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Hydrocephalus: A Complex Health Challenge

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Title: Hydrocephalus: A Complex Health Challenge


1
  • Complex Health Challenges
  • A Babys Life Story

2
  • Jessica Potts is a 32 year old woman who is
    pregnant for the first time. She is working full
    time
  • but she and her
  • partner, Susan,
  • are just making
  • ends meet with
  • the expenses for
  • IVF.

3
  • Jessica is an average height and weight woman as
    is Susan. When they decided to have a child it
    was
  • discovered that
  • Susan has
  • endometriosis and
  • fibroids and can not
  • get pregnant
  • therefore Jessica will
  • be carrying the child.

4
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 enhanced
    fertility
  • Assisted reproduction may be used

5
  • After being
  • unsuccessful
  • with artificial
  • insemination,
  • the couple opt
  • for in vitro
  • fertilization, Jessica is successful with IVF
    therapy on the first attempt.

6
IVF
  • In vitro fertilization
  • Successfully used in 1978

7
IVF Who May Benefit?
  • Women who have a blocked or damaged fallopian
    tube
  • Mild problem with male partners sperm
  • No cause identified for inability to conceive
  • Patients who have tried IUI or ovulation
    induction with no success

8
IVF Procedure
  • Fertility drugs
  • -gonadotrophin-releasing hormones (GnRH)
  • -human-menopausal gonadtrophin (hMG)
  • -human chorionic gonadotrophin (hCG).
  • Monitor blood hormone levels
  • Ultrasound scan
  • Remove ova through ultrasound-guided transvaginal
    retrieval or laparoscopy

9
IVF Procedure Contd
  • 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 lining

10
IVF Procedure Contd
  • 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
  • weeks

11
IVF
  • IVF treatment takes 4-6 weeks to complete
  • Success rates vary
  • Advantages
  • gives women with blocked, damaged, or missing
    fallopian tubes a chance to have a baby
  • Disadvantages
  • increased chances for multiple births, increase
    risk for miscarriage and other complications,
    hormones not closely monitored lead to ovarian
    hyperstimulation syndrome, ectopic pregnancy

12
(No Transcript)
13
MULTIPLE PREGNANCY
Multiply pregnancy occurs when the use of
ovulation inducing medication triggers the
release of multiple eggs, which, when fertilized
produce multiple embryos that are then implanted
14
Case Study
  • The couple live in a small town about half an
    hour from the city.
  • The medical clinic arranges for Jessicas
    prenatal care and any tests and assessments she
    may need for the duration of her pregnancy and
    delivery.

15
Case Study
  • Jessica and Susan are so happy to finally be
    pregnant, they refuse most prenatal tests.
  • Theyre just so happy to have a baby in their
    lives, they are not too concerned about genetics
    and anomalies with the baby.
  • They due, however, agree to have an ultrasound.

16
Non-invasive Prenatal Tests
17
Doppler Ultrasound
  • This noninvasive test measures blood flow in
    different parts of your baby's body such as the
    umbilical cord, brain, liver, and heart to help
    your caregiver assess your baby's health. It can
    be done at the same time as an ultrasound and
    uses the same equipment.
  • Student presentation for more information

18
Case Study
Mrs. Potts goes into spontaneous labor at 3am at
39 weeks gestation. Her labor and delivery are
unremarkable and Jessica and Susan welcome baby
Isabelle at 556am. The couple become concerned
when the nurses start whispering about the baby.
Jessica keeps asking them what the problem is,
but gets no immediate response. One nurse finally
tells Susan and Jessica that the doctor will be
in soon to talk to them. Eventually, the doctor
delivers the news that their precious little baby
girl infact has Down Syndrome. Jessica and Susan
react with anger. They are dissappointed that
they didnt go through with the serum screening
tests however, they must move forward now.
19
Down Syndrome
20
Down Syndrome
  • Is the most frequently occurring chromosomal
    disorder
  • Is universal across race and gender
  • Is caused by an error in cell division
  • Occurs at conception
  • Why it occurs is unknown

21
History
  • John Langdon Down (the father of down syndrome)
  • 1866 published an accurate description of a
    person with down syndrome
  • Jerome Lejeune
  • Identified down syndrome as a chromosomal
    anomaly

22
Incidence
  • Incidence increases with age
  • In Canada Down Syndrome occurs in approximately 1
    in 800 live births
  • Increases to 1 in 100 in second birth if your
    first child had Down Syndrome
  • In the US more than 350 000 people have Down
    Syndrome

23
Incidence of Down Syndrome
Maternal Age Incidence of Down Syndrome
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
24
Trisomony 21
  • Also called Downs Syndrome
  • Specific characteristics
  • Chromosome abnormality
  • Genetic testing

25
Down Syndrome
  • The presence of 47 chromosomes instead of 46
  • More specifically it is the presence of extra
    genetic material associated with the 21st
    chromosome. (Trisomy)
  • Caused by an error in cell division

26
Mitosis
  • The process of cell division involved in all cell
    growth, differentiation, and repair
  • The chromosomes of each cell duplicate
  • Two daughter cells are produced
  • They are diploid (contain 46 chromosomes in 23
    pairs)
  • Occurs in all cells except for the oocytes and
    sperm

27
Mitosis
28
Meiosis
  • Occurs in reproductive cells
  • There is a reduction in the number of chromosomes
    occurs (end up with 23 chromosomes)
  • Oocytes and sperm are referred to as being
    haploid (contain a single copy of each
    chromosome)
  • The paired chromosomes come together in
    preparation for cell division, portions cross
    over and genetic material is exchanged
  • recombination creates greater diversity in
    oocytes and sperm

29
Meiosis
30
Nondisjunction
  • Accounts for approximately 95 of down syndrome
    cases
  • A pair of chromosomes may fail to separate
    completely creating a sperm or oocyte that
    contains either 2 copies or no copies of a
    particular chromosome.
  • Trisomy when there are 2 copies
  • Monosomy when there are no copies
  • Prior to or at conception, a pair of 21st
    chromosomes in either the sperm or the egg fails
    to separate. As the embryo develops, the extra
    chromosome is replicated in every cell of the
    body

31
Trisomy
  • Down syndrome is a form of trisomy
  • there is extra genetic material on the 21st
    chromosome
  • Trisomy can occur on any chromosome but the only
    forms that are frequently seen in live births are
    on the 13, 18, and 21 chromosome

32
Mosaicism
  • Mosaicism occurs when nondisjunction of
    chromosome 21 takes place in one of the initial
    cell divisions after fertilization causing a
    person to have 46 chromosomes in some of their
    cells and 47 in others
  • This is the least common form of Down syndrome
  • accounts for only 1 to 2 percent of all cases

33
Translocation
  • Occurs when part of chromosome 21 breaks off
    during cell division and attaches to another
    chromosome, usually chromosome 14. While the
    total number of chromosomes in the cells remains
    46, the presence of an extra part of chromosome
    21 causes the characteristics of Down syndrome
  • Maternal age is not linked to the chance of
    having a baby with translocation. Most cases are
    sporadic, chance events, but in about one third
    of translocation cases, one parent is a carrier
    of a translocated chromosome. For this reason,
    the chance of translocation in a second pregnancy
    is higher than that seen in nondisjunction.
  • Accounts for 3 to 4 percent

34
Appearance
  • low nasal bridge
  • epicanthal folds (eyes)
  • protruding tongue
  • low set ears
  • poor muscle tone (hypotonia)
  • short stature
  • single crease across palm of hand
  • slightly flattened facial profile

35
Characteristics Conditions
  • 3/4 fetuses are spontaneously aborted
  • 20 die before the age of 10 r/t complications
  • have and IQ ranging from 25-50
  • 1-3 to1-2 have congenital heart defects
  • most common are an atrioventricular septal
    defect, persistant ductus arteriosus, and
    tetraology of fallot
  • decreased ability to fight respiratory infections
  • increased susceptibility to leukemia
  • usually develop Alzheimer symptoms by the age of
    40
  • increased risk for thyroid and vision problems
  • usually accompanied by some level of mental
    retardation
  • average life expectancy is 55

36
Treatment
  • Down syndrome is not treated
  • The symptoms are treated
  • It is important to encourage individuals with
    down syndrome to develop their gifts and talents
  • Early intervention programs can be initiated
  • Many individuals with down syndrome go to school
    (elementary, secondary, and post-secondary) and
    some adults are capable of working in the
    community.
  • With proper care individuals with down syndrome
    can lead healthy lives

37
Diagnosis
  • Prenatal screening (usually diagnosed here, but
    not in Jessicas case)
  • maternal serum screening
  • ultrasound (sonogram) screening
  • Diagnostic testing
  • chorionic villus sampling (CVS)
  • amniocentesis
  • percutaneous umbilical blood sampling (PUBS)
  • Diagnosis is made at the birth of baby Isabelle

38
Along with the diagnosis of Downs
  • The doctor informs Susan and Jessica that he
    would like to run some tests on baby Isabelle as
    it appears her head is abnormally large
  • Jessica and Susan are panicked at this point and
    the nurse tries to comfort the couples anxiety
  • The doctor refers to a neurologist who examines
    baby Isabelle

39
  • The neurologist is a stoic man in his late 60s
    and doesnt 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
  • Isabelle has

40
What is the Diagnosis?
  • Hydrocephalus

41
Structure of the Brain
42
Ventricles of the Brain
A Lateral View
An Anterior View
43
Ventricles of the Brain
  • Lateral Ventricles
  • Each cerebral hemisphere contains a large lateral
    ventricle Right and Left ventricle or First and
    Second ventricle
  • The septum pellucidum separates the two lateral
    ventricles
  • Third Ventricle
  • Located in the diencephalons
  • Two lateral ventricles are not directly connected
    to each other
  • communicates with the third ventricle through an
    interventricular foramen (foramen of Monro)
  • Third and Fourth ventricle are connected through
    a slender canal known as the mesencephalic
    aqueduct (the aqueduct of Sylvius) located in the
    mesencephalon
  • Fourth Ventricle
  • Superior portion lies between the posterior
    surface of the pons and the anterior surface of
    the cerebellum
  • Extends into the superior portion of the medulla
    oblongata
  • Then narrows and becomes continuous with the
    central canal of the spinal cord

44
Ependymal Cells
45
Ependymal Cells
  • Central canal narrow passageway in the spinal
    cord
  • In the brain, the passageway forms the ventricles
  • The central canal and ventricles are lined by a
    cellular layer of epithelial cells called the
    ependyma and are filled with cerebrospinal fluid
    (CSF)
  • During embroyonic development and early
    childhood, the free surface of ependymal cells
    are covered with cilia
  • The cilia persists in adults only within the
    ventricles of the brain, where they assist in the
    circulation of CSF
  • In other areas, the ependymal cells typically
    have scattered microvilli
  • Function
  • participate in the secretion of the CSF
  • sensory functions, such as monitoring the
    composition of the CSF

46
The Cranial Meninges
  • Layers
  • Cranial dura mater
  • Consists of outer (Endosteal) and inner
    (Meningeal) fibrous layers
  • Layers are typically separated by a slender gab
    that contains tissues fluids and blood vessels,
    including several large venous sinuses (Dural
    sinus).
  • The veins of the brain open into these sinuses,
    which deliver the venous blood to the internal
    jugular veins in the neck.
  • Arachnoid
  • Consists of the arachnoid membrane, an epithelial
    layer, and the cells and fibers of the arachnoid
    trabeculae that cross the subarachnoid space to
    the pia mater.
  • Arachnoid membrane covers the brain, providing a
    smooth surface that does not follow the brains
    underlying folds.
  • Pia mater
  • Sticks to the surface of the brain
  • It extends into every fold, and accompanies the
    branches of cerebral blood vessels as they
    penetrate the surface of the brain to reach
    internal structures.

47
Cranial Meninges
48
The Cranial Meninges
  • Function
  • To protect the brain
  • Dural folds provide additional stabilization and
    support to the brain.
  • Dural sinuses are large collecting veins
  • Three layers of dural folds
  • Falx cerebri
  • Superior sagittal sinus and the inferior sagittal
    sinus (venous sinuses) lie within this dural
    fold.
  • Tentorium cerebelli
  • Transverse sinus lies within the tentorium
    cerebelli.
  • Falx cerebelli

49
Dural Folds
50
Cerebrospinal Fluid
  • Function
  • Completely surrounds and bathes the exposed
    surfaces of the CNS and has several important
    functions
  • Cushioning Delicate Neural Structures
  • Supporting the Brain
  • The brain is suspended inside the cranium and
    floats in the CSF.
  • A human brain weighs about 1400 g in the air, but
    only about 50 g when supported by the CSF.
  • Transporting Nutrients, Chemical Messengers, and
    Waste Products
  • Ependymal lining is freely permeable (exception
    choroids plexus)
  • CSF is in constant chemical communication with
    the interstitial fluid of the CNS

51
CSF continued
  • Formation of CSF
  • Choroid plexus consists of a combination of
    specialized ependymal cells and permeable
    capillaries for the production of cerebrospinal
    fluid.
  • Location
  • Two extensive folds of the choroid plexus
    originate in the roof of the third ventricle and
    extend through the interventricular foramina.
    These folds cover the floors of the lateral
    ventricles.
  • In the inferior brain stem, a region of the
    choroid plexus in the roof of the fourth
    ventricle projects between the cerebrellum and
    the pons.
  • Specialized ependymal cells, interconnected by
    tight junctions, surround the capillaries of the
    choroid plexus.
  • The ependymal cells secrete CSF into the
    ventricles
  • Also remove waist products from the CSF and
    adjust its composition over time.

52
Circulation of Cerebral Spinal Fluid
53
CSF continued
  • Circulating CSF
  • Choroid plexus produces CSF at a rate of about
    500 ml/day
  • Total volume of CSF at any moments is
    approximately 150 ml/day
  • entire volume of CSF is replaced every eight
    hours
  • CSF circulates from the choroid plexus through
    the ventricles and the central canal of the
    spinal cord
  • As the CSF circulates, diffusion between it and
    the interstitial fluids (the extracellular fluids
    in most tissues is called interstitial fluid) of
    the CNS is unrestricted between and across the
    ependymal cells.
  • The CSF reaches the subarachnoid space through
    the two lateral apertures and the single median
    aperture, opening in the roof of the fourth
    ventricle.
  • CSF then flows through the subarachnoid space
    surrounding the brain, spinal cord, and cauda
    equine.
  • Fingerlike extensions of the arachnoid membrane,
    called arachnoid villi, penetrate the meningeal
    layer of the dura mater and extend into the
    superior sagittal sinus.
  • In adults, clusters of villi form large arachnoid
    granulations.
  • CSF is absorbed into the venous circulation at
    the arachnoid granulations where it is filtered
    and discarded by the body.

54
The Blood Brain Barrier
  • Neural tissue in the CNS is isolated from the
    general circulation by the blood-brain barrier
  • This barrier exists because the endothelial cells
    that line the capillaries of the CNS are
    extensively interconnected by tight junctions
  • These junctions prevent the diffusion of
    materials between adjacent endothelial cells
  • Only lipid-soluble compounds can diffuse across
    the endothelial cells membranes into the
    interstitial fluid of the brain and spinal cord
  • Astrocyte Cells
  • Restricted permeability characteristics of the
    endothelial lining of brain capillaries are
    depended on chemicals secreted by the astrocytes
  • cells that are in close contact with CNS
    capillaries
  • Outer surfaces of the endothelia cells are
    covered by the processes of astrocytes
  • Release chemicals that control the permeability
    of the endothelium to various substances
  • If damaged or stop stimulating the endothelial
    cells, the blood-brain barrier disappears.

55
Astrocyte Cells
56
Blood-CSF Barrier
  • Choroids plexus
  • Not part of the neural tissue of the brain
    therefore no astrocytes are in contact with the
    endothelial cells.
  • As a result, capillaries in the choroids plexus
    are highly permeable
  • Blood-CSF barrier
  • Substances do not have free access to the CNS
  • Specialized ependymal cells interconnected by
    tight junctions, surround the capillaries of the
    choroids plexus
  • Transport across the blood-brain and blood-CSF
    barriers is selective and directional
  • Even the passage of small ions (sodium, hydrogen,
    potassium, or chloride) is controlled
  • Some organic compounds are readily transported,
    and others cross only in minute amounts.

57
  • Susan and Jessica and angry and bitter
  • They want to know why this diagnosis of
    Hydrocephalus wasnt 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

58
What is Hydrocephalus?
  • The term hydrocephalus is derived from two words
  • "hydro" meaning water
  • "cephalus" referring to the head
  • A condition in which excess CSF builds up within
    the ventricles of the brain or in the
    subarachnoid space and may increase pressure
    within the head
  • Can occur at any age
  • most common in infants and adults age 60 and
    older
  • In most instances, hydrocephalus is a lifelong
    condition in that the patient is treated rather
    than "cured"
  • If left untreated in the infant, they can suffer
    from some degree of mental retardation and/or
    motor dysfunction.

59
Epidemiology
  • In the United States, a little over 1 in 1000
    births are affected by hydrocephalus.
  • As high as 1 in 500 births.
  • Hydrocephalus is one of the most common "birth
    defects" and afflicts in excess of 10,000 babies
    each year.
  • Studies by the World Health Organization show
    that one birth in every 2,000 result in
    hydrocephalus.
  • There are 70,000 discharges a year from hospitals
    in the United States with a diagnosis of
    hydrocephalus.
  • More than 50 of hydrocephalus cases are
    congenital.
  • As many as 75 of children with hydrocephalus
    will have some form of motor disability.
  • Over the past 25 years, death rates associated
    with hydrocephalus have decreased from 54 to 5
    intellectual disabilitity has decreased from 62
    to 30.
  • Ocular gaze and movement disorders are found in
    approximately 25 to 33 of children with
    hydrocephalus.
  • About 80 of hydrocephalus patients are born with
    other defects.

60
Types of Hydrocephalus
  • Congenital when the condition exists at birth
  • Acquired when it occurs as the result of a
    trauma to the brain after birth.

61
Pathophysiology
  • Impaired absorption of CSF from the subarachnoid
    space occurs when an obstructive process disrupts
    the flow of CSF through the subarachnoid space.
  • The fluid does not reach the convex portion of
    the cerebrum, where the arachnoid granulations
    are located.
  • With acute hydrocephalus, there is increased ICP
    that has a rapid onset. The patient can
    deteriorate rapidly into a deep coma if it is not
    treated promptly.
  • ICP rises if production of CSF exceeds
    absorption. This occurs if CSF is overproduced,
    resistance to CSF flow is increased, or venous
    sinus pressure is increased.

62
  • CSF production falls as ICP rises. Compensation
    may occur through transventricular absorption of
    CSF and also by absorption along nerve root
    sleeves.
  • Temporal and frontal horns dilate first, often
    asymmetrically.
  • This may result in elevation of the corpus
    callosum, stretching or perforation of the septum
    pellucidum, thinning of the cerebral mantle, or
    enlargement of the third ventricle downward into
    the pituitary fossa (which may cause pituitary
    dysfunction).

63
(No Transcript)
64
Causes of Hydrocephalus
This is grouped into 3 main causes 1. 
Excessive secretion of CSF by the choroid plexus
as in cases of choroid plexus papilloma (rare,
bening tumour) or carcinoma. This is a rare
cause.
Choroid Plexus Papilloma
65
  • 2.  Blockage to CSF circulation. This could be
    at any level of the CSF circulation.
  • It could be at the level of the foramen of Monro
    where we there is unilateral or bilateral
    coverage of the foramen of Monro giving
    dilatation of one or both lateral ventricles.
  • This is commonly seen in the colloid cyst and
    tumours of the third ventricle.
  • Suprasellar lesion as suprasellar arachnoid cyst
    or hypothalamic tumours (craniopharyngioma
    congenital pituitary tumour).
  • Posterior fossa tumours are a common cause of
    obstructive hydrocephalus due to blockage of the
    4th ventricle.
  • Medulloblastoma, cystic astrocytoma and
    ependymoma can all lead to obstructive
    hydrocephalus.
  • 3. Poor secretion of CSF into the venous sinuses
    caused by scarring of the arachnoid villi and is
    commonly seen after meningitis or hemorrhage.
  •  

66
  • Forms of Hydrocephalus

67
Causes of Hydrocephalus
68
  • Congenital Causes in Infants and Children
  • Characterized by an increased volume of CSF
  • May be caused by
  • A blockage within the ventricular system in
    which the CSF flows
  • An imbalance in production of the CSF
  • Reduced reabsorption of the CSF that results in
    enlargement of the ventricles, and increased ICP
  • This pressure within the ventricular system
    pushes and compresses the brain against the skull
    cavity.
  • Before the cranial sutures fuse, the skull can
    increase to accommodate the additional
    space-occupying volume to preserve neuronal
    function.

69
Stenosis of the Aqueduct of Sylvius Due to
malformation This is responsible for 10 of all
cases of hydrocephalus in newborns, and is the
most common cause. Bickers-Adam
Malformation 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.
70
Dandy-Walker Malformation
  • This affects 2-4 of newborns with
    hydrocephalus.
  • Dandy-Walker Malformation is a rare malformation
    of the brain that is present at birth
    (congenital).
  • Dandy-Walker Malformation is a form of
    "Obstructive" or "Internal Noncommunicating
    Hydrocephalus," meaning that the normal flow of
    cerebrospinal fluid is blocked resulting in the
    widening of the ventricles.
  • It is characterized by an abnormally enlarged
    space at the back of the brain (cystic 4th
    ventricle) that interferes with the normal flow
    of cerebrospinal fluid through the openings
    between the ventricle and other parts of the
    brain (foramina of Magendia and Luschka).
  • Excessive amounts of fluid accumulate around the
    brain and cause abnormally high pressure within
    the skull, swelling of the head (congenital
    hydrocephalus), and neurological impairment.
    Motor delays and learning problems may also
    occur.

71
Arnold-Chiari Malformation
  • Chiari malformations (CMs) are structural
    defects in the cerebellum, the part of the brain
    that controls balance.
  • The cerebellum and brainstem can be pushed
    downward.
  • The resulting pressure on the cerebellum can
    block the flow of cerebrospinal fluid and can
    cause a range of symptoms including dizziness,
    muscle weakness, numbness, vision problems,
    headache, and problems with balance and
    coordination.

72
  • Is accompanied by a myelomeningocele-a form of
    spina bifida that occurs when the spinal canal
    and backbone do not close before birth, causing
    the spinal cord to protrude through an opening in
    the back.
  • This can cause partial or complete paralysis
    below the spinal opening, and hydrocephalus.

73
Agenesis of the Foramen of Monro
  • AKA Interventricular foramen.
  • Narrowing of the foramen of Monroe.
  • Since the foramen narrows, this leads to
    increased pressure to push the CSF through the
    foramen of Monroe.

74
Congenital Toxoplasmosis
  • Group of symptoms and characteristics caused by
    infection of the fetus with the organism
    Toxoplasma gondii.
  • Fetal infection results when a nonimmune
    pregnant woman is initially infected with
    toxoplasmosis (from certain foods, cat feces, or
    if she has a history of toxoplasmosis during
    previous pregnancies).
  • Congenital toxoplasmosis is characterized by
    damage to the eyes, nervous system, skin, and
    ears.
  • Can occur as a result of ingestion of raw or
    inadequately cooked infected meat, ingestion of
    oocysts, an environmentally resistant form of the
    organism that cats pass in their feces, with
    exposure of humans occurring through exposure to
    cat litter or soil (e.g., from gardening or
    unwashed fruits or vegetables), and a newly
    infected pregnant woman passing the infection to
    her unborn fetus.

75
MRIs Congenital Causes of Hydrocephalus
Arnold-Chiari Malformation
Dandy-Walker Malformation
76
  • Acquired Causes in Infants and Children
  • Mass lesions account for 20 of all cases of
    hydrocephalus in children. These are usually
    tumors (eg, medulloblastoma, astrocytoma), but
    cysts, abscesses, or hematoma also can be the
    cause.
  • Intraventricular hemorrhage can be related to
    prematurity, head injury, or rupture of a
    vascular malformation.
  • Infections Meningitis (especially bacterial)
    and, in some geographic areas, cysticercosis can
    cause hydrocephalus.
  • Increased venous sinus pressure This can be
    related to achondroplasia, some craniostenoses,
    or venous thrombosis.
  • Iatrogenic (result of medical interventions)
    Hypervitaminosis A, by increasing secretion of
    CSF or by increasing permeability of the
    blood-brain barrier, can lead to hydrocephalus.
  • Idiopathic

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Signs and Symptoms of Hydrocephalus
  • Clinical features of hydrocephalus
  • are influenced by the following
  • Patient's age
  • Cause
  • Location of obstruction
  • Duration
  • Rapidity of onset

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Symptoms in Infants
  • Poor feeding
  • Irritability
  • Reduced activity
  • Vomiting
  • Seizures
  • Bulging fontanelle
  • Thin, shiny skin over fontanelles
  • Papilledema (swelling of the eyes nerves) and
    later optic atrophy

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  • Symptoms in Children
  • Slowing of mental capacity
  • Headaches (initially in the morning) that are
    more significant than in infants because of skull
    rigidity
  • Neck pain suggesting tonsillar herniation
  • Vomiting, more significant in the morning
  • Blurred vision - Consequence of papilledema
    (swelling of the eyes nerves) and later of optic
    atrophy
  • Double vision - Related to unilateral or
    bilateral sixth nerve palsy
  • (affects abducens cranial nerve, and eyes cannot
    turn outward beyond midline, double vision also
    occurs, but disappears when one eye is closed)

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  • Stunted growth and sexual maturation from third
    ventricle dilatation This can lead to obesity
    and to precocious or delayed onset of puberty.
  • Difficulty in walking secondary to spasticity
    This affects the lower limbs preferentially
    because the periventricular pyramidal tract is
    stretched by the hydrocephalus.
  • Drowsiness

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Physical Assessment of A Neonate
Similar to adult head-to-toe assessment, with
the following exceptions
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  • Vital signs
  • Skin and hair Lanugo, vernix caseosa (thick,
    cheezy protective integumentary deposit that
    consists of sebum, and shed epithelial cells).
    Stork bites (back of neck, lower occiput, upper
    eyelids, and upper lip).
  • Head, Face and Eyes Infants have anterior or
    posterior fontanelles, and they should not bulge
    or sink.

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  • Newborns dont produce tears until 2-3 months.
  • The Eustachian tube is more horizontal, wider,
    and shorter, thus can increase likelihood of
    middle ear infections.
  • Thorax and Lungs up to 3-4 months abdominal
    breathing. Measure chest circumference.
  • Cardiovascular Infants have a higher
    circulating blood volume
  • Abdomen Liver is proportionately larger.
  • Musculoskeletal Bone growth ends at 20 (when
    epiphysis closes).

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  • Neurological Apgar Scores Method to reassess
    need for newborn resuscitation in the delivery
    room.
  • Given at 1 and 5 minutes following birth. Score
    of 8-10 Newborn in good condition, 4-7 Moderately
    depressed newborn, 0-3 indicates severe
    depression, and needs immediate resuscitation
    (See overhead)
  • Reflexes Rooting, sucking, palmar grasp, tonic
    neck, stepping, plantar grasp, Babinskis, and
    Moro.

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  • Genitourinary System During infancy, the
    bladder is located in between the symphysis pubis
    and the umbilicus. Monitor I O.
  • Gastrointestinal Meconium stools, then after 3
    days, yellow coloured. Important to monitor bowel
    function and I O to ensure that infant does not
    become dehydrated.
  • Inspection of genitalia

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Clinical Manifestations Upon Physical Assessment
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  • Infants
  • Head enlargement Head circumference is in the
    98th percentile for the age or greater.
  • Dysjunction of sutures This can be seen or
    palpated.
  • Dilated scalp veins The scalp is thin and shiny
    with easily visible veins.
  • Tense fontanelle The anterior fontanelle in
    infants who are held erect and are not crying may
    be excessively tense.

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  • Setting-sun sign In infants it is characteristic
    of increased ICP. Both ocular globes are deviated
    downward, the upper lids are retracted, and the
    white sclerae may be visible above the iris.
  • Increased limb tone Spasticity affects the lower
    limbs. The cause is stretching of the
    periventricular pyramidal tract fibers by
    hydrocephalus.

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  • Children
  • Papilledema if the raised ICP is not treated,
    this can lead to optic atrophy and vision loss.
  • Failure of upward gaze This is due to pressure
    on the tectal plate through the suprapineal
    recess.
  • Macewen sign A "cracked pot" sound is noted on
    percussion of the head.

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  • Unsteady gait This is related to spasticity in
    the lower extremities.
  • Large head Sutures are closed, but chronic
    increased ICP will lead to progressive abnormal
    head growth.
  • Unilateral or bilateral sixth nerve palsy
    (affects abducens cranial nerve, and eyes cannot
    turn outward beyond midline, double vision also
    occurs, but disappears when one eye is closed) is
    secondary to increased ICP.

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Getting the Diagnosis
  • With newborns, hydrocephalus is detected almost
    immediately as the child's head may be larger
    than normal (macrocephaly). However, with older
    children or adults, hydrocephalus usually starts
    to reveal itself with a variety of signs and
    symptoms weeks or months before it is detected.
  • It may be detected by signs and symptoms of
    increased cranial pressure.

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CT and MRI
  • X-Rays do not provide enough contrast to see the
    tissues of the brain.
  • CT ? Clearer pictures of the bodies organs,
    tissues and bones. Approx. 2-5 minutes.
  • MRI ? Internal structures can be seen. Approx. an
    hour in length.
  • Provide a clearer view of gray and white matter
    of the brain, as well as the vascular system.
    Primary use for neurosurgeons.
  • CT and MRI scans take pictures of the complete
    cranial and intracranial anatomy, including the
    subarachnoid spaces and the structures of the
    posterior fossa.
  • Taken laterally and sagitally (front-back)

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Diagnosis of Hydrocephalus
  • Abnormal Head Growth (Macrocephaly)
  • Infants and small children? primary indicator.
    Kids sutures have not fused together yet.
  • Continue to monitor the growth of the childs
    head until the child reaches the age of 6 or 7.
  • Signs and Symptoms
  • Irritable
  • High pitched cry/scream
  • Split sutures of the skull
  • Distended veins in the scalp-bulging or widening
    of the fontanels
  • Absence of up ward's gaze, known as sun setting
    ? usually in acute non-communicating
    hydrocephalus.
  • Impaired lateral gaze (Sun setting one or both
    eyes)
  • Loss of vision-weakness or spasticity of limbs.

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Initial Diagnosis
  • Initially, when one or more symptoms become
    evident.
  • Infant ? Childs head is bulging or larger than
    normal
  • Child ? Painful headaches, gait disorder or
    vision problems
  • Should be referred to a neurosurgeon
  • Neurological Examination ? History of milestones,
    as well as a physical examination for
    neurological deficits.

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Full-Term Infant
  • 1 year or older ? Examine the infant to see if
    they are reaching mental and physical
    developmental milestones.
  • Mental Milestones
  • Is your infant communicating verbally?
  • Is your infant performing well in school?
  • Has your infant fallen behind his peers in recent
    months?
  • Is your child having a hard time remembering
    things?
  • Have you noticed any changes in personality in
    the last few weeks/months?

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Continued
  • Physical Milestones
  • Has your child started to show signs of walking
    by the time they were 1?
  • Is your infants gait steady or unbalanced?
  • Does your child drift to the side while they
    walk?
  • Get the child to balance on one foot, with their
    eyes closed. Place both feet together side by
    side to maintain balance.
  • Place the index finger in front of the face and
    ask to follow movement. (testing for paralysis of
    the abducens- 6th cranial nerve). Controls side
    to side (lateral) movement.
  • Walk heal-to-heal. If child has difficulty could
    be an indicator of pressure on the cerebellum.
  • Check plantar, Babinski reflex.
  • If the big toe moves upward, results an extensor
    response or Babinski reflex. Babinski reflex is a
    clear indication of some form of brain or spinal
    cord disease. Usually skip this till the infant
    is at least 1 year old because it is usually
    positive whether the infant has it or not.
  • Pronator drift Close eyes while standing, extend
    both arms in front with palms up. See if one arm
    wavers or drifts. Indication of injury to the
    motor areas of the brain.

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Effects on Family Dynamics
  • Emotions can range from worry to fear, as well as
    resentment and jealousy.
  • Children also have active imaginations.
  • Usually their emotions are worse than their
    reality perceives.
  • Talk through their fears.
  • Siblings may feel completely overwhelmed.
  • Resentment and jealousy are common feelings
    experienced by siblings.
  • Let them know they are loved and valued.

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Preventing Childhood Hydrocephalus
  • Protecting the head of the infant or child from
    injury by handling the child carefully may help
    prevent the development of injury induced
    hydrocephalus.
  • Prompt treatment of infections such as meningitis
    and others associated with hydrocephalus may
    reduce the risk of developing the disease.
  • Women who take cytomegalovirus or toxoplasmosis
    acquired by a mother during pregnancy may cause
    hydrocephalus. May reduce the risk of being
    infected by toxoplasmosis by
  • Cooking meet and veggies carefully.
  • Cleaning contaminated knives and cutting services
    properly.
  • Avoid handling cat litter, or wearing gloves when
    cleaning the litter box.
  • Lymphocytic choriomengitis virus (LCV) which pet
    rodants (mice) often carry can lead to
    hydrocephalus in pregnancy.
  • Infection with chickenpox or mumps during or
    right after pregnancy may also lead to
    hydrocephalus in the baby.

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Role of Nurses
  • Bedside nurse is in a unique position to have an
    impact on patients and families lives.
  • Nurse needs to empower and educate the family of
    the importance of aseptic technique when taking
    care of the childs surgical site.
  • Stress the importance to the family that their
    child should maintain optimal health with proper
    nutrition and exercise.
  • Needs to supply the families with life-saving
    information of the signs and symptoms of a shunt
    malfunction and or infection.

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Multidisciplinary Workers
  • Nutritional Support
  • Physical Therapy
  • Occupational Therapy
  • Neurosurgeon
  • Pediatrician
  • Nurses
  • Ophthalmologist

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Isabelles scalp, over the anterior fontanelle,
is shiny and thin and the tiny veins are
prominent. Isabelle is then sent to the NICU to
be closely monitored for complications associated
with increased intracranial pressure. Exactly 2
weeks after Isabelle was born, she undergoes
surgery to insert a VP shunt. The surgery went
very well with no complications.
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Ventriculo-Peritoneal Shunts In Infants
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Hydrocephalus Shunt Statistics
  • There are 25,000 shunt operations performed each
    year in the United States. Of those, some 18,000
    are initial shunt placements.
  • Some 85 of people with shunts have had at least
    two shunt operations.
  • Studies show that the risk of shunt failure in an
    infant's first year is 30.
  • Shunts are revised about 2 times in the first ten
    years of use per patient.
  • 95 of shunt infections occur within 3 to 5 days
    of surgery.
  • The reported frequency of shunt infection varies
    from 1.5 to 39 with an average of 10 to 15.
  • More than 50 of staphylococcal infections occur
    with in 2 weeks of the operation, and 70 of
    infections occur within 2 months.
  • The overall complication rate of CSF shunts
    remains quite high 25 to 60.
  • Shunt malfunctions occur in about two to 40 of
    cases.

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What is a Ventriculo-Peritoneal Shunt?
  • Primary Goal of a VP shunt To ensure on a
    regular basis that the shunt continues to
    function!
  • A VP is a long, plastic tube that allows fluid to
    drain from the brain to another part of the body
    (Peritoneal Cavity). This drainage prevents
    increased pressure on the brain.
  • VP Shunt has at least three parts
  • 1) Ventricular Catheter Goes in the brain
  • 2) Valve It controls the pressure within the
    brain.
  • 3) Distal Catheter Is underneath the skin and
    connects the other parts of the VP shunt to a
    space within the body, usually the abdominal
    cavity (peritoneal cavity). This may also be
    placed behind the infants ear.
  • The fluid flows through this tube from the brain
    into the abdominal cavity. In this area, the body
    absorbs the fluid. It does not go into the
    stomach.

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Advantages of a VP Shunt
  • Advantages of Peritoneal Shunting.
  • If an infection develops, it is not as
    potentially life threatening, as with shunts in
    the venous system.
  • A large amount of tubing can be place
    intra-peritoneal to minimize the need for
    elective lengthening.
  • The overall ease in placing peritoneal shunts in
    a relatively short operation.

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Problems that may Arise with VP Shunts
  • Risks that may Arise
  • Abdomen Bowel twisting and excess fluid
    overload.
  • Blockage of the Shunt
  • Brain Injury Clots, Loss of Sensation, Memory
    Loss, Paralysis, Seizures, Speech Problems,
    Headaches caused by overdraining, and Mechanical
    Failure
  • Bleeding, Problems with anesthesia
  • Body may react negative because of foreign
    material
  • Approximately 10 of shunts fail within 10 years
    of placement.
  • May require as many as 5 surgeries

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How Shunts Work
  • Before shunt placement a CT image of the brain
    will show a build up of CSF in the ventricles.
    Figure 1. Dark area in the middle is the build up
    of CSF.

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How Shunts Work
  • Shunt implantation ? Goal is for the shunt system
    to mimic what would occur in the body naturally.
    CSF will be chained by the shunt, and the flow
    will be regulated so that a constant ICP is
    maintained.
  • After shunt placement ?Post-op CT scan image
  • Ventricles have been drained and have resumed
    normal size.

White spot in the middle is the shunt.
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  • CSF enters the shunt system through small holes
    or slits near the tip of the proximal catheter.
  • As CSF is produced by the choroid plexus, the
    shunt valve will regulate the amount of ICP by
    draining fluid from the ventricles.
  • From the proximal catheter CSF flows through the
    valve system and into the distal catheter, drains
    the CSF into another area where it is reabsorbed
    either directly or indirectly by the bloodstream.
    Ex. Peritoneal cavity with a VP shunt.
  • No harm because CSF is normal. Reabsorbed by the
    superior sagittal sinus, a large venous structure
    that carries the blood flow away from the brain.

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VP Shunt Insertion
Ventricular Catheter
Reservoir
Valve
Ventriculoarterial Shunt
Stastic Tubing
Ventriculoperitoneal Shunt
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Valve Pressure Ratings
  • Valve Pressure Settings
  • Most shunt valves are known as differential
    pressure valves.
  • A valve is self-regulating . They are capable of
    gauging the amount of ICP and can adjust to
    different pressures between the ventricles and
    the distal cavity that the shunt drains into.
  • Most common pressure ratings for differential
    pressure valves are
  • Extra-low pressure 0-10mmH2O
  • Low 10-50mmH2O
  • Medium 51-100mmH2O
  • High 101-200mmH2O
  • Amount of fluid that is allowed to flow through
    the shunt valve depends on the specific design
    characteristics of the valve, as well as the
    level rating by the manufacturer.
  • Normal ICP range from 50mmH2O-200mmH2O.
  • Infants normal ICP usually less than 60 and less
    than 40 for premature infants.

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VP Shunt Vs. VA Shunt
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Ventriculo-Atrial Shunt (VA)
  • Shunt tubing is passed from the valve to the neck
    where it is inserted into a vein. It is then
    passed through the vein until the tip of the
    catheter (shunt) is in the atrium (a chamber) of
    the heart.
  • In the heart, the CSF passes into the blood
    stream and is filtered along with other body
    fluids.
  • Vascular shunts functioned very well, but they
    were prone to multiple problems including early
    and late infection, as well as rare, potentially
    fatal heart failure due to blockage of blood
    vessels within the lungs by particles of blood
    clot flaking off the shunt's catheter tip.
  • The use of the heart has been largely abandoned
    as an initial choice because of these problems,
    but it remains a viable second option when
    infection or surgery has rendered the abdominal
    cavity unaccommodating of the distal shunt
    catheter.

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Ventriculopleural Shunt
  • The chest cavity is another cavity which can be
    used as a backup to the abdominal cavity
    (ventriculopleural shunt).
  • Occasionally, this cavity cannot resorb the CSF
    rapidly and the lung becomes compressed by the
    excess CSF resulting in difficulty in breathing.
    The catheter must be moved to a different cavity
    is such cases.

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Non-Surgical Treatments
  • Pharmacological ? Acetazolamide (Diamox) and
    Furosemide (Lasix) Diuretics. Given to control
    ICP and fluid retention. Temporary relief of
    increased ICP, but are usually not helpful.
  • Used to decrease the production of CSF by the
    choroid plexus and serial lumbar punctures of the
    spine to drain CSF.
  • Serial lumbar punctures are predominantly used on
    premature babys who had an intraventricular
    hemorrhage.
  • Drain excess CSF within the ventricles of an
    intraventricular hemorrhage will block CSF flow
    within the ventricles or in the basal cistein,
    causing non-communicating hydrocephalus making
    serial lumbar puncture ineffective.
  • Non-operational procedures provide moderate
    success until the client is shunted.

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Patient and Family Education
  • Parents, older children, friends and roommates
    must be taught the signs and symptoms of shunt
    failure.
  • Persistent headache, emesis, lethargy, change in
    the neurological exam, visual changes such as
    diplopia or loss of conjugate gaze, or swelling
    or redness along the shunt valve or tubing are
    signs that your child needs medical attention.
  • Children are counseled to avoid contact sports
    that may cause injury to the shunt valve or head
    trauma.
  • Discourage patients from wearing purses ,
    shoulder bags, or backpacks on the side where the
    shunt tubing passes down the neck. Continuous
    pressure on the tubing can cause a break or kink
    in the tubing.
  • Constipation may be a factor in the development
    of a shunt malfunction due to increased abdominal
    pressure, d/t decreased CSF drainage.
  • Medical Alert Bracelet.

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  • 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 life
  • Every day they feel blessed to have her in their
    lives

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Any Questions??
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  • The End
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