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EMERGENCIES IN NEUROSURGERY 2012

Symposium 7: Hydrocephalus

HYDROCEPHALUS
AND ITS CAUSES
29-9-2012
Dewan Auditorium, HTAA
WHAT IS HYDROCEPHALUS?

Abnormal build-up of cerebrospinal fluid

Ventricular System in the Brain or subarachnoid space

causes an increase in pressure
Ventricular System in the Brain

• Cerebral Spinal Fluid
(CSF) is mostly made in
the ventricular system of
the brain. This fluid then
circulates throughout the
brain as well as down the
spine.
Normal Cerebral Spinal Fluid Flow
• Most of the CSF is produced in
the body of the lateral
ventricle.
• Normally, it will flow from the
lateral ventricle down through
the Foramen of Monro into the
III ventricle.
• From there it proceeds down
the aqueduct into the IV
ventricle.
• There are three openings in
the IV ventricle:
– Laterally are the foramen of
Lushka
– Medially or in the middle is
the foramen of Magendie.
Cranio-caudal systolic CSF flow
cardiac systole

Transmission of cerebral
arterial pulsation

Monro-Kellie doctrine
The volume of the intracranial
contents (brain, CSF, blood)
remains constant all times.

Generalized expansion of the
intracranial volume

spinal subarachnoid space

Increase in intracranial volume is
dissipated by an outflow of CSF
from intracranial subarachnoid
spaces and the ventricular
system down to the compliant
spinal subarachnoid space,

Xavier Morandi *, Seyed F.A. Amlashi, Laurent Riffaud; Medical Hypotheses (2006) 67, 79–81
Caudo-cranial diastolic CSF flow
Diastole
decreased cerebral blood flow
The elastic property of the spinal dura
mater

CSF is pushed in the
opposite direction in the intracranial space

Xavier Morandi *, Seyed F.A. Amlashi, Laurent Riffaud; Medical Hypotheses (2006) 67, 79–81
Ventricular System in the Brain
• When there is too much
fluid in the ventricular
system, it dilates and can
squeeze the surrounding
brain tissue.
• This is hydrocephalus
Ventricular System in the Brain
• HYDROCEPHALUS
– Ventricular system is
becoming larger
– The ventricles are
becoming round
• Since the skull is a closed
space containing the
brain, increasing the size
of the fluid compartment
(the ventricles) begins to
displace the adjacent
parts of the brain
resulting in neurological
symptoms.
WHAT IS OBSTRUCTIVE
HYDROCEPHALUS?
• Obstruction to normal
cerebral spinal fluid
pathways may involve
any portion of the
ventricular system.

Multicompartmental model of ventricular volume regulation. (Rekate et al. 1988)
Obstructive Hydrocephalus: Example
• There is a mass (tumor) at the
level of the Foramen of
Monroe and the III Ventricle.
• The CSF flow is obstructed
and cannot flow down into the
III Ventricle to the aqueduct,
into the IV ventricle and then
out around the brain and down
into the spinal cord.
• The ventricular system is
enlarged proximal to (before)
the level of the blockage.
• Both lateral ventricles,
especially the frontal horns are
dilated.
Basic Clinical Anatomy:
Radiographic Correlation
Causes of Hydrocephalus
Obstructive Hydrocephalus: Causes
• Aqueductal
Stenosis, Tumors, Cy
sts and Hypertensive
Hemorrhages
Obstructive Hydrocephalus:
Aqueductal Stenosis
• Aqueductal Stenosis is seen
mostly in infants and young
adults.
• It can present later on in the
teenage years as well.
• With more and more people
having CT and MRI brain scans,
we are seeing people in their 50's
and even 70's (adult) with
aqueductal stenosis.
• Essentially, the aqueduct is
atretic
• Since cerebral spinal fluid (CSF)
cannot flow down into the IV
Ventricle, obstructive
hydrocephalus develops.
• Characteristically, everything
proximal to the aqueduct dilates.
The IV Ventricle remains normal
in size.
Aqueductal Stenosis : CT Scan
• The frontal horns, the III
ventricle and the temporal
horns are all enlarged.
• The IV ventricle which is
distal to the aqueduct is
normal in size.
Obstructive Hydrocephalus: TUMOR
• Cerebellar Brain Tumor
pushing on the IV Ventricle
• This is a common location
where brain tumors cause
hydrocephalus, invading the
IV ventricle and blocking
CSF (cerebral spinal fluid)
flow
• Sometimes strokes or
hemorrhages may cause this
sequence of events as well.
Obstructive Hydrocephalus: TUMOR
• Cerebellar Brain Tumor pushing
on the IV Ventricle
• The CT Scan shows a cross
section of the problem in a patient
with a cerebellar tumor and
swelling.
• The CSF can flow down its usual
pathway, but it is beginning to
become obstructed by the tumor.
• Unless treated, as the tumor
grows the ventricle will close off
and obstructive hydrocephalus
will develop.
• Symmetry of the IV ventricle is
lost. The black in the IV Ventricle
is Cerebral Spinal Fluid. The
lighter grey tissue is the
cerebellum.
• The darker grey tissue is tumor
plus swollen cerebellum.
Obstructive Hydrocephalus: CYST
• Loculated cysts in the III
ventricle causing early
obstructive hydrocephalus
• The MRI shows an enlarged
III ventricle.
• There are loculated cysts
within the III ventricle.
• These cyst formed due to
scarring from a patient with
meningitis.
• CSF cannot flow past the III
ventricle, so the lateral
ventricles begin to enlarge.
• The aqueduct and IV ventricle
are normal in size.
Obstructive Hydrocephalus: CYST
• Enlarged III Ventricle
With Development of
Early Hydrocephalus
• The CSF can flow down
its usual pathway, but it is
beginning to become
obstructed by the
enlarged III ventricle.
• Unless treated,
obstructive
hydrocephalus will
develop.
Obstructive Hydrocephalus: CYST
• Porencephalic Cyst and
Enlarged Ventricles... is this
Hydrocephalus?
• When a cyst communicates (is
continuous with) one of the
ventricles, it is usually
congenital (formed during
pregnancy).
• There is a large cerebellar cyst
communicating with an enlarged
IV ventricle.
• The III ventricle is mildly
enlarged as well. Is this early
hydrocephalus?
Obstructive Hydrocephalus:
HYPERTENSIVE HEMORRHAGE
• Bleeding into the brain from high
blood pressure causing early
obstructive hydrocephalus
• The CT Scan shows an enlarged
ventricular system.
• CSF cannot flow past the blood
clot so the lateral ventricles
begin to enlarge.
• High blood pressure
(Hypertension) is a common
cause of brain hemorrhages.
• When hemorrhages occur from
hypertension, many times the
patient will bleed into the
ventricular system and/or into
the surrounding brain structures.
• These structures are often the
thalamus and basal ganglia.
Obstructive Hydrocephalus:
HYPERTENSIVE HEMORRHAGE
• Bleeding into the brain
from high blood pressure
causing early obstructive
hydrocephalus
• The CSF can flow down
its usual pathway, but it is
beginning to become
obstructed by the
hemorrhage.
• Obstuctive hydrocephalus
is developing
COMMUNICATING
HYDROCEPHALUS
• Communicating hydrocephalus
develops when there is more fluid
being produced than can be
absorbed.
• The ventricular system enlarges to
hold the excess fluid.
• This compresses the surrounding
brain tissue.
• Communicating Hydrocephalus
may result from various types of
hemorrhages.(Post
Hemorrhagic, Subarachnoid
Hemorrhage, Other Hemorrhage).
• There is a special case of
Communicating Hydrocephalus
called Normal Pressure
Hydrocephalus.
Communicating Hydrocephalus: CT
Scan
• Notice how all the
ventricles are
enlarged. Compare
this image to the CT
Scans below.

Normal, No Hydrocephalus: CT Scan
Communicating Hydrocephalus:
SUBARACHNOID HEMORRHAGE
• Bleeding into the brain from an
aneurysm rupture may cause
communicating hydrocephalus
• The CT Scan to the left shows an
enlarging ventricular system.
• The blood results in decreased
absorption of the CSF.
• The production of CSF continues
so a net increase of CSF occurs.
• Thus, the ventricles begin to
enlarge.
• About 10% of people who suffer
SAH (subarachnoid hemorrhage)
develop hydrocephalus.
Communicating Hydrocephalus:
SUBARACHNOID HEMORRHAGE

• Delayed communicating
hydrocephalus from SAH
• Sometimes hydrocephalus is not
apparent during the acute
treatment of an aneurysm rupture.
• It may present itself several
months later.
• Follow-up CT scans are important
to diagnose delayed
hydrocephalus if clinically
indicated.
Communicating Hydrocephalus:
NORMAL PRESSURE
HYDROCEPHALUS (NPH)
• Clinical Triad
• Various degrees of the
following three complaints:
– Worsening Gait,
– Worsening Memory and
– Increasing Urinary
Incontinence.
Communicating Hydrocephalus:
NORMAL PRESSURE
HYDROCEPHALUS (NPH)
• MRI scans and normal pressure
hydrocephalus
• The MRI scan usually
demonstrates large ventricles.
• In elderly individuals there may
be cerebral atrophy (shrinkage
of the gray matter).
• Cerebral Spinal Fluid will fill up
the space of a brain with
atrophy.
• However, in NPH, the enlarged
fluid space (ventricles) are
enlarged out of proportion to the
cerebral atrophy.
1913 Johns Hopkins University

Dr. Walter Dandy
The first studies in experimental animals related to the
injection of a supravital dye into the ventricle and
attempting to recover it from the spinal subarachnoid space
(SSAS) via lumbar puncture.

communicating

non-communicating
“obstructive”
Why questions the simple
classification of Dandy?
• Some patients who developed
hydrocephalus in infancy or had arachnoid
cysts treated with shunts in infancy
develop severely increased intracranial
pressure with no expansion of the
ventricles or cyst at the time of shunt
failure
• Never lead to the understanding of normal
pressure hydrocephalus (NPH) and
pseudotumor cerebri (PC)
CSF dynamics
• Techniques for the study of CSF dynamics
had improved dramatically since the
studies of Dandy.
• Tools such as magnetic resonance
imaging (MRI), Cine MRI flow studies,
cisternography utilizing dye studies and
especially long-term studies of the
outcomes of treatment decisions make it
possible to accurately define a point of
restriction of flow.
CSF compliance
• Between 30% and 70% of total CSF system
compliance has been attributed to the spinal
compartment
• The remaining one to two third of CSF system
compliance consists of changes in the vascular pool,
especially the intracranial venous system (Vascular
compliance)
• These two systems are in equilibrium instantaneously
and any variations in either compartment result in rapid
compensatory changes in the other
Compliance= to the ratio of volume and pressures changes (dV/dP)

Xavier Morandi *, Seyed F.A. Amlashi, Laurent Riffaud; Medical Hypotheses (2006) 67, 79–81
Compliance of the spinal compartment
• Based on
– an increased elasticity of the spinal dura mater
– a wide epidural space with an extended
compressible epidural venous plexus
– sub-atmospheric epidural pressure

Xavier Morandi *, Seyed F.A. Amlashi, Laurent Riffaud; Medical Hypotheses (2006) 67, 79–81
ICD 10

Until now there has been no consensus as
to a more
contemporary classification scheme
Members of the Hydrocephalus
Classification Study Group

January of 2010
The structure of the consensus
First level of
classification of hydrocephalus
Based on the point
where the flow of CSF is restricted

Foramina of Monro,
The aqueduct of Sylvius,
The basal cisterns,
The arachnoid granulations,
Outflow of venous blood from the dural
venous sinuses

without a point of
obstruction or increased resistance to flow.

communicating hydrocephalus
overproduction of CSF
(e.g. by choroid
plexus papillomas)

Second level of
classification of hydrocephalus
Modified by

The etiology
The chronicity or rapidity of onset
The age of the person
Thank You

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Hydrocephalus and it's causes

  • 1. EMERGENCIES IN NEUROSURGERY 2012 Symposium 7: Hydrocephalus HYDROCEPHALUS AND ITS CAUSES 29-9-2012 Dewan Auditorium, HTAA
  • 2. WHAT IS HYDROCEPHALUS? Abnormal build-up of cerebrospinal fluid Ventricular System in the Brain or subarachnoid space causes an increase in pressure
  • 3. Ventricular System in the Brain • Cerebral Spinal Fluid (CSF) is mostly made in the ventricular system of the brain. This fluid then circulates throughout the brain as well as down the spine.
  • 4. Normal Cerebral Spinal Fluid Flow • Most of the CSF is produced in the body of the lateral ventricle. • Normally, it will flow from the lateral ventricle down through the Foramen of Monro into the III ventricle. • From there it proceeds down the aqueduct into the IV ventricle. • There are three openings in the IV ventricle: – Laterally are the foramen of Lushka – Medially or in the middle is the foramen of Magendie.
  • 5. Cranio-caudal systolic CSF flow cardiac systole Transmission of cerebral arterial pulsation Monro-Kellie doctrine The volume of the intracranial contents (brain, CSF, blood) remains constant all times. Generalized expansion of the intracranial volume spinal subarachnoid space Increase in intracranial volume is dissipated by an outflow of CSF from intracranial subarachnoid spaces and the ventricular system down to the compliant spinal subarachnoid space, Xavier Morandi *, Seyed F.A. Amlashi, Laurent Riffaud; Medical Hypotheses (2006) 67, 79–81
  • 6. Caudo-cranial diastolic CSF flow Diastole decreased cerebral blood flow The elastic property of the spinal dura mater CSF is pushed in the opposite direction in the intracranial space Xavier Morandi *, Seyed F.A. Amlashi, Laurent Riffaud; Medical Hypotheses (2006) 67, 79–81
  • 7. Ventricular System in the Brain • When there is too much fluid in the ventricular system, it dilates and can squeeze the surrounding brain tissue. • This is hydrocephalus
  • 8. Ventricular System in the Brain • HYDROCEPHALUS – Ventricular system is becoming larger – The ventricles are becoming round • Since the skull is a closed space containing the brain, increasing the size of the fluid compartment (the ventricles) begins to displace the adjacent parts of the brain resulting in neurological symptoms.
  • 9. WHAT IS OBSTRUCTIVE HYDROCEPHALUS? • Obstruction to normal cerebral spinal fluid pathways may involve any portion of the ventricular system. Multicompartmental model of ventricular volume regulation. (Rekate et al. 1988)
  • 10. Obstructive Hydrocephalus: Example • There is a mass (tumor) at the level of the Foramen of Monroe and the III Ventricle. • The CSF flow is obstructed and cannot flow down into the III Ventricle to the aqueduct, into the IV ventricle and then out around the brain and down into the spinal cord. • The ventricular system is enlarged proximal to (before) the level of the blockage. • Both lateral ventricles, especially the frontal horns are dilated.
  • 13. Obstructive Hydrocephalus: Causes • Aqueductal Stenosis, Tumors, Cy sts and Hypertensive Hemorrhages
  • 14. Obstructive Hydrocephalus: Aqueductal Stenosis • Aqueductal Stenosis is seen mostly in infants and young adults. • It can present later on in the teenage years as well. • With more and more people having CT and MRI brain scans, we are seeing people in their 50's and even 70's (adult) with aqueductal stenosis. • Essentially, the aqueduct is atretic • Since cerebral spinal fluid (CSF) cannot flow down into the IV Ventricle, obstructive hydrocephalus develops. • Characteristically, everything proximal to the aqueduct dilates. The IV Ventricle remains normal in size.
  • 15. Aqueductal Stenosis : CT Scan • The frontal horns, the III ventricle and the temporal horns are all enlarged. • The IV ventricle which is distal to the aqueduct is normal in size.
  • 16. Obstructive Hydrocephalus: TUMOR • Cerebellar Brain Tumor pushing on the IV Ventricle • This is a common location where brain tumors cause hydrocephalus, invading the IV ventricle and blocking CSF (cerebral spinal fluid) flow • Sometimes strokes or hemorrhages may cause this sequence of events as well.
  • 17. Obstructive Hydrocephalus: TUMOR • Cerebellar Brain Tumor pushing on the IV Ventricle • The CT Scan shows a cross section of the problem in a patient with a cerebellar tumor and swelling. • The CSF can flow down its usual pathway, but it is beginning to become obstructed by the tumor. • Unless treated, as the tumor grows the ventricle will close off and obstructive hydrocephalus will develop. • Symmetry of the IV ventricle is lost. The black in the IV Ventricle is Cerebral Spinal Fluid. The lighter grey tissue is the cerebellum. • The darker grey tissue is tumor plus swollen cerebellum.
  • 18. Obstructive Hydrocephalus: CYST • Loculated cysts in the III ventricle causing early obstructive hydrocephalus • The MRI shows an enlarged III ventricle. • There are loculated cysts within the III ventricle. • These cyst formed due to scarring from a patient with meningitis. • CSF cannot flow past the III ventricle, so the lateral ventricles begin to enlarge. • The aqueduct and IV ventricle are normal in size.
  • 19. Obstructive Hydrocephalus: CYST • Enlarged III Ventricle With Development of Early Hydrocephalus • The CSF can flow down its usual pathway, but it is beginning to become obstructed by the enlarged III ventricle. • Unless treated, obstructive hydrocephalus will develop.
  • 20. Obstructive Hydrocephalus: CYST • Porencephalic Cyst and Enlarged Ventricles... is this Hydrocephalus? • When a cyst communicates (is continuous with) one of the ventricles, it is usually congenital (formed during pregnancy). • There is a large cerebellar cyst communicating with an enlarged IV ventricle. • The III ventricle is mildly enlarged as well. Is this early hydrocephalus?
  • 21. Obstructive Hydrocephalus: HYPERTENSIVE HEMORRHAGE • Bleeding into the brain from high blood pressure causing early obstructive hydrocephalus • The CT Scan shows an enlarged ventricular system. • CSF cannot flow past the blood clot so the lateral ventricles begin to enlarge. • High blood pressure (Hypertension) is a common cause of brain hemorrhages. • When hemorrhages occur from hypertension, many times the patient will bleed into the ventricular system and/or into the surrounding brain structures. • These structures are often the thalamus and basal ganglia.
  • 22. Obstructive Hydrocephalus: HYPERTENSIVE HEMORRHAGE • Bleeding into the brain from high blood pressure causing early obstructive hydrocephalus • The CSF can flow down its usual pathway, but it is beginning to become obstructed by the hemorrhage. • Obstuctive hydrocephalus is developing
  • 23. COMMUNICATING HYDROCEPHALUS • Communicating hydrocephalus develops when there is more fluid being produced than can be absorbed. • The ventricular system enlarges to hold the excess fluid. • This compresses the surrounding brain tissue. • Communicating Hydrocephalus may result from various types of hemorrhages.(Post Hemorrhagic, Subarachnoid Hemorrhage, Other Hemorrhage). • There is a special case of Communicating Hydrocephalus called Normal Pressure Hydrocephalus.
  • 24. Communicating Hydrocephalus: CT Scan • Notice how all the ventricles are enlarged. Compare this image to the CT Scans below. Normal, No Hydrocephalus: CT Scan
  • 25. Communicating Hydrocephalus: SUBARACHNOID HEMORRHAGE • Bleeding into the brain from an aneurysm rupture may cause communicating hydrocephalus • The CT Scan to the left shows an enlarging ventricular system. • The blood results in decreased absorption of the CSF. • The production of CSF continues so a net increase of CSF occurs. • Thus, the ventricles begin to enlarge. • About 10% of people who suffer SAH (subarachnoid hemorrhage) develop hydrocephalus.
  • 26. Communicating Hydrocephalus: SUBARACHNOID HEMORRHAGE • Delayed communicating hydrocephalus from SAH • Sometimes hydrocephalus is not apparent during the acute treatment of an aneurysm rupture. • It may present itself several months later. • Follow-up CT scans are important to diagnose delayed hydrocephalus if clinically indicated.
  • 27. Communicating Hydrocephalus: NORMAL PRESSURE HYDROCEPHALUS (NPH) • Clinical Triad • Various degrees of the following three complaints: – Worsening Gait, – Worsening Memory and – Increasing Urinary Incontinence.
  • 28. Communicating Hydrocephalus: NORMAL PRESSURE HYDROCEPHALUS (NPH) • MRI scans and normal pressure hydrocephalus • The MRI scan usually demonstrates large ventricles. • In elderly individuals there may be cerebral atrophy (shrinkage of the gray matter). • Cerebral Spinal Fluid will fill up the space of a brain with atrophy. • However, in NPH, the enlarged fluid space (ventricles) are enlarged out of proportion to the cerebral atrophy.
  • 29. 1913 Johns Hopkins University Dr. Walter Dandy The first studies in experimental animals related to the injection of a supravital dye into the ventricle and attempting to recover it from the spinal subarachnoid space (SSAS) via lumbar puncture. communicating non-communicating “obstructive”
  • 30. Why questions the simple classification of Dandy? • Some patients who developed hydrocephalus in infancy or had arachnoid cysts treated with shunts in infancy develop severely increased intracranial pressure with no expansion of the ventricles or cyst at the time of shunt failure • Never lead to the understanding of normal pressure hydrocephalus (NPH) and pseudotumor cerebri (PC)
  • 31. CSF dynamics • Techniques for the study of CSF dynamics had improved dramatically since the studies of Dandy. • Tools such as magnetic resonance imaging (MRI), Cine MRI flow studies, cisternography utilizing dye studies and especially long-term studies of the outcomes of treatment decisions make it possible to accurately define a point of restriction of flow.
  • 32. CSF compliance • Between 30% and 70% of total CSF system compliance has been attributed to the spinal compartment • The remaining one to two third of CSF system compliance consists of changes in the vascular pool, especially the intracranial venous system (Vascular compliance) • These two systems are in equilibrium instantaneously and any variations in either compartment result in rapid compensatory changes in the other Compliance= to the ratio of volume and pressures changes (dV/dP) Xavier Morandi *, Seyed F.A. Amlashi, Laurent Riffaud; Medical Hypotheses (2006) 67, 79–81
  • 33. Compliance of the spinal compartment • Based on – an increased elasticity of the spinal dura mater – a wide epidural space with an extended compressible epidural venous plexus – sub-atmospheric epidural pressure Xavier Morandi *, Seyed F.A. Amlashi, Laurent Riffaud; Medical Hypotheses (2006) 67, 79–81
  • 34. ICD 10 Until now there has been no consensus as to a more contemporary classification scheme
  • 35. Members of the Hydrocephalus Classification Study Group January of 2010
  • 36. The structure of the consensus First level of classification of hydrocephalus Based on the point where the flow of CSF is restricted Foramina of Monro, The aqueduct of Sylvius, The basal cisterns, The arachnoid granulations, Outflow of venous blood from the dural venous sinuses without a point of obstruction or increased resistance to flow. communicating hydrocephalus overproduction of CSF (e.g. by choroid plexus papillomas) Second level of classification of hydrocephalus Modified by The etiology The chronicity or rapidity of onset The age of the person