1. Hydrocephalus
in Paediatric
By:
Hieder A`ala
601
1

2. Cerebro-Spinal Fluid (CSF)
 Definition: is a clear bodily fluid that occupies the
subarachnoid space in the brain (the space between the
skull and the cerebral cortex—more specifically, between
the arachnoid and pia layers of the meninges). It is a very
pure saline solution with microglia and acts as a "cushion"
or buffer for the cortex.
2

3. 3

4. Physiology
 Cerebrospinal fluid also occupies the ventricular system of the
brain and the spinal cord. It is a prime example of the
separation of brain function from the rest of the body, as all
CSF is generated locally in the brain. It is produced by the
choroid plexus which is formed by specialized ependymal
cells. The choroid plexus enter the lateral ventricles through
the choroid fissure, along the line of the fimbria/fornix, and
the third and fourth ventricle through their roofs. The CSF
formed by the choroid plexuses in the ventricles, circulates
through the interventricular foramina (foramen of Monro) into
the third ventricle and then via the mesencephalic duct
(cerebral aqueduct) into the fourth ventricle, whence it exits
through two lateral apertures (foramina of Luschka) and one
median aperture (foramen of Magendie). It then flows through
the cerebromedullary cistern down the spinal cord and over
the cerebral hemispheres.
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5. 5

6. Physiology
 Traditionally, it has been thought that CSF returns to the vascular system
by entering the dural venous sinuses via the arachnoid granulations.
However, some have suggested that CSF flow along the cranial nerves and
spinal nerve roots allow it into the lymphatic channels and that this flow
may play a substantial role in CSF reabsorbtion, particularly in the neonate
(in which arachnoid granulations are sparsely distributed).
 The cerebrospinal fluid is produced by the ventricles (mostly the lateral
ventricles) at a rate of 500 ml/day. Since the volume that may be contained
by the brain is of 150 ml, it is frequently replaced (3-4 times per day
turnover), exceeding amounts getting into the blood. This continuous flow
through the ventricular system into the subarachnoid space and finally
exiting into the venous system provides somewhat of a "sink" that reduces
the concentration of larger, lipoinsoluble molecules penetrating into the
brain and CSF.
 The CSF contains approximately 0.3% plasma proteins, also being 15 to 40
mg/dL, depending on sampling site.
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7. 7

8. What is Hydrocephalus?
 The term hydrocephalus is derived from the Greek words
“hydro” meaning water and “cephalus” meaning head. As the
name implies, (water head) it is a condition in which the
primary characteristic is excessive accumulation of fluid in the
brain. The excessive accumulation of (CSF) cerebrospinal
fluid results in an abnormal dilatation of the spaces in the brain
called ventricles. This dilatation causes potentially harmful
pressure on the tissue of the brain.
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9. Aetiology of hydrocephalus
9

10. classifications of Hydrocephalus
We will classify causes of hydrocephalus into
two forms according to two principles.
1-according to relation or comminucation
between the ventricular system and the venous
sinuses (i.e. obstructive or non-obstructive).
2-according to the nature of the aetiology
whether acquired or congenital.
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11. 1st classification
A-Obstructive (non-communicating)
This type of hydrocephalus
results from an obstruction
within the ventricular
system of the brain that
prevents CSF from flowing
or “communicating” within
the brain. The most
common type is a narrowing
of a channel in the brain that
connects two ventricles
together.
11

12. 1st classification
B-Non-obstructive (communicating)
This type results from
problems with the
production or
absorption of CSF. The
most common is caused
by bleeding into the
subarachnoid space in
the brain.
12

13. 2nd classification
A-Congenital causes in infants and children
 Stenoses of the aqueduct of Sylvius due to malformation: This is
responsible for 10% of all cases of hydrocephalus in newborns.
 Dandy-Walker malformation: This affects 2-4% of newborns
with hydrocephalus.
 Arnold-Chiari malformation type 1 and type 2 and type three
 Agenesis of the foramen of Monro
 Congenital toxoplasmosis
 Bickers-Adams syndrome: This is an X-linked hydrocephalus
accounting for 7% of cases in males. It is characterized by
stenosis of the aqueduct of Sylvius, severe mental retardation,
and in 50% by an adduction-flexion deformity of the thumb
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14. 2nd classification
B-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: 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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15. 15

16. Some important definitions
CHIARI MALFORMATION
 TYPE I:
 DISPLACEMENT OF CEREBELLAR TONSILS INTO THE
CERVICAL CANAL.
 GIVES SYMPTOMS IN ADOLESCENCE OR ADULT LIFE.
(HEADACHE, NECK PAIN)
 NO HYDROCEPHALUS
 TYPE II :
 DISPLACEMENT OF INFERIOR VERMIS, PONS, AND
MEDULLA INTO CERVICAL CANAL
 PROGRESSIVE HYDROCEPHALUS AND
MYELOMENINGOCELE.
 ELONGATION OF THE 4TH VENTRICLE.
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17. Some important definitions
 DANDY-WALKER SYNDROME
1. CYSTIC EXPANSION OF THE 4TH
VENTRICLE IN THE POSTERIOR
FOSSA.
2. DEVELOPMENTAL FAILURE OF ROOF
OF 4TH VENTRICLE DURING
EMBRYOGENESIS.
3. 90 % HAVE HYDROCEPHALUS
4. PROMINENT OCCIPUT
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18. Other types of Hydrocephalus
Two other forms of hydrocephalus which don’t fit distinctly
into those categories are hydrocephalus ex-vacuo, and normal
pressure hydrocephalus.
 N.B.: * Ex-vacuo occurs when there is damage to the
brain caused by stroke of a traumatic injury.
 * Normal pressure hydrocephalus commonly occurs in
the elderly, due to aging. The triad (Hakim triad) of gait
instability, urinary incontinence and dementia is a relatively
typical manifestation of the distinct entity normal pressure
hydrocephalus (NPH). The triad can easily be remembered as
"Wacky, Wet, and Wobbly!"
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19. Some important definitions
IVH
 DEFINITION:
 BLEEDING IN SUBEPENDIMAL GERMINAL
MATRIX WITH/WITHOUT EXTENSION INTO
VENTRICLES AND BRAIN PARENCHYMA
 INCIDENCE:
 IN PREMATURES 25 - 40 %
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20. Some important definitions
 PATHOLOGY:
 INTRAVASCULAR
 VASCULAR
 EXTRAVASCULAR
 COMPLICATIONS:
 HYDROCEPHALUS
 20 % IN MODERATE BLEEDS
 65-100 % IN LARGE BLEEDS
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21. Pathophysiology
 Pathological basis of clinical picture:
1. increased intracranial tension (ICP).
2. Age of onset.
3. Type of hydrocephalus (obs. or non obs.)
4. Closure of fontanels and sutures of the skull
5. Associated disorders.
6. Treatment.( relapsing of the symptoms due to
complication associated with treatment )
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22. Pathophysiology
 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).
22

23. Pathophysiology
 Age of onset
There are three groups in which hydrocephalus
can develop:
1. Fetuses (diagnosed antenataly )
2. Infants .
3. Children.
Each group has its own criteria of hydrocephalus
 Type of hydrocephalus (obs.or non obs.)
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24. Pathophysiology
 Closure of fontanels and sutures of the skull :
As there is limited space for expansion in the
skull, CSF pressure (as total intracranial
pressure) effects the arterial profusion to the
brain. When CSF pressure is elevated, cerebral
blood flow may be diminished .
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25. Pathophysiology
 Associated disorders. ( see later )
Meningitis, ventriculaitis , meningeocele ,
cerebellar herniation ..etc.
 Treatment as option, time of intervention ,
modality , complications and rate of
recurrence .
25

26. Pathophysiology
 As there is limited space for expansion in the
skull, CSF pressure (as total intracranial
pressure) effects the arterial profusion to the
brain. When CSF pressure is elevated, cerebral
blood flow may be diminished .
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27. 27

28. Mortality/Morbidity
 Mortality : In untreated hydrocephalus, death may occur by
tonsillar herniation secondary to raised ICP with compression
of the brain stem and subsequent respiratory arrest.
 Morbidity:
1. Shunt dependence occurs in 75%
2. frequent hospitalizations for scheduled shunt revisions .
3. Poor development of cognitive function in infants and children
4. Visual loss can complicate untreated hydrocephalus and may
persist after treatment
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29. Male : Female
 Sex: Generally, incidence is equal in males
and females. The exception is Bickers-Adams
syndrome, an X-linked hydrocephalus
transmitted by females and manifested in
males. NPH has a slight male preponderance.
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30. 30

31. Fetal hydrocephalus.
 During antenatal care , Fetal ventriculomegaly
can be detected through ultrasound (sonogram)
towards the end of the first trimester.
Evaluation of the brain and cranial structure is
part of the routine ultrasound examination
done by many obstetricians as part of their
prenatal care. If the condition is detected on
ultrasound, the patient may undergo a fetal
brain MRI (magnetic resonance imaging) to
determine the severity of the finding.
31

32. Sonographic demonstration
 Fetal hydrocephalus:
gross enlargement of the
lateral ventricles,
thinning of the cortex,
asymmetric choroid
plexuses.
32

33. Infants
Symptoms
 Poor feeding
 Irritability
 Reduced activity
 Vomiting
33

34. Infants
Signs
 Head enlargement: Head circumference is in the 98th percentile for the
age or greater.(remember h.c. to the age)
 Dysjunction of sutures: This can be seen or palpated.
 Dilated scalp veins: The scalp is thin and shiny with easily visible
veins.(why?)
 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 preferentially affects the lower limbs.
The cause is stretching of the periventricular pyramidal tract fibers by
hydrocephalus.
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35. Children
Symptoms
 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 and later of optic atrophy
 Double vision - Related to unilateral or bilateral sixth nerve palsy
 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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36. Children
Signs
 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. Tapping with the fingertips on the
skull
may show abnormal sounds associated with
thinning and separation of skull bones.
 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.
 36
Unilateral or bilateral sixth nerve palsy is secondary to

37. Certain pics
.
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38. Certain pics
38

39. Differential diagnosis
 Macrocephaly
 Macrocephaly means a large head—greater than 2 standard
deviations from the normal distribution; 2% of the “normal”
population has macrocephaly. Investigation of such
individuals may show an abnormality causing macrocephaly,
but many are normal, often with a familial tendency for a large
head. When asked to evaluate a large head in an otherwise
normal child, first ask the parents for their hat sizes.
 The causes of a large head include hydrocephalus (an
excessive volume of CSF in the skull), megalencephaly
(enlargement of the brain), thickening of the skull, and
hemorrhage into the subdural or epidural spaces.
Hydrocephalus is the main cause of macrocephaly at birth in
which intracranial pressure is increased
39

40. Differential diagnosis
1- extracranial causes:
Cephalhematoma, subgleal hemorrhage .
2- cranial causes:
 Anemia
 Cleidocranial dysostosis
 Craniometaphyseal dysplasia of Pyle
 Epiphyseal dysplasia
 Hyperphosphatemia
 Leontiasis ossea
 Orodigitofacial dysostosis
 Osteogenesis imperfecta
 OsteopetrosisPyknodysostosis
 Rickets
 Russell dwarf
3-intracranial causes :
Megalencephaly , hydrocephalus and strugg – Webber syndrome .
40

41. Work up
1-Imaging Studies
• CT scan of the head
• MRI scan of head
• Fetal and neonatal cranial ultrasound
2-Diagnostic Procedures
• Lumbar puncture
41

42. Work up
imaging studies
 CT scan of the head delineates the degree of
ventriculomegaly and, in many cases, the
etiology. When performed with contrast, it can
show infection and tumors causing
obstruction. It also helps with operative
planning. Ventricles usually are dilated
proximal to the point of obstruction. In
pseudotumor cerebri, the CT scan findings
usually are normal.
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43. Work up
imaging studies
 Perform MRI scan of head in most, if not all,
congenital cases of hydrocephalus. This
delineates the extent of associated brain
anomalies such as corpus callosum agenesis,
Chiari malformations, disorders of neuronal
migration, and vascular malformations.
43

44. Work up
Imaging studies
 Fetal and neonatal cranial ultrasound is a good
study for monitoring ventricular size and
intraventricular hemorrhage in the neonatal
ICU setting. Certainly, prior to treatment,
perform other imaging studies.
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45. Imaging studies
 Noncommunicating
obstructive hydrocephalus
caused by obstruction of the
foramina of Luschka and
Magendie. This MRI
sagittal image demonstrates
dilatation of lateral
ventricles with stretching of
corpus callosum and
dilatation of the fourth
ventricle.
45

46. Imaging studies
 Noncommunicating
obstructive
hydrocephalus caused
by obstruction of
foramina of Luschka
and Magendie. This
MRI axial image
demonstrates dilatation
of the lateral ventricles.
46

47. Imaging studies
 Non-communicating
obstructive
hydrocephalus caused
by obstruction of
foramina of Luschka
and Magendie. This
MRI axial image
demonstrates fourth
ventricle dilatation.
47

48. Imaging studies
 Dandy-Walker
malformation. CT
shows cystic dilation of
the fourth ventricle and
partial agenesis of the
cerebellar vermis
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49. Imaging studies
 Aqueductal stenosis. CT
shows marked
enlargement of the third
ventricle (arrow) and
the lateral ventricles.
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50. Imaging studies
 Chiari I malformation :
Sagittal C-T1W MR
image of the brain
shows a downward
herniation of the
cerebellar tonsils
without associated
syrinx.
50

51. Imaging studies
 Chiari II malformation :
Axial FSE T2W MR
image of the brain
shows colpocephaly.
51

52. Imaging studies
MR images demonstrating a massively dilated ventricular system with
congenital hydrocephalus secondary to acqueductal stenosis. The sagittal
image(left) shows a normal-appearing fourth ventricle. The tremendous
enlargement of the lateral ventricles has led to compression of the
cerebral mantle with only the frontal lobes being visible (right).
52

53. Work up
Diagnostic Procedures
 Lumbar puncture can be
used to measure
intracranial pressure,
but it should only be
performed after imaging
studies rule out an
obstruction. Spinal fluid
can show the type and
severity of infection
( i.e. in meningitis )
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54. TREATMENT
Medical therapy :
 Indications: In transient conditions, such as meningitis, or
neonatal intraventricular hemorrhage .
 Modalities :
1. Acetazolamide (25 mg/kg/d in 3 doses): Careful monitoring
of respiratory status and electrolytes is crucial. Treatment
beyond 6 months is not recommended.
2. Furosemide (1 mg/kg/d in 3 doses): Again, electrolyte
balance and fluid balance need to be monitored carefully.
54

55. Treatment
3-Lumbar punctures: In neonates recovering
from intraventricular hemorrhage, serial
lumbar punctures can resolve hydrocephalus
in some cases. If possible, this is the
preferred method of treatment.
4-Removal of the underlying cause resolves
hydrocephalus in most cases
55

56. Treatment
Surgical therapy
 Principle of surgical intervention :
Relief increased ICP by diversion the exessive
CSF from ventricular system into an
absorptive surface out side the brain such as
pleura or peritoneum or into the atria of the
heart…….this is called shunt operation
 The most indication for shunt operation is
progressive hydrocephalus .
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57. Treatment
 Contraindications:
1. The patient in whom a successful surgery would not affect
the outcome (eg, a child with hydranencephaly) or cortical
thickenning is less than one cm.
2. Arrested hydrocephalus is defined as a rare condition in
which the neurologic status of the patient is stable in the
presence of stable ventriculomegaly.
3. Benign hydrocephalus of infancy is found in neonates and
young infants. The children are asymptomatic, and head
growth is normal. CT scan shows mildly enlarged ventricles
and subarachnoid spaces.
57

58. Treatment
 Types of shunts :
1. Third ventriculostomy
2. Ventriculoperitoneal shunting (the common
procedure ) .
3. Ventriculoatrial shunting .
4. Ventriculopleural shunting .
5. Torkildsen shunts or internal shunts .
6. Lumboperitoneal shunts .
58

59. Treatment
59

60. Treatment
60

61. Follow-up care
 Perform CT scan for baseline at 2-4 weeks
postsurgery.
 Monitor all children with shunts every 6-12 months.
Carefully monitor head growth in infants. Check
distal tubing length with plain radiographs when the
child grows. Appropriate specialists should carefully
assess child development.
 What happens to ventricular size in patients who have
a third ventriculostomy or Torkildsen shunt is not
known. Other methods of assessment of patency need
to be used, such as MRI flow studies and clinical
evaluations (eg, detailed funduscopic examinations).
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62. COMPLICATIONS
1. Infection is the most feared complication
2. Subdural hematomas .
3. Shunt failure is mostly due to suboptimal
proximal catheter placement .
4. Overdrainage is more common in
lumboperitoneal shunts .
5. Slit ventricle syndrome .
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63. Shunt failure
63

64. PROGNOSIS
 In general, outcome is good. A typical patient should
return to baseline after shunting. The neurologic
function of children is optimized with shunting.
 The best long-term results in the most carefully
selected patients are no better than 60% in normal
pressure hydrocephalus. Few complete recoveries
occur. Often, gait and incontinence respond to
shunting, but dementia responds less frequently.
 Often, various other neurologic abnormalities
associated with hydrocephalus are the limiting factor
in patient recovery. Examples are migrational
abnormalities and postinfectious hydrocephalus.
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65. Before and After
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66. 66

67. THE END
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