• Hydrocephalus
o Definition
o Causes
o Pathophysiology
o Assessment and common findings
o Management
• Neural tube / spina bifida Wong 1154 Juta 885
o Classification
o Assessment and common findings
o Management
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Congenital conditions
1. The nervous system:
Congenital conditions
Hydrocephalus &
Neural tube/Spina bifida Wong
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2. Outcomes
At the end of the unit, the student
will be able to:
• Apply knowledge regarding: patho-physiology, disease
process, clinical manifestations, specific diagnostic and
therapeutic interventions (diagnostic tests and
examinations) of Congenital diseases.
• Assess, relate and apply the scientific process of nursing,
provision and facilitation of nursing care.
• Evaluate, analyse and solve problems in familiar and
unfamiliar context in the Comprehensive Health Care
system.
• Understand the relationship between social, cultural and
economic factors that may impact significantly on the
health status of clients / patients and groups.
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3. Hydrocephalus- pg.884
• Hydrocephalus is characterised by head enlargement.
• Hydrocephalus can be defined broadly as a disturbance
of cerebrospinal fluid (CSF) formation, flow, or
absorption, leading to an increase in volume occupied
by this fluid in the central nervous system (CNS).
• Accumulation of CSF in the ventricles.
• Choroid plexus:
– a network of blood vessels in each ventricle of the brain,
producing the cerebrospinal fluid.
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11. Cerebrospinal fluid & its functions
• Cerebrospinal fluid is a clear, colourless body fluid
found in the brain and spinal cord. It is produced
in the choroid plexuses of the ventricles of the
brain.
– CSF protects brain and spinal cord from trauma.
– CSF supplies nutrients to nervous system tissue.
– CSF removes waste products from cerebral
metabolism.
– VIDEO* Cerebrospinal fluid flow with audio
description
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12. Causes of Hydrocephalus
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• Developmental abnormalities of the brain and
CSF system
• Failure of the arachnoid to develop properly
• Overproduction of CSF
• Developmental defects in the spinal cord
• May begin during adulthood especially following
trauma, hyperplasia or tumours
• Video* - Hydrocephalus explained
13. Types of Hydrocephalus- pg.884
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• Communicating hydrocephalus
– The CSF is able to move out from the ventricles
into the subarachnoid space, but the drainage
channels that allow movement of CSF back into
the circulation are blocked.
– Associated with conditions that cause scarring of
the meninges such as meningitis or intracranial
haemorrhage in the perinatal period.
– Occasionally the arachnoid villi that allow the CSF
to return to the blood are underdeveloped.
14. Types of Hydrocephalus- pg.884
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• Communicating hydrocephalus
15. Types of Hydrocephalus
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• Non - Communicating hydrocephalus
– The CSF is not able to move from the ventricular
system to the subarachnoid space.
– Congenital causes include developmental
abnormalities of the brain.
16. Communicating
• Occurs when the flow of
CSF is blocked after it exits
the ventricles.
• This form is called
communicating because the
CSF can still flow between
the ventricles, which remain
open.
Non communicating
• Also called "obstructive"
hydrocephalus, occurs when
the flow of CSF is blocked
along one or more of the
narrow passages connecting
the ventricles.
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17. Pathophysiology- pg. 884
• The CSF accumulates within the cranial cavity
ventricles of the brain.
• This causes an increased pressure in the
cranial cavity.
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18. Assessment and common findings
pg.884
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• An enlarged head
• Widened cranial sutures and fontanelles
• ‘sunset eyes’
• Baby has difficulty lifting his/her head
• Developmental milestones are retarded
• Convulsions as intracranial pressure increases
• If the condition is left untreated, mental development will
be affected
• In adults, or children whose fontanelles already have been
fused, the clinical manifestations include signs of raised
intracranial pressure, convulsions and mental clouding
19. Risk factors of Hydrocephalus
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• Premature birth: Infants born preterm have a
higher risk of intraventricular hemorrhage, or
bleeding within the ventricles of the brain, which
may result in hydrocephalus.
• Problems during pregnancy: An infection in the
uterus during pregnancy increases the risk of
hydrocephalus in the developing fetus
• Problems with fetal development: Examples
include incomplete closure of the spinal column.
20. Risk factors of Hydrocephalus
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• Infections during pregnancy can affect the
development of the baby's brain. Examples
include:
• German measles (rubella)
• Mumps
• Syphilis
• Toxoplasmosis
21. Risk factors of Hydrocephalus
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• Other conditions that increase the risk
include:
• lesion and tumours of the spinal cord or brain
• infections of the nervous system
• bleeding in the brain
• severe head injury
22. Management
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• Surgical: Done by means of a shunt, which is inserted
into ventricles and connected to either the right atrium
or peritoneal cavity, from where it is reabsorbed into
the circulation.
• Sepsis & blockage of shunts are possible complications
• This predisposes the patient to infection therefore
antibiotics may be prescribed during or after shunt
insertion.
• Ongoing follow up is vital. Shunts need replacing and
revision from time to time.
• VIDEO* How to perform insertion of a ventriculo peritoneal
shunt
24. Neural tube/ Spina bifida -
pg. 885
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• (in an embryo) a hollow structure from which
the brain and spinal cord form.
• Defects in its development can result in
congenital abnormalities such as spina bifida.
25. What is the Neural tube?
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• The neural tube is what eventually becomes the
brain and spinal cord.
• In the early days of pregnancy, the embryo is
basically a ball of cells. That “ball” folds in on
itself as the cells start to form different body
parts.
• The neural tube typically closes — or forms a
complete tube — by the fourth week of
pregnancy.
• If the tube doesn’t fully close for some reason,
the baby is said to have a neural tube defect.
26. What is the Neural tube?
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• The neural tube is what eventually becomes the
brain and spinal cord. In the early days of
pregnancy, the embryo is basically a ball of cells.
That “ball” folds in on itself as the cells start to
form different body parts. The inner cells form a
tube-like structure that is called the neural tube.
The neural tube typically closes — or forms a
complete tube — by the fourth week of
pregnancy. If the tube doesn’t fully close for
some reason, the baby is said to have a neural
tube defect. Common neural tube defects include
spina bifida, anencephaly, and encephalocele.
27. The neural tube
• Taking enough folic acid before and during
pregnancy can help decrease the chances of a
neural tube defect.
• Because neural tube development happens
before most women even know they’re pregnant,
the Centers for Disease Control recommend that
all women of childbearing age eat a diet high in
folic acid or take a multivitamin containing 0.4 mg
of folic acid daily.
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28. Anencephaly- pg. 885
A baby born with an underdeveloped brain
and an incomplete skull.
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29. Anencephaly- pg.885
A baby born with an underdeveloped brain
and an incomplete skull.
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30. Encephalocele- pg.885
Characterized by sac-like protrusions of the brain and the
membranes that cover it through openings in the skull. These
defects are caused by failure of the neural tube to close
completely during fetal development.
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31. Encephalocele- pg.885
Characterized by sac-like protrusions of the brain and the
membranes that cover it through openings in the skull. These
defects are caused by failure of the neural tube to close
completely during fetal development.
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32. Classification of spina bifida- pg.885
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• Spina bifida occulta
– The defect only occurs in the vertebrae.
– The spinal cord and meninges are unaffected.
• Meningocele
– The meninges protrude through the defect in the
vertebrae, forming a sac filled with CSF
• Meningomyelocele
– This is the most serious form of the condition. the
meninges as well as the spinal cord protrude through
the vetebral defect
34. Risk factors
• Scientists suspect the factors that cause spina
bifida are multiple:
– Genetic
– Nutritional
– Environmental factors all play a role.
– Research studies indicate that insufficient intake
of folic acid—a common B vitamin—in the
mother's diet is a key factor in causing spina bifida
and other neural tube defects.
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35. Assessment and common findings
pg.885
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• The defect can be seen protruding at the back(in the case
of meningocele & meningomyelocele)
• Depending on the severity, a range of neurological
symptoms can be noted
• In spina bifida occulta often the only sign is the presence of
a dimple or hairy at the site, lipoma tuft of hair or port
wine birth mark at the site of lesion.
• Neurological disturbances may occur, such as motor and/or
sensory disturbances in the lower limbs and disturbances in
the bladder or bowel sphincter control.
• The two more severe types of spina bifida (meningocele &
meningomyelocele) may be accompanied by paralysis,
complete lack of bladder and bowel control.
36. Management of spina bifida
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• Treatment may not be indicated unless
neurological symptoms occur
• Surgical correction may be required
• Usually done as soon as possible after birth
• The care of the site and the prevention of
infection is important
• The nurse should note that the is a direct
connection between the outside air and the
nervous tissue of the brain and spinal cord
37. • VIDEO * What Is Spina Bifida?
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