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Hydrocephalus
Dr. Ali Omar Saadoon
Physiology and circulation of CSF
• The normal volume of circulating CSF is around 140ml
• daily production is about 500ml/day
• the CSF volume is replaced approximately …?……..times daily
• Function:
• The CSF is both protects and support the brain and spinal cord.
• transport medium for transmitters and as a method of removing the
end-products of metabolism.
• CSF is mainly produced by the choroid plexus
Definition
HYDROCEPHALUS
It is defined as a disproportionate increase
in the amount of CSF within the cranium,
usually in association with a rise in ICP.
(Ventricular enlargement with excessive
CSF) And it should be differentiated from
hydrocephalus ex vacuo (due to brain
atrophy)
hydrocephalus
Brain atrophy
Epidemiology and etiology
.the overall incidence …not known
. Incidence of congenital hydrocephalus is 1-
2/1000 live births
-congenital : maternal malnutrition ,toxins,infection
with toxoplasmosis and cytomegalic virus . A
rare (2% of all congenital HC) ..is X-linked
recessive aqueductal stenosis ( only in male
and female are carrier)
Pathogenesis
1. Obstruction of the CSF pathways
a. Lesions that obstruct the ventricular
system…
b. Lesions that obstruct the subarachnoid
space…..
2. CSF over secretion…choroid plexus
papilloma ..
3. Impaired venous drainage
Pathophysiology of
hydrocephalus
• Acute hydrocephalus
-in animals…
- In humans :
1. ICH
2. Acute decompensation of tumors bordering the ventricular system
3. Gunshot
4. SAH
5. Head injuries
6. Sudden shunt obstruction
If the CSF obstruction is complete , ventricular enlargement may
reach significant proportions in 3 to 6 hr.
. Chronic hydrocephalus
Several compensatory adjustments can
occur including:
1-expansion of the skull
2-contraction of the cerebral vascular
volume
3- volumetric enlargement of the ventricular
system
Types of hydrocephalus
• Communicating (non-obstructive): CSF
circulation blocked at the level of the basal
cisterns, the subarachnoid space or the level of
the arachnoid granulation.
• Non-communicating (obstructive): the normal
pathways of CSF flow are for some reason
occluded such as aqueduct stenosis or as a
result of a local compression from a tumor.
(enlargement of ventricles proximal to the block)
further types of classifications
• Acute and chronic
• Congenital and acquired
• Can be classified by etiology
- posttraumatic,
- post hemorrhagic
- postmeningitis
Etiology
imbalance has occurred between the
normal physiological production of CSF
and its absorption
Etiology
• Congenital …..?
– aqueduct stenosis
– Chiari malformations
– Dandy-Walker malformation
• Acquired
– infection (post-meningitis)
– post – hemorrhagic(SAH,IVH)
– secondary to masses( non- neoplastic like vascular
malformation, tumors like posterior foss tumors,
pituitary and colloid cyst )
Clinical features
In neonatal period
• Cranium enlarges at a rate more than the facial growth
(craniofacial disproportion).
• irritability , poor head control, nausea and vomiting
• Fontanel full , bulging and wide.
• Thin and glistening scalp with enlargement and engorgement of
scalp veins ;due to reversal of flow from intracerebral sinuses due
to increase intra cranial pressure.
• Macewen's sign ( cracked pot sound on head percussion)
• Sixth nerve (abducens) palsy, long intracranial course
make this nerve very pressure sensitive.
• Setting sun sign, (upward gaze palsy, from pressure on
region of suprapineal recess.
• Hyperactive reflexes.
• Irregular respiration with apneic spells.
• Separation of cranial sutures (sutures diastasis).
In older children and adult
(with rigid cranial vault(
• headache,
• vomiting.
• gait changes,
• aducent palsy ,
• upgaze palsy.( Parinaud's syndrome )…due to dilatation
of the suprapineal recess ,which then act as a pressure
against the quadrigeminal plate
• Papilledema.
• Lose of consciousness
• Episodes of transient or sustained blindness
..due to transtentorial herniation and entrapment
of the PCA against the free edge of the
tentorium.
• 3rd
nerve palsy …transtentorial herniation of the
temporal lobe.
• Slowing of the pulse and respirations and
elevation of the systolic and diastolic
pressures….death
Investigation
• Ultrasonography to visualize the ventricular
system(when the anterior fontanelle is patent).
Also for antenatal diagnosis
• CT and /or MRI of the head; it is important to
exclude any abnormal masses and to study
the size and the shape of the ventricles, and
some time needs contrast study.
• LP in cases of communicating hydrocephalus
for both diagnostic and therapeutic…..( NPH)
Management
• Medical ………ineffective
-Acetazolamide
-frucemide
surgical treatment
Think about the Surgical treatment
• reducing the CSF production …..
• bypassing the blockage to normal CSF
flow ……
• drainage of CSF externally ……
• drainage of CSF to another absorptive
viscus
• Treat the cause (remove the
obstruction)
Surgical treatment
• removal of obstruction like posterior foss tumor
• shunting: many types of shunts
-ventriculo-peritoneal(VP),( the most common method)
-ventriculo-atrial(VA)
-Torkildsen shunt (overcame the aqueduct stenosis by passing a
catheter from the lateral ventricles into the cisternal space)
-Lumbo-peritonial shunt.
-External drainage , temporary for management for acute
hydrocephalus but the risk of infection preclude this as a means
of long term management.
-miscellaneous : the lower end may inserted into the plural space,gall
blader , ureter or bladder. (in past)
• cannulation of the aqueduct of Sylvius or third ventriculostomy (by
endoscopy or open operation) (ETV)…..the most advanced one.
• choroids plexectomy )in past)
Complications of shunts
In general shunts may have this complications
• infection, usually apparent within the first few weeks or months
following implantation ,treatment is by removal of the device and
antibiotics (intravenous or intrathecal) and usually it due to low
grade organisms .
• Obstruction.
• Acute hemorrhages
• shunt malfunction and over drainage resulting in low pressure
symptoms
• hematoma
• disconnections
• seizure
• erosion of skin (in infants) and perforation of viscus
• metastases of tumors
Arrested hydrocephalus
• Normal pressure hydrocephalus NPH
• Normotensive hydrocephalus
• Hakim's syndrome
• Hakim's-Adams syndrome
• Occult hydrocephalus
NPH
• A broad based shuffling gait (gait apraxia)
• Dementia (memory loss and a generalized
slowing of thought)
• Urinary incontinence
In addition to CT and MRI
lumbar puncture (LP) is important in the
diagnosis of NPH
….surgery
25/Octobers
Thanks.
Questions?

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Hydrocephalus

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  • 5. Physiology and circulation of CSF • The normal volume of circulating CSF is around 140ml • daily production is about 500ml/day • the CSF volume is replaced approximately …?……..times daily • Function: • The CSF is both protects and support the brain and spinal cord. • transport medium for transmitters and as a method of removing the end-products of metabolism. • CSF is mainly produced by the choroid plexus
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  • 9. Definition HYDROCEPHALUS It is defined as a disproportionate increase in the amount of CSF within the cranium, usually in association with a rise in ICP. (Ventricular enlargement with excessive CSF) And it should be differentiated from hydrocephalus ex vacuo (due to brain atrophy)
  • 12. Epidemiology and etiology .the overall incidence …not known . Incidence of congenital hydrocephalus is 1- 2/1000 live births -congenital : maternal malnutrition ,toxins,infection with toxoplasmosis and cytomegalic virus . A rare (2% of all congenital HC) ..is X-linked recessive aqueductal stenosis ( only in male and female are carrier)
  • 13. Pathogenesis 1. Obstruction of the CSF pathways a. Lesions that obstruct the ventricular system… b. Lesions that obstruct the subarachnoid space….. 2. CSF over secretion…choroid plexus papilloma .. 3. Impaired venous drainage
  • 14. Pathophysiology of hydrocephalus • Acute hydrocephalus -in animals… - In humans : 1. ICH 2. Acute decompensation of tumors bordering the ventricular system 3. Gunshot 4. SAH 5. Head injuries 6. Sudden shunt obstruction If the CSF obstruction is complete , ventricular enlargement may reach significant proportions in 3 to 6 hr.
  • 15. . Chronic hydrocephalus Several compensatory adjustments can occur including: 1-expansion of the skull 2-contraction of the cerebral vascular volume 3- volumetric enlargement of the ventricular system
  • 16. Types of hydrocephalus • Communicating (non-obstructive): CSF circulation blocked at the level of the basal cisterns, the subarachnoid space or the level of the arachnoid granulation. • Non-communicating (obstructive): the normal pathways of CSF flow are for some reason occluded such as aqueduct stenosis or as a result of a local compression from a tumor. (enlargement of ventricles proximal to the block)
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  • 18. further types of classifications • Acute and chronic • Congenital and acquired • Can be classified by etiology - posttraumatic, - post hemorrhagic - postmeningitis
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  • 20. Etiology imbalance has occurred between the normal physiological production of CSF and its absorption
  • 21. Etiology • Congenital …..? – aqueduct stenosis – Chiari malformations – Dandy-Walker malformation • Acquired – infection (post-meningitis) – post – hemorrhagic(SAH,IVH) – secondary to masses( non- neoplastic like vascular malformation, tumors like posterior foss tumors, pituitary and colloid cyst )
  • 22. Clinical features In neonatal period • Cranium enlarges at a rate more than the facial growth (craniofacial disproportion). • irritability , poor head control, nausea and vomiting • Fontanel full , bulging and wide. • Thin and glistening scalp with enlargement and engorgement of scalp veins ;due to reversal of flow from intracerebral sinuses due to increase intra cranial pressure. • Macewen's sign ( cracked pot sound on head percussion)
  • 23. • Sixth nerve (abducens) palsy, long intracranial course make this nerve very pressure sensitive. • Setting sun sign, (upward gaze palsy, from pressure on region of suprapineal recess. • Hyperactive reflexes. • Irregular respiration with apneic spells. • Separation of cranial sutures (sutures diastasis).
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  • 27. In older children and adult (with rigid cranial vault( • headache, • vomiting. • gait changes, • aducent palsy , • upgaze palsy.( Parinaud's syndrome )…due to dilatation of the suprapineal recess ,which then act as a pressure against the quadrigeminal plate • Papilledema. • Lose of consciousness
  • 28. • Episodes of transient or sustained blindness ..due to transtentorial herniation and entrapment of the PCA against the free edge of the tentorium. • 3rd nerve palsy …transtentorial herniation of the temporal lobe. • Slowing of the pulse and respirations and elevation of the systolic and diastolic pressures….death
  • 29. Investigation • Ultrasonography to visualize the ventricular system(when the anterior fontanelle is patent). Also for antenatal diagnosis • CT and /or MRI of the head; it is important to exclude any abnormal masses and to study the size and the shape of the ventricles, and some time needs contrast study. • LP in cases of communicating hydrocephalus for both diagnostic and therapeutic…..( NPH)
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  • 36. Think about the Surgical treatment • reducing the CSF production ….. • bypassing the blockage to normal CSF flow …… • drainage of CSF externally …… • drainage of CSF to another absorptive viscus • Treat the cause (remove the obstruction)
  • 37. Surgical treatment • removal of obstruction like posterior foss tumor • shunting: many types of shunts -ventriculo-peritoneal(VP),( the most common method) -ventriculo-atrial(VA) -Torkildsen shunt (overcame the aqueduct stenosis by passing a catheter from the lateral ventricles into the cisternal space) -Lumbo-peritonial shunt. -External drainage , temporary for management for acute hydrocephalus but the risk of infection preclude this as a means of long term management. -miscellaneous : the lower end may inserted into the plural space,gall blader , ureter or bladder. (in past) • cannulation of the aqueduct of Sylvius or third ventriculostomy (by endoscopy or open operation) (ETV)…..the most advanced one. • choroids plexectomy )in past)
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  • 40. Complications of shunts In general shunts may have this complications • infection, usually apparent within the first few weeks or months following implantation ,treatment is by removal of the device and antibiotics (intravenous or intrathecal) and usually it due to low grade organisms . • Obstruction. • Acute hemorrhages • shunt malfunction and over drainage resulting in low pressure symptoms • hematoma • disconnections • seizure • erosion of skin (in infants) and perforation of viscus • metastases of tumors
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  • 53. • Normal pressure hydrocephalus NPH • Normotensive hydrocephalus • Hakim's syndrome • Hakim's-Adams syndrome • Occult hydrocephalus
  • 54. NPH • A broad based shuffling gait (gait apraxia) • Dementia (memory loss and a generalized slowing of thought) • Urinary incontinence
  • 55. In addition to CT and MRI lumbar puncture (LP) is important in the diagnosis of NPH ….surgery