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					 Complex Health
   Challenges:
A Baby’s Life Story
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.
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.
         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
After being
unsuccessful
with artificial
insemination,
the couple opt
for in vitro
fertilization, Jessica is successful
  with IVF therapy on the first
  attempt.
                                IVF
   In vitro fertilization
   Successfully used in 1978
IVF: Who May Benefit?
          Women who have a blocked or
           damaged fallopian tube
          Mild problem with male
           partner’s sperm
          No cause identified for inability
           to conceive
          Patient’s who have tried IUI or
           ovulation induction with no
           success
                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
        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
    lining
        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
weeks
                           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
        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
              Case Study
   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.
               Case Study
   Jessica and Susan are so happy to finally
    be pregnant, they refuse most prenatal
    tests.
   They’re 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.
Non-invasive Prenatal Tests
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…
                      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 5:56am. 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 didn’t go
through with the serum screening tests however, they must
move forward now.
Down Syndrome
     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
                        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
                     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
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
              Trisomony 21

   Also called
    Down’s Syndrome
   Specific
    characteristics
   Chromosome
    abnormality
   Genetic testing
             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
                       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
Mitosis
                          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
Meiosis
                      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
                          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
                        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
                       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
                   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
                           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
                      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
                   Diagnosis
   Prenatal screening (usually diagnosed here,
    but not in Jessica’s 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
    Along with the diagnosis of
             Down’s
   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
   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
   Isabelle has…
 What is the Diagnosis?



Hydrocephalus
Structure of the Brain
 Ventricles of the Brain




A Lateral View     An Anterior View
               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
Ependymal Cells
                   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
                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 brain’s 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.
Cranial Meninges
              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
Dural Folds
                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
                        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.
Circulation of Cerebral Spinal
            Fluid
                            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.
            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.
Astrocyte Cells
                   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.
   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
          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.
                            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.
             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.
                       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.
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).
           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
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.
         Forms of Hydrocephalus
    Forms of Hydrocephalus                       Description
                                 (non-obstructive hydrocephalus) caused by
 Communicating Hydrocephalus     inadequate absorption of CSF when the
                                 ventricular pathways are not obstructed.

                                 (obstructive hydrocephalus) caused by
Noncommunicating Hydrocephalus   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
   Obstructive hydrocephalus     used to contrast the hydrocephalus caused by
                                 overproduction of CSF.


                                 Stabilization of known ventricular
                                 enlargement, probably secondary to
     Arrested hydrocephalus      compensatory mechanisms. These patients
                                 may decompensate, especially following
                                 minor head injuries.
Causes of Hydrocephalus
 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.
   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.
            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.
          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.
• 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.
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.
                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.
MRI’s: Congenital Causes of
      Hydrocephalus




  Arnold-Chiari    Dandy-Walker
  Malformation
                    Malformation
 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
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
               Symptoms in Infants


 Poor feeding
 Irritability
 Reduced activity
 Vomiting
 Seizures
 Bulging fontanelle
Thin, shiny skin over fontanelles
Papilledema (swelling of the eye’s nerves) and later optic
atrophy
            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 eye’s
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)
 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
Physical Assessment of A Neonate
          *Similar to adult head-to-toe
   assessment, with the following exceptions:*
• 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.
• Newborns don’t 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).
• 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, Babinski’s, and Moro.
• 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
 Clinical Manifestations
Upon Physical Assessment
                    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.
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.
                  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.
 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.
         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.
                        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)
      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 child’s 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.
              Initial Diagnosis
   Initially, when one or more symptoms become
    evident.
   Infant  Child’s 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.
                      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?
                         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.
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.
                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.
                      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 child’s 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.
      Multidisciplinary Workers
   Nutritional Support
   Physical Therapy
   Occupational Therapy
   Neurosurgeon
   Pediatrician
   Nurses
   Ophthalmologist
Isabelle’s 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.
Ventriculo-Peritoneal Shunts
         In Infants
    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.
    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 infant’s 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.
Advantages of a VP Shunt
   Advantages of Peritoneal Shunting.
    1. If an infection develops, it is not as potentially
       life threatening, as with shunts in the venous
       system.
    2. A large amount of tubing can be place intra-
       peritoneal to minimize the need for elective
       lengthening.
    3. The overall ease in placing peritoneal shunts in a
       relatively short operation.
    Problems that may Arise with
             VP Shunts
   Risks that may Arise:
    1.   Abdomen= Bowel twisting and excess fluid overload.
    2.   Blockage of the Shunt
    3.   Brain Injury= Clots, Loss of Sensation, Memory Loss, Paralysis,
         Seizures, Speech Problems, Headaches caused by overdraining, and
         Mechanical Failure
    4.   Bleeding, Problems with anesthesia
    5.   Body may react negative because of foreign material
    6.   Approximately 10% of shunts fail within 10 years of placement.
    7.   May require as many as 5 surgeries
                 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.
                   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.
   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.
      VP Shunt Insertion
                  Ventricular Catheter
    Reservoir



      Valve




                              Ventriculoarterial
                                   Shunt




Stastic Tubing                 Ventriculoperitoneal
                                      Shunt
            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.
VP Shunt Vs. VA Shunt
    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.
      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.
         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 baby’s
    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.
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.
   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
Any Questions??
The End

				
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