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Hydrocephalus
Dr. Kuotho T Nyuwi
Moderator – Dr. Th Gojen
Definition: Abnormal collection of cerebrospinal fluid
(CSF) resulting in abnormal widening of spaces in the
brain
Greek word: Hydro - Cephalus
Physiology and circulation of CSF
- Normal volume of circulating CSF is around 150ml
- Daily production is about 500ml/day, 20ml/hr
Function:
- Protects and support the brain and spinal cord.
-Transport medium for transmitters and as a method of
removing the end-products of metabolism.
Imbalance excessive accumulation of CSF
Hydrocephalus
- Excessive production
- Decreased absorbtion
- Obstruction
Etiology %
Intraventricular hemorrhage 24
Myelomeningocele 21
Brain tumors 9
Aqueduct stenosis 7
CSF infection 5
Head injury 2
Others 11
Unknown 12
Types of hydrocephalus
1. Communicating and Non-communicating
2. Acute and chronic
3. Congenital and acquired
Hydrocephalus Ex Vacuo
Arrested hydrocephalus
Non-communicating (obstructive): CSF circulation is
blocked at or proximal to fourth ventricular outlet
foramina.(enlargement of ventricles proximal to the
block) – Aqueduct stenosis, tumors
Communicating (non-obstructive): At the level of the
cisterns, the subarachnoid space or the level of the
arachnoid granulation
- Post meningitis, post hemorrhagic
1. Congenital : Present at birth or few
weeks/months after birth (1-2/1000 live births)
Aqueduct stenosis
Chiari malformations
Dandy-Walker malformation
2. Acquired
Infection (post-meningitis)
Post – hemorrhagic (SAH,IVH)
Tumors
1. Acute : Develops within days or few weeks
- Manifests with rapid progression of symptoms -
Requires early attention and treatment
hydrocephalus caused by tumor
2. Chronic : Over months (or even years)
- Subtle signs of memory impairment, walking
difficulty, urinary incontinence
- A classic example is NPH
Chronic hydrocephalus can present acutely
because of changes in the pathophysiology of the
CSF absorption or flow.
Structural characteristics
- Dilatation of temporal and frontal horns of the
lateral ventricles
- Enlargement of ant. or post recesses of 3rd
ventricle
- Narrowing of ventricular angle
- Effacement of cortical sulci
Clinical Features
Due to increased ICP and dilation of ventricles,
causing compression of the adjacent brain
In neonatal period
Skull - thin and relatively non rigid allows for an
overall cranial expansion
Craniofacial disproportion
Irritable Fussy
May not accept feeds Vomiting
Poor head control Lethargy
Drowsiness
In extreme cases, lapse into a comatose state
Cont….
- Fontanel full , bulging and wide
- Thin and glistening scalp with enlargement and
engorgement of scalp veins
- Macewen's sign ( cracked pot sound on head
percussion)
- Sixth nerve (abducens) palsy
- Setting sun sign - upward gaze palsy
- Hyperactive reflexes.
- Irregular respiration with apneic spells.
- Separation of cranial sutures (sutures diastasis)
In older children and adults
The enlarging ventricles result in raised ICP and
compression of the adjacent brain
2 common modes of presentation
a) rapidly progressive hydrocephalus
b) chronic hydrocephalus.
Rapidly progressive hydrocephalus
Increased ICP - new-onset headache and vomiting
If untreated, these symptoms worsen and blurring of vision
often occurs.
In patients with long-standing raised pressure, papilledema
can result
If still untreated, drowsiness and progression to coma
follow.
Chronic hydrocephalus
- CSF accumulates more slowly - gradually
compressing the brain
- Predominantly seen in older adults
- The patient becomes progressively dull,
apathetic, and uninvolved with the surroundings.
- Memory impairment for recent events is commonly
seen, but usually the remote memory is well
preserved.
- Short stepped gait with a wide stance and unsteadiness
- Urinary incontinence
- No significant headache
Cerebellar fits or hydrocephalic attacks:
(severe headache, patient lapses into sudden
unconsciousness associated with a decerebrate or
decorticate response, downward deviation of the
eyeballs, and respiratory distress)
Medical emergency
Investigation
Ultrasonography to visualize the ventricular
system(when the anterior fontanelle is patent)
CT /MRI of the head
LP in cases of communicating hydrocephalus for both
diagnostic and therapeutic
Management
The ultimate goal is to reverse the neurologic
damage caused by the raised ICP
Medical treatment - not proved to be useful
Used as a temporary measure and in
conjunction with surgical management.
Acetazolamide - Commonly used - reduce CSF
production.
However, benefits are minimal and high doses of
the drug, which cause metabolic acidosis, are
required to achieve the effect.
Surgical
 Involves diversion of the accumulated CSF
(1) by reopening the obstruction to allow the CSF
to flow into its natural pathway
(2) by creating a diversion before the obstruction
to allow the CSF to drain into the intracranial
pathways distal to the block
(3) by diversion of the CSF into another cavity so
it becomes absorbed into the bloodstream.
 Removal of obstruction like posterior fossa tumor
Although shunts have been the mainstay of
treatment for several decades, endoscopic
procedures have now become more popular.
These include:
i) Endoscopic third ventriculostomy- into the
basal cisterns
ii) Endoscopic aqueductoplasty – 3 fr forgarty
catheter
iii) Endoscopic aqueductal stenting.
CSF diversion procedure :
- Children <5 years : difficult to assess intellectual
development
- Protects against the effects of persistent ventriculomegaly
and ensures an optimal environment for future intellectual
development
- >5 years and adults with asymptomatic ventriculomegaly
often are closely watched, with frequent assessment
of intellectual development, before considering a shunt
insertion.
Cerebrospinal Fluid Shunts
-Ventriculo-peritoneal(VP) - most common
-Ventriculo-atrial(VA)
-Torkildsen shunt (in aqueduct stenosis by passing a
catheter from the lateral ventricles into the cisternal
space)
-Lumbo-peritonial shunt.
-External drainage – temporary
Complications of shunts
Shunt malfunction Infection
Obstruction. Acute hemorrhages
Over drainage Hematoma
Disconnections Seizure
Distal Complication
Ascitis
Pseudoperitoneal cyst
Normal-Pressure Hydrocephalus
- Older patients with excessive accumulation of the
CSF in the intracranial compartment leading to
dilation of the ventricles and subarachnoid spaces
- The clinical picture is typically of an older patient
who presents with the triad of
gait ataxia, dementia, and urinary incontinence
Exact cause unknown - reduced absorption
-ICP not usually raised - brain parenchyma is less stiff
to allow it to be compressed by the developing
ventriculomegaly
-Diagnosis: combination of clinical features with
prominent ventricles seen by CT /MRI, with no other
abnormalities.
- A therapeutic trial of CSF drainage has been used
for patients suspected of having NPH to predict
response to treatment.
Treatment
Diversion of CSF:
i) ventriculoperitoneal shunt
ii) lumboperitoneal shunt
Thank you

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Hydrocephalus

  • 1. Hydrocephalus Dr. Kuotho T Nyuwi Moderator – Dr. Th Gojen
  • 2. Definition: Abnormal collection of cerebrospinal fluid (CSF) resulting in abnormal widening of spaces in the brain Greek word: Hydro - Cephalus
  • 3. Physiology and circulation of CSF - Normal volume of circulating CSF is around 150ml - Daily production is about 500ml/day, 20ml/hr Function: - Protects and support the brain and spinal cord. -Transport medium for transmitters and as a method of removing the end-products of metabolism.
  • 4.
  • 5.
  • 6. Imbalance excessive accumulation of CSF Hydrocephalus - Excessive production - Decreased absorbtion - Obstruction
  • 7.
  • 8. Etiology % Intraventricular hemorrhage 24 Myelomeningocele 21 Brain tumors 9 Aqueduct stenosis 7 CSF infection 5 Head injury 2 Others 11 Unknown 12
  • 9. Types of hydrocephalus 1. Communicating and Non-communicating 2. Acute and chronic 3. Congenital and acquired Hydrocephalus Ex Vacuo Arrested hydrocephalus
  • 10. Non-communicating (obstructive): CSF circulation is blocked at or proximal to fourth ventricular outlet foramina.(enlargement of ventricles proximal to the block) – Aqueduct stenosis, tumors Communicating (non-obstructive): At the level of the cisterns, the subarachnoid space or the level of the arachnoid granulation - Post meningitis, post hemorrhagic
  • 11. 1. Congenital : Present at birth or few weeks/months after birth (1-2/1000 live births) Aqueduct stenosis Chiari malformations Dandy-Walker malformation 2. Acquired Infection (post-meningitis) Post – hemorrhagic (SAH,IVH) Tumors
  • 12. 1. Acute : Develops within days or few weeks - Manifests with rapid progression of symptoms - Requires early attention and treatment hydrocephalus caused by tumor 2. Chronic : Over months (or even years) - Subtle signs of memory impairment, walking difficulty, urinary incontinence - A classic example is NPH Chronic hydrocephalus can present acutely because of changes in the pathophysiology of the CSF absorption or flow.
  • 13. Structural characteristics - Dilatation of temporal and frontal horns of the lateral ventricles - Enlargement of ant. or post recesses of 3rd ventricle - Narrowing of ventricular angle - Effacement of cortical sulci
  • 14. Clinical Features Due to increased ICP and dilation of ventricles, causing compression of the adjacent brain In neonatal period Skull - thin and relatively non rigid allows for an overall cranial expansion Craniofacial disproportion Irritable Fussy May not accept feeds Vomiting Poor head control Lethargy Drowsiness In extreme cases, lapse into a comatose state
  • 15. Cont…. - Fontanel full , bulging and wide - Thin and glistening scalp with enlargement and engorgement of scalp veins - Macewen's sign ( cracked pot sound on head percussion) - Sixth nerve (abducens) palsy - Setting sun sign - upward gaze palsy - Hyperactive reflexes. - Irregular respiration with apneic spells. - Separation of cranial sutures (sutures diastasis)
  • 16.
  • 17.
  • 18. In older children and adults The enlarging ventricles result in raised ICP and compression of the adjacent brain 2 common modes of presentation a) rapidly progressive hydrocephalus b) chronic hydrocephalus.
  • 19. Rapidly progressive hydrocephalus Increased ICP - new-onset headache and vomiting If untreated, these symptoms worsen and blurring of vision often occurs. In patients with long-standing raised pressure, papilledema can result If still untreated, drowsiness and progression to coma follow.
  • 20. Chronic hydrocephalus - CSF accumulates more slowly - gradually compressing the brain - Predominantly seen in older adults - The patient becomes progressively dull, apathetic, and uninvolved with the surroundings. - Memory impairment for recent events is commonly seen, but usually the remote memory is well preserved.
  • 21. - Short stepped gait with a wide stance and unsteadiness - Urinary incontinence - No significant headache Cerebellar fits or hydrocephalic attacks: (severe headache, patient lapses into sudden unconsciousness associated with a decerebrate or decorticate response, downward deviation of the eyeballs, and respiratory distress) Medical emergency
  • 22. Investigation Ultrasonography to visualize the ventricular system(when the anterior fontanelle is patent) CT /MRI of the head LP in cases of communicating hydrocephalus for both diagnostic and therapeutic
  • 23. Management The ultimate goal is to reverse the neurologic damage caused by the raised ICP Medical treatment - not proved to be useful Used as a temporary measure and in conjunction with surgical management. Acetazolamide - Commonly used - reduce CSF production. However, benefits are minimal and high doses of the drug, which cause metabolic acidosis, are required to achieve the effect.
  • 24. Surgical  Involves diversion of the accumulated CSF (1) by reopening the obstruction to allow the CSF to flow into its natural pathway (2) by creating a diversion before the obstruction to allow the CSF to drain into the intracranial pathways distal to the block (3) by diversion of the CSF into another cavity so it becomes absorbed into the bloodstream.  Removal of obstruction like posterior fossa tumor
  • 25. Although shunts have been the mainstay of treatment for several decades, endoscopic procedures have now become more popular. These include: i) Endoscopic third ventriculostomy- into the basal cisterns ii) Endoscopic aqueductoplasty – 3 fr forgarty catheter iii) Endoscopic aqueductal stenting.
  • 26. CSF diversion procedure : - Children <5 years : difficult to assess intellectual development - Protects against the effects of persistent ventriculomegaly and ensures an optimal environment for future intellectual development - >5 years and adults with asymptomatic ventriculomegaly often are closely watched, with frequent assessment of intellectual development, before considering a shunt insertion.
  • 27. Cerebrospinal Fluid Shunts -Ventriculo-peritoneal(VP) - most common -Ventriculo-atrial(VA) -Torkildsen shunt (in aqueduct stenosis by passing a catheter from the lateral ventricles into the cisternal space) -Lumbo-peritonial shunt. -External drainage – temporary
  • 28.
  • 29. Complications of shunts Shunt malfunction Infection Obstruction. Acute hemorrhages Over drainage Hematoma Disconnections Seizure Distal Complication Ascitis Pseudoperitoneal cyst
  • 30. Normal-Pressure Hydrocephalus - Older patients with excessive accumulation of the CSF in the intracranial compartment leading to dilation of the ventricles and subarachnoid spaces - The clinical picture is typically of an older patient who presents with the triad of gait ataxia, dementia, and urinary incontinence
  • 31. Exact cause unknown - reduced absorption -ICP not usually raised - brain parenchyma is less stiff to allow it to be compressed by the developing ventriculomegaly -Diagnosis: combination of clinical features with prominent ventricles seen by CT /MRI, with no other abnormalities. - A therapeutic trial of CSF drainage has been used for patients suspected of having NPH to predict response to treatment.
  • 32. Treatment Diversion of CSF: i) ventriculoperitoneal shunt ii) lumboperitoneal shunt