesencephalic aqueduct (the aqueduct of Sylvius) located in themesencephalon
Fo urth Ventricle
Superior portion liesbetween theposterior surfaceof theponsand theanterior surfaceof
Extendsinto thesuperior portion of themedullaoblongata
Then narrowsand becomescontinuouswith thecentral canal of thespinal cord
Central canal: narrow passageway in thespinal cord
In thebrain, thepassageway formstheventricles
Thecentral canal and ventriclesarelined by acellular layer of
epithelial cellscalled theependymaand arefilled with
cerebrospinal fluid (CSF)
During embroyonic development and early childhood, thefree
surfaceof ependymal cellsarecovered with cilia
Theciliapersistsin adultsonly within theventriclesof thebrain, where
they assist in thecirculation of CSF
In other areas, theependymal cellstypically havescattered microvilli
participatein thesecretion of theCSF
sensory functions, such asmonitoring thecomposition of theCSF
Consistsof outer (Endosteal) and inner (Meningeal) fibrouslayers
Layersaretypically separated by aslender gab that containstissuesfluidsand blood
vessels, including several largevenoussinuses(Dural sinus).
Theveinsof thebrain open into thesesinuses, which deliver thevenousblood to the
internal jugular veinsin theneck.
Consistsof thearachnoid membrane, an epithelial layer, and thecellsand fibersof the
arachnoid trabeculaethat crossthesubarachnoid spaceto thepiamater.
Arachnoid membranecoversthebrain, providing asmooth surfacethat doesnot follow the
Sticksto thesurfaceof thebrain
It extendsinto every fold, and accompaniesthebranchesof cerebral blood vesselsasthey
penetratethesurfaceof thebrain to reach internal structures.
Completely surroundsand bathestheexposed surfacesof the
CNSand hasseveral important functions:
Cushioning DelicateNeural Structures
Thebrain issuspended insidethecranium and floatsin theCSF.
A human brain weighsabout 1400 g in theair, but only about 50 g when
supported by theCSF.
Transporting Nutrients, Chemical Messengers, and WasteProducts:
Ependymal lining isfreely permeable(exception: choroidsplexus)
CSF isin constant chemical communication with theinterstitial fluid of the
Fo rmatio n o f CSF:
Choroid plexus: consistsof acombination of specialized ependymal cells
and permeablecapillariesfor theproduction of cerebrospinal fluid.
Two extensivefoldsof thechoroid plexusoriginatein theroof of thethird
ventricleand extend through theinterventricular foramina. Thesefoldscover
thefloorsof thelateral ventricles.
In theinferior brain stem, aregion of thechoroid plexusin theroof of thefourth
ventricleprojectsbetween thecerebrellum and thepons.
Specialized ependymal cells, interconnected by tight junctions, surround the
capillariesof thechoroid plexus.
Theependymal cellssecreteCSF into theventricles
Also remove waist productsfrom theCSF and adjust itscomposition over time.
Choroid plexusproducesCSF at arateof about 500 ml/day
Total volumeof CSF at any momentsisapproximately 150 ml/day
entirevolumeof CSF isreplaced every eight hours
CSF circulatesfrom thechoroid plexusthrough theventriclesand thecentral canal of thespinal
AstheCSF circulates, diffusion between it and theinterstitial fluids(theextracellular fluidsin
most tissuesiscalled interstitial fluid) of theCNSisunrestricted between and acrossthe
TheCSF reachesthesubarachnoid spacethrough thetwo lateral aperturesand thesinglemedian
aperture, opening in theroof of thefourth ventricle.
CSF then flowsthrough thesubarachnoid spacesurrounding thebrain, spinal cord, and cauda
Fingerlikeextensionsof thearachnoid membrane, called arachnoid villi, penetratethe
meningeal layer of theduramater and extend into thesuperior sagittal sinus.
In adults, clustersof villi form largearachnoid granulations.
CSF isabsorbed into thevenouscirculation at thearachnoid granulationswhereit isfiltered and
discarded by thebody.
TheBlood Brain Barrier
Neural tissuein the CNSisisolated from thegeneral circulation by theblood-brain
Thisbarrier existsbecausetheendothelial cellsthat linethecapillariesof theCNS
areextensively interconnected by tight junctions
Thesejunctionsprevent thediffusion of materialsbetween adjacent endothelial cells
Only lipid-solublecompoundscan diffuseacrosstheendothelial cellsmembranes
into theinterstitial fluid of thebrain and spinal cord
Astro cyte Cells:
Restricted permeability characteristicsof theendothelial lining of brain capillaries
aredepended on chemicalssecreted by theastrocytes
cellsthat arein closecontact with CNScapillaries
Outer surfacesof theendotheliacellsarecovered by theprocessesof astrocytes
Releasechemicalsthat control thepermeability of theendothelium to various
If damaged or stop stimulating theendothelial cells, theblood-brain barrier
Not part of theneural tissueof thebrain thereforeno astrocytesarein contact
with theendothelial cells.
Asaresult, capillariesin thechoroidsplexusarehighly permeable
Substancesdo not havefreeaccessto theCNS
Specialized ependymal cellsinterconnected by tight junctions, surround the
Transport acrosstheblood-brain and blood-CSF barriersisselectiveand
Even the passage of small ions(sodium, hydrogen, potassium, or chloride) is
Someorganic compoundsarereadily transported, and otherscrossonly in
Susan and Jessica and angry and bitter
They want to know why this diagnosis of
Hydrocephalus wasn’t caught sooner? Why
were they missed?
An ultrasound will normally show that the
fetus’ fontanelle is bulging, and that the
head circumference is larger than normal
for the gestational age
Theterm hydrocephalusisderived from two words:
"hydro " meaning water
"cephalus" referring to thehead
A condition in which excessCSF buildsup within theventricles
of thebrain or in thesubarachnoid spaceand may increase
Can occur at any age
most common in infantsand adultsage60 and older
In most instances, hydrocephalusisalifelong condition in that
thepatient istreated rather than "cured"
If left untreated in theinfant, they can suffer from somedegree
of mental retardation and/or motor dysfunction.
In theUnited States, alittleover 1 in 1000 birthsareaffected by hydrocephalus.
Ashigh as1 in 500 births.
Hydrocephalusisoneof themost common "birth defects" and afflictsin excessof
10,000 babieseach year.
Studiesby theWorld Health Organization show that onebirth in every 2,000 result
Thereare70,000 dischargesayear from hospitalsin theUnited Stateswith a
Morethan 50% of hydrocephaluscasesarecongenital.
Asmany as75% of children with hydrocephaluswill havesomeform of motor
Over thepast 25 years, death ratesassociated with hydrocephalushavedecreased
from 54% to 5%; intellectual disabilitity hasdecreased from 62% to 30%.
Ocular gazeand movement disordersarefound in approximately 25 to 33% of
children with hydrocephalus.
About 80% of hydrocephaluspatientsareborn with other defects.
Congenital: when the condition exists at birth
Acquired: when it occurs as the result of a trauma to the brain
Impaired absorption of CSF from thesubarachnoid spaceoccurswhen an obstructive
processdisruptstheflow of CSF through thesubarachnoid space.
Thefluid doesnot reach theconvex portion of thecerebrum, wherethearachnoid
With acutehydrocephalus, thereisincreased ICPthat hasarapid onset. Thepatient can
deterioraterapidly into a deep comaif it isnot treated promptly.
ICPrisesif production of CSF exceedsabsorption. Thisoccursif CSF isoverproduced,
resistanceto CSF flow isincreased, or venoussinuspressureisincreased.
CSF production fallsasICPrises. Compensation may occur through
transventricular absorption of CSF and also by absorption along nerve
Temporal and frontal hornsdilatefirst, often asymmetrically.
Thismay result in elevation of thecorpuscallosum, stretching or
perforation of theseptum pellucidum, thinning of thecerebral mantle,
or enlargement of thethird ventricledownward into thepituitary fossa
(which may causepituitary dysfunction).
Thisisgrouped into 3 main causes:
1. Excessivesecretion of CSF by thechoroid plexusasin casesof
choroid plexuspapilloma(rare, bening tumour) or carcinoma. This
2. Blockageto CSF circulation. Thiscould beat any level of theCSF
•It could beat thelevel of theforamen of Monro wherewethereisunilateral
or bilateral coverageof theforamen of Monro giving dilatation of oneor
both lateral ventricles.
•Thisiscommonly seen in thecolloid cyst and tumoursof thethird ventricle.
• Suprasellar lesion assuprasellar arachnoid cyst or hypothalamic tumours
(craniopharyngioma; congenital pituitary tumour).
• Posterior fossatumoursareacommon causeof obstructivehydrocephalus
dueto blockageof the4th ventricle.
• Medulloblastoma, cystic astrocytomaand ependymomacan all lead to
3. Poor secretion of CSF into thevenoussinusescaused by scarring of the
arachnoid villi and iscommonly seen after meningitisor hemorrhage.
Forms of Hydrocephalus Description
(non-obstructive hydrocephalus) caused by
inadequate absorption of CSF when the
ventricular pathways are not obstructed.
(obstructive hydrocephalus) caused by
blockage in the ventricular pathways through
which CSF flows.
Results from obstruction of the flow of CSF
(intraventricular or extraventricular). Most
hydrocephalus is obstructive, and the term is
used to contrast the hydrocephalus caused by
overproduction of CSF.
Stabilization of known ventricular
enlargement, probably secondary to
compensatory mechanisms. These patients
may decompensate, especially following
minor head injuries.
Forms of Hydrocephalus
Congenital Causesin Infantsand
Characterized by an increased volumeof CSF
May becaused by:
A blockagewithin theventricular system in which theCSF flows
An imbalancein production of theCSF
Reduced reabsorption of theCSF that resultsin enlargement of the
ventricles, and increased ICP
This pressurewithin theventricular system pushesand compressesthe
brain against theskull cavity.
Beforethecranial suturesfuse, theskull can increaseto accommodate
theadditional space-occupying volumeto preserveneuronal function.
Stenosis of the Aqueduct of Sylvius
Due to malformation: This is responsible for10% of all cases of
hydrocephalus in newborns, and is the most common cause.
This is an X-linked hydrocephalus. It is characterized by stenosis of the
aqueduct of Sylvius, severe mental retardation, and in 50% by an
adduction-flexion deformity of the thumb.
• Thisaffects2-4% of newbornswith hydrocephalus.
•Dandy-Walker Malformation isararemalformation of thebrain that ispresent
at birth (congenital).
•Dandy-Walker Malformation isaform of "Obstructive" or "Internal
Noncommunicating Hydrocephalus," meaning that thenormal flow of
cerebrospinal fluid isblocked resulting in thewidening of theventricles.
•It ischaracterized by an abnormally enlarged spaceat theback of thebrain
(cystic 4th ventricle) that interfereswith thenormal flow of cerebrospinal fluid
through theopeningsbetween theventricleand other partsof thebrain
(foraminaof Magendiaand Luschka).
•Excessiveamountsof fluid accumulatearound thebrain and causeabnormally
high pressurewithin theskull, swelling of thehead (congenital hydrocephalus),
and neurological impairment. Motor delaysand learning problemsmay also
• Chiari malformations(CMs) arestructural defectsin the
cerebellum, thepart of thebrain that controlsbalance.
• Thecerebellum and brainstem can bepushed downward.
• Theresulting pressureon thecerebellum can block theflow of
cerebrospinal fluid and can causearangeof symptomsincluding
dizziness, muscleweakness, numbness, vision problems, headache,
and problemswith balanceand coordination.
• Isaccompanied by amyelomeningocele-aform of spinabifida
that occurswhen thespinal canal and backbonedo not closebefore
birth, causing thespinal cord to protrudethrough an opening in the
•Thiscan causepartial or completeparalysisbelow thespinal
opening, and hydrocephalus.
Agenesis of the Foramen of Monro
• Narrowing of theforamen of Monroe.
• Sincetheforamen narrows, thisleadsto increased pressureto
push theCSF through theforamen of Monroe.
• AKA Interventricular foramen.
• Group of symptomsand characteristicscaused by infection of thefetuswith the
organism To xo plasma go ndii.
• Fetal infection resultswhen anonimmunepregnant woman isinitially infected
with toxoplasmosis(from certain foods, cat feces, or if shehasahistory of
• Congenital toxoplasmosisischaracterized by damageto theeyes, nervous
system, skin, and ears.
• Can occur asaresult of ingestion of raw or inadequately cooked infected meat,
ingestion of oocysts, an environmentally resistant form of theorganism that cats
passin their feces, with exposureof humansoccurring through exposureto cat
litter or soil (e.g., from gardening or unwashed fruitsor vegetables), and anewly
infected pregnant woman passing theinfection to her unborn fetus.
MRI’s: Congenital Causes of
Acquired Causesin Infantsand
Masslesionsaccount for 20% of all casesof hydrocephalusin
children. Theseareusually tumors(eg, medulloblastoma,
astrocytoma), but cysts, abscesses, or hematomaalso can bethe
Intraventricular hemorrhagecan berelated to prematurity, head
injury, or ruptureof avascular malformation.
Infections: Meningitis(especially bacterial) and, in some
geographic areas, cysticercosiscan causehydrocephalus.
•Increased venoussinuspressure: Thiscan berelated to
achondroplasia, somecraniostenoses, or venousthrombosis.
Iatrogenic (result of medical interventions): HypervitaminosisA,
by increasing secretion of CSF or by increasing permeability of
theblood-brain barrier, can lead to hydrocephalus.
Signs and Symptoms of Hydrocephalus
Clinical featuresof hydrocephalus
areinfluenced by thefollowing:
Location of obstruction
Rapidity of onset
Thin, shiny skin over fontanelles
Papilledema(swelling of theeye’snerves) and later optic
Slowing of mental capacity
Headaches(initially in themorning) that aremoresignificant than in
infantsbecauseof skull rigidity
Neck pain suggesting tonsillar herniation
Vomiting, moresignificant in themorning
Blurred vision - Consequenceof papilledema(swelling of theeye’s
nerves) and later of optic atrophy
Doublevision - Related to unilateral or bilateral sixth nervepalsy
(affectsabducenscranial nerve, and eyescannot turn outward beyond midline,
doublevision also occurs, but disappearswhen oneeyeisclosed)
Stunted growth and sexual maturation from third ventricle
dilatation: Thiscan lead to obesity and to precociousor delayed
onset of puberty.
Difficulty in walking secondary to spasticity: Thisaffectsthe
lower limbspreferentially becausetheperiventricular pyramidal
tract isstretched by thehydrocephalus.
Physical Assessment of A Neonate
*Similar to adult head-to -to e
assessment, with the fo llo wing exceptio ns: *
• Vital signs
• Skin and hair – Lanugo, vernix caseosa(thick, cheezy
protectiveintegumentary deposit that consistsof sebum, and
shed epithelial cells). Stork bites(back of neck, lower occiput,
upper eyelids, and upper lip).
• Head, Faceand Eyes– Infantshaveanterior or posterior
fontanelles, and they should not bulgeor sink.
• Newbornsdon’t producetearsuntil 2-3 months.
• TheEustachian tubeismorehorizontal, wider, and
shorter, thuscan increaselikelihood of middleear
• Thorax and Lungs: up to 3-4 monthsabdominal
breathing. Measurechest circumference.
• Cardiovascular: Infantshaveahigher circulating blood
• Abdomen: Liver isproportionately larger.
• Musculoskeletal: Bonegrowth endsat 20 (when
• Neurological: ApgarScores – Method to reassessneed for
newborn resuscitation in thedelivery room.
• Given at 1 and 5 minutesfollowing birth. Scoreof 8-10
Newborn in good condition, 4-7 Moderately depressed
newborn, 0-3 indicatesseveredepression, and needs
• Reflexes: Rooting, sucking, palmar grasp, tonic neck,
stepping, plantar grasp, Babinski’s, and Moro.
• Genitourinary System: During infancy, thebladder islocated in
between thesymphysispubisand theumbilicus. Monitor I & O.
• Gastrointestinal: Meconium stools, then after 3 days, yellow
coloured. Important to monitor bowel function and I & O to
ensurethat infant doesnot becomedehydrated.
• Inspection of genitalia
Upon Physical Assessment
Head enlargement: Head circumferenceisin the98th
percentilefor theageor greater.
Dysjunction of sutures: Thiscan beseen or palpated.
Dilated scalp veins: Thescalp isthin and shiny with easily
Tensefontanelle: Theanterior fontanellein infantswho are
held erect and arenot crying may beexcessively tense.
Setting-sun sign: In infantsit ischaracteristic of
increased ICP. Both ocular globesaredeviated
downward, theupper lidsareretracted, and thewhite
Increased limb tone: Spasticity affectsthelower limbs.
Thecauseisstretching of theperiventricular pyramidal
tract fibersby hydrocephalus.
Papilledema: if theraised ICPisnot treated, thiscan
lead to optic atrophy and vision loss.
Failureof upward gaze: Thisisdueto pressureon the
tectal platethrough thesuprapineal recess.
Macewen sign: A "cracked pot" sound isnoted on
percussion of thehead.
Unsteady gait: Thisisrelated to spasticity in thelower
Largehead: Suturesareclosed, but chronic increased ICPwill
lead to progressiveabnormal head growth.
Unilateral or bilateral sixth nervepalsy (affectsabducens
cranial nerve, and eyescannot turn outward beyond midline,
doublevision also occurs, but disappearswhen oneeyeis
closed) issecondary to increased ICP.
With newborns, hydrocephalusisdetected almost
immediately asthechild'shead may belarger than
normal (macrocephaly). However, with older children
or adults, hydrocephalususually startsto reveal itself
with avariety of signsand symptomsweeksor months
It may bedetected by signsand symptomsof increased
CT and MRI
X-Raysdo not provideenough contrast to seethetissuesof thebrain.
CT Clearer picturesof thebodiesorgans, tissuesand bones. Approx. 2-5
MRI Internal structurescan beseen. Approx. an hour in length.
Provideaclearer view of gray and whitematter of thebrain, aswell as
thevascular system. Primary usefor neurosurgeons.
CT and MRI scanstakepicturesof thecompletecranial and intracranial
anatomy, including thesubarachnoid spacesand thestructuresof the
Taken laterally and sagitally (front-back)
Abnormal Head Growth (Macrocephaly)
Infantsand small children primary indicator. Kidssutureshavenot fused
Continueto monitor thegrowth of thechild’shead until thechild reachestheage
of 6 or 7.
High pitched cry/scream
Split suturesof theskull
Distended veinsin thescalp-bulging or widening of thefontanels
Absenceof up ward'sgaze, known as“sun setting” usually in acutenon-
Impaired lateral gaze(Sun setting oneor both eyes)
Lossof vision-weaknessor spasticity of limbs.
Initially, when oneor moresymptomsbecomeevident.
Infant Child’shead isbulging or larger than normal
Child Painful headaches, gait disorder or vision
Should bereferred to aneurosurgeon
Neurological Examination History of milestones, as
well asaphysical examination for neurological
1 year or older Examinetheinfant to seeif they arereaching mental and physical
Isyour infant communicating verbally?
Isyour infant performing well in school?
Hasyour infant fallen behind hispeersin recent months?
Isyour child having ahard timeremembering things?
Haveyou noticed any changesin personality in thelast few weeks/months?
Hasyour child started to show signsof walking by thetimethey were1?
Isyour infantsgait steady or unbalanced?
Doesyour child drift to thesidewhilethey walk?
Get thechild to balanceon onefoot, with their eyesclosed. Placeboth feet
together sideby sideto maintain balance.
Placetheindex finger in front of thefaceand ask to follow movement.
(testing for paralysisof theabducens- 6th
cranial nerve). Controlssideto
Walk heal-to-heal. If child hasdifficulty could bean indicator of pressure
Check plantar, Babinski reflex.
If thebig toemovesupward, resultsan extensor responseor Babinski
reflex. Babinski reflex isaclear indication of someform of brain or spinal
cord disease. Usually skip thistill theinfant isat least 1 year old becauseit
isusually positivewhether theinfant hasit or not.
Pronator drift: Closeeyeswhilestanding, extend both armsin front with
palmsup. Seeif onearm waversor drifts. Indication of injury to themotor
Effectson Family Dynamics
Emotionscan rangefrom worry to fear, aswell
asresentment and jealousy.
Children also haveactiveimaginations.
Usually their emotionsareworsethan their
Talk through their fears.
Siblingsmay feel completely overwhelmed.
Resentment and jealousy arecommon feelings
experienced by siblings.
Let them know they areloved and valued.
Protecting thehead of theinfant or child from injury by handling thechild
carefully may help prevent thedevelopment of injury induced
Prompt treatment of infectionssuch asmeningitisand othersassociated
with hydrocephalusmay reducetherisk of developing thedisease.
Women who takecytomegalovirusor toxoplasmosisacquired by amother during
pregnancy may cause hydrocephalus. May reducetherisk of being infected by
Cooking meet and veggiescarefully.
Cleaning contaminated knivesand cutting servicesproperly.
Avoid handling cat litter, or wearing gloveswhen cleaning thelitter box.
Lymphocytic choriomengitisvirus(LCV) which pet rodants(mice) often carry
can lead to hydrocephalusin pregnancy.
Infection with chickenpox or mumpsduring or right after pregnancy may also lead
to hydrocephalusin thebaby.
Bedsidenurseisin auniqueposition to havean impact on patients’ and
Nurseneedsto empower and educatethefamily of theimportanceof aseptic
techniquewhen taking careof thechild’ssurgical site.
Stresstheimportanceto thefamily that their child should maintain optimal
health with proper nutrition and exercise.
Needsto supply thefamilieswith life-saving information of thesignsand
symptomsof ashunt malfunction and or infection.
Isabelle’sscalp, over theanterior fontanelle, is
shiny and thin and thetiny veinsareprominent.
Isabelleisthen sent to theNICU to beclosely
monitored for complicationsassociated with
increased intracranial pressure. Exactly 2 weeks
after Isabellewasborn, sheundergoessurgery
to insert aVPshunt. Thesurgery went very well
with no complications.
Thereare25,000 shunt operationsperformed each year in theUnited States.
Of those, some18,000 areinitial shunt placements.
Some85% of peoplewith shuntshavehad at least two shunt operations.
Studiesshow that therisk of shunt failurein an infant'sfirst year is30%.
Shuntsarerevised about 2 timesin thefirst ten yearsof useper patient.
95% of shunt infectionsoccur within 3 to 5 daysof surgery.
Thereported frequency of shunt infection variesfrom 1.5 to 39% with an
averageof 10 to 15%.
Morethan 50% of staphylococcal infectionsoccur with in 2 weeksof the
operation, and 70% of infectionsoccur within 2 months.
Theoverall complication rateof CSF shuntsremainsquitehigh: 25 to 60%.
Shunt malfunctionsoccur in about two to 40% of cases.
Primary Goal of aVPshunt: To ensureon aregular basisthat theshunt continuesto
A VPisalong, plastic tubethat allowsfluid to drain from thebrain to another part of
thebody (Peritoneal Cavity). Thisdrainagepreventsincreased pressureon thebrain.
VPShunt hasat least threeparts:
1) Ventricular Catheter: Goesin thebrain
2) Valve: It controlsthepressurewithin thebrain.
3) Distal Catheter: Isunderneath theskin and connectstheother partsof theVP
shunt to aspacewithin thebody, usually theabdominal cavity (peritoneal
cavity). Thismay also beplaced behind theinfant’sear.
Thefluid flowsthrough thistubefrom thebrain into theabdominal cavity. In
thisarea, the body absorbsthefluid. It doesnot go into thestomach.
Advantages of Peritoneal Shunting.
1. If an infection develops, it isnot aspotentially life
threatening, aswith shuntsin thevenoussystem.
2. A largeamount of tubing can beplaceintra-
peritoneal to minimizetheneed for elective
3. Theoverall easein placing peritoneal shuntsin a
relatively short operation.
Problemsthat may Arisewith
Risks that may Arise:
1. Abdomen= Bowel twisting and excessfluid overload.
2. Blockageof theShunt
3. Brain Injury= Clots, Lossof Sensation, Memory Loss, Paralysis,
Seizures, Speech Problems, Headachescaused by overdraining, and
4. Bleeding, Problemswith anesthesia
5. Body may react negativebecauseof foreign material
6. Approximately 10% of shuntsfail within 10 yearsof placement.
7. May requireasmany as5 surgeries
Beforeshunt placement aCT imageof thebrain will show abuild up of CSF
in theventricles. Figure1. Dark areain themiddleisthebuild up of CSF.
Shunt implantation Goal isfor theshunt system to mimic what would occur in the
body naturally. CSF will bechained by theshunt, and theflow will beregulated so
that aconstant ICPismaintained.
After shunt placement Post-op CT scan image
Ventricleshavebeen drained and haveresumed normal size.
Whitespot in the
CSF enterstheshunt system through small holesor slitsnear
thetip of theproximal catheter.
AsCSF isproduced by thechoroid plexus, theshunt valve
will regulatetheamount of ICPby draining fluid from the
From theproximal catheter CSF flowsthrough thevalve
system and into thedistal catheter, drainstheCSF into
another areawhereit isreabsorbed either directly or
indirectly by thebloodstream. Ex. Peritoneal cavity with a
No harm becauseCSF isnormal. Reabsorbed by thesuperior
sagittal sinus, alargevenousstructurethat carriestheblood
flow away from thebrain.
Most shunt valvesareknown asdifferential pressurevalves.
A valveisself-regulating . They arecapable of gauging theamount of ICPand
can adjust to different pressuresbetween theventriclesand thedistal cavity that
Most common pressureratingsfor differential pressurevalvesare:
Extra-low pressure: 0-10mmH2O
Amount of fluid that isallowed to flow through theshunt valvedependson
thespecific design characteristicsof thevalve, aswell asthelevel rating by
Normal ICPrangefrom 50mmH2O-200mmH2O.
Infantsnormal ICPusually lessthan 60 and lessthan 40 for premature
Ventriculo-Atrial Shunt (VA)
Shunt tubing ispassed from thevalveto theneck whereit isinserted into a
vein. It isthen passed through thevein until thetip of thecatheter (shunt) is
in theatrium (achamber) of theheart.
In theheart, theCSF passesinto theblood stream and isfiltered along with
other body fluids.
Vascular shuntsfunctioned very well, but they wereproneto multiple
problemsincluding early and lateinfection, aswell asrare, potentially fatal
heart failuredueto blockageof blood vesselswithin thelungsby particlesof
blood clot flaking off theshunt'scatheter tip.
Theuseof theheart hasbeen largely abandoned asan initial choicebecause
of theseproblems, but it remainsaviablesecond option when infection or
surgery hasrendered theabdominal cavity unaccommodating of thedistal
Thechest cavity isanother cavity which can beused asa
backup to theabdominal cavity (ventriculopleural shunt).
Occasionally, thiscavity cannot resorb theCSF rapidly and
thelung becomescompressed by theexcessCSF resulting
in difficulty in breathing. Thecatheter must bemoved to a
different cavity issuch cases.
Pharmacological Acetazolamide(Diamox) and Furosemide(Lasix)
Diuretics. Given to control ICPand fluid retention. Temporary relief of
increased ICP, but areusually not helpful.
Used to decreasetheproduction of CSF by thechoroid plexusand serial
lumbar puncturesof thespineto drain CSF.
Serial lumbar puncturesarepredominantly used on prematurebaby’swho
had an intraventricular hemorrhage.
Drain excessCSF within theventriclesof an intraventricular hemorrhage
will block CSF flow within theventriclesor in thebasal cistein, causing
non-communicating hydrocephalusmaking serial lumbar puncture
Non-operational proceduresprovidemoderatesuccessuntil theclient is
Patient and Family Education
Parents, older children, friendsand roommatesmust betaught thesignsand
symptomsof shunt failure.
Persistent headache, emesis, lethargy, changein theneurological exam, visual
changessuch asdiplopiaor lossof conjugategaze, or swelling or rednessalong
theshunt valveor tubing aresignsthat your child needsmedical attention.
Children arecounseled to avoid contact sportsthat may causeinjury to the
shunt valveor head trauma.
Discouragepatientsfrom wearing purses, shoulder bags, or backpackson the
sidewhere theshunt tubing passesdown theneck. Continuouspressureon the
tubing can causeabreak or kink in thetubing.
Constipation may beafactor in thedevelopment of ashunt malfunction dueto
increased abdominal pressure, d/t decreased CSF drainage.
Medical Alert Bracelet.
Despite the many complications with
Susan, Jessica, and baby Isabelle, the
family does well
Isabelle continues to grow and learn
Jessica and Susan become even closer in
their marriage and say that Isabelle has
brought them so much joy and happiness
and she has taught them the importance of
Every day they feel blessed to have her in