Seminar on Hydrocephalus and

Management Principles

Moderator: Dr. Salahadin B. (Neurosurgeon)

Presenter: Nurhusien A. (Year II Surgical

Resident)
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Aim of the Presentation:
1. Understand the normal physiology of CSF flow and pathways
2. Understand the definition and pathophysiology of hydrocephalus
3. To have basic knowledge on the etiologies of hydrocephalus
4. To understand the basic of management principles of
hydrocephalus and complications.

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Normal CSF Pathway

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 Hydrocephalus
• An abnormal accumulation of cerebrospinal fluid within the ventricles of the brain
Epidemiology
Estimated prevalence: 1–1.5%
Incidence of congenital hydrocephalus is ≈ 0.9–1.8/1000 births
Etiologies of hydrocephalus
General information:
1. Subnormal CSF reabsorption
• Obstructive hydrocephalus (AKA non-communicating)
• Communicating hydrocephalus (AKA non-obstructive)
2. CSF overproduction

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Common sites and causes of Obstruction

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 Specific etiologies of
hydrocephalus
The etiologies in of pediatric patients
1. Congenital
a) Chiari Type 2 malformation and/or myelomeningocele (MM)
b) Chiari Type 1 malformation
c) Primary aqueductal stenosis
d) Secondary aqueductal gliosis
e) Dandy Walker malformation
f) X-linked inherited disorder

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2. Acquired
a) Infectious (the most common cause of communicating HCP)
• Post-meningitis
• Cysticercosis
b) Post-hemorrhagic (2nd most common cause of communicating HCP)
• Post-SAH
• Post-intraventricular hemorrhage (IVH)
c) Secondary to masses
 Non neoplastic
 Neoplastic
d) Post-op
e) Neurosarcoidosis
f) Constitutional ventriculomegaly
g) Associated with spinal tumors

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Special forms of hydrocephalus
1. Normal pressure hydrocephalus (NPH)
2. Entrapped fourth ventricle
3. Arrested hydrocephalus
Signs and symptoms of HCP
In older children (with rigid cranial vault) and adults
Papilledema
Headache
N/V
Gait changes
Up gaze and/or abducens palsy
Asymptomatic

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In young children
Abnormalities in head circumference (OFC)
Cranium enlarges at a rate >facial growth
Irritability, poor head control, N/V
Fontanelle full and bulging
Enlargement and engorgement of scalp veins
Macewen’s sign
6th nerve (abducens) palsy
“Setting sun sign ” (upward gaze palsy)
Parinaud’s syndrome from pressure on region of suprapineal recess
Hyperactive reflexes
Irregular respirations with apneic spells
Splaying of cranial sutures

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Blindness from hydrocephalus
Blindness is a rare complication of hydrocephalus
 Occlusion of posterior cerebral arteries (PCA caused by downward
transtentorial herniation)
 Chronic papilledema causing injury to optic nerve at the optic disc
 Dilatation of the 3rd ventricle with compression of optic chiasm

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CT/MRI criteria for hydrocephalus
Specific imaging criteria for hydrocephalus
Size of both temporal horns (TH) is ≥2 mm in width and the sylvian &
interhemispheric fissures and cerebral sulci are not visible OR
Both TH are ≥2 mm, and the ratio FH:ID > 0:5
Other features suggestive of hydrocephalus
1. Ballooning of frontal horns of lateral ventricles (“Mickey Mouse”
ventricles) and/or 3rd ventricle
2. Periventricular low density on CT, or periventricular high intensity
signal on T2WI on MRI suggesting

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3. The ratio FH:ID alone
< 40%----normal
40 - 50%---borderline
> 50%---suggests hydrocephalus
4. Evans ratio - ratio of FH to maximal biparietal diameter (BPD)
measured in the same CT slice: >0.3 suggests hydrocephalus
5. Sagittal MRI may show thinning of the corpus callosum and/or
upward bowing of the corpus callosum

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Differential diagnosis of hydrocephalus
• Hydrocephalus ex vacuo
• Hydranencephaly
Chronic HCP
Features indicative of chronic hydrocephalus
Beaten copper cranium on plain skull x-ray
3rd ventricle herniating into Sella (seen on CT or MRI)
Erosion of Sella turcica which sometimes produces an empty Sella
Temporal horns may be less prominent on imaging than in acute HCP
Macrocrania
Atrophy of corpus callosum: on sagittal MRI
In infants
 Sutural diastasis
 Delayed closure of fontanelles
 Failure to thrive or developmental delay

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External hydrocephalus (Benign external
hydrocephalus)
o Enlarged subarachnoid space (usually over the cortical sulci of the
frontal poles) seen in infancy usually accompanied by abnormally
increasing head circumference with normal or mildly dilated
ventricles
o There are often enlarged basal cisterns and widening of the anterior
interhemispheric fissure
o No other symptoms or signs should be present (although there may
be slight delay only in motor milestones due to the large head)
o Etiology is unclear

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Treatment
 EH usually compensates by 12–18 mos age
 Follow serial ultrasound and/or CT to rule out abnormal ventricular
enlargement
 A shunt may rarely be indicated when the collections are bloody or for
cosmetic reasons for severe macrocrania or frontal bossing

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X-linked hydrocephalus
• Inherited hydrocephalus (HCP) with phenotypic expression in males
passed on through carrier mothers
• Abnormal gene expression results in poor differentiation and
maturation of cortical neurons macroscopic anatomical abnormalities
• L1CAM membrane bound receptor plays a significant role in CNS
development
• Classical syndromes include
• CRASH
• MSAS
• HSAS
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Radiographic findings likely present if
severe L1
• Severe symmetric HCP with predominant posterior horn dilation
• Hypoplastic CC/ACC
• Hypoplastic anterior cerebellar vermis
• Large massa intermedia
• Large quadrigeminal plate
• Rippled ventricular wall following VP shunt placement (pathognomonic)
Treatment
No intervention demonstrates improvement in retardation

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Arrested hydrocephalus
• Refer to a situation where there is no progression or deleterious sequelae
due to hydrocephalus
• State in which ventricular size remains unchanged in the absence of a shunt
or in the presence of a nonfunctioning one
• Careful follow-up is still required, particularly in children, because of
reported cases of sudden death, sometimes years after the initial diagnosis
Criteria
1. Near normal ventricular size
2. Normal head growth curve
3. Continued psychomotor development
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Entrapped fourth ventricle
• Isolated fourth ventricle
• Possibly as a result of adhesions forming from prolonged apposition
of the ependymal lining of the aqueduct due to the diversion of CSF
through the shunt
• The choroid plexus of the 4th ventricle continues to produce CSF
which enlarges the ventricle when there is 4th ventricular outlet
obstruction

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Presentation may include:
Headache
Lower cranial nerve palsies
Pressure on the floor of the 4th ventricle →facial diplegia and
bilateral abducens palsy
Ataxia
Reduced level of consciousness
Nausea/vomiting
May also be an incidental finding

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Treatment
• Shunting the ventricle either with a separate VP shunt, or linking into
an existing shunt

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Slit Ventricle Syndrome
• Lateral ventricles may collapse in some patients secondary to over
shunting or remain at a fixed size because of sub ependymal gliosis
• Raised ICP without ventricular enlargement, and therefore imaging
findings may be falsely reassuring in such cases (unresponsive
ventricles)
• Patients with progressive neurological deterioration secondary to
raised ICP may require subtemporal decompression

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Normal pressure hydrocephalus (NPH)
• Clinically important because it may cause treatable symptoms, including
one of the few forms of remediable dementia
Possible etiologies of NPH include:
 Post-SAH
 Post-traumatic
 Post-meningitis
 Following posterior fossa surgery
 Tumors, including carcinomatous meningitis
 Deficiency of the arachnoid granulations
 Aqueductal stenosis
 Idiopathic
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Clinical triad
Triad is not pathognomonic
Gait disturbance: Wide based with short, shuffling steps and unsteadiness on
turning + Patients often feel like they are “glued to the floor”+ Absence of
appendicular ataxia
Dementia: bradyphrenia (slowness of thought) and bradykinesia
Urinary incontinence: usually unwitting
Other clinical features
Age usually > 60 yrs.
Slight male preponderance
Psychiatric disturbances
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Imaging in NPH
Features on CT and MRI
1. Ventricular enlargement without block (i.e. communicating hydrocephalus)
2. Features that suggest the hydrocephalus is not due to atrophy alone
a. Periventricular low density on CT or high intensity on T2WI MRI
b. Compression of convexity sulci
c. Rounding of the frontal horns
Ancillary tests for NPH
Lumbar puncture (LP)-NPH the average OP is 15 ± 4.5 cm H2O (11 ± 3.3 mm Hg)
“Tap Test”- Consists of lumbar puncture with removal of a specific quantity of CSF
and assessment of response
Resistance testing
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Ambulatory lumbar drainage (ALD)
Continuous CSF pressure monitoring
Treatment
CSF diversionary procedures
a. VP shunt
b. Lumbar-peritoneal shunts
Follow patients clinically and with CT for ≈ 6–12 months
Endoscopic third ventriculostomy (ETV)
Outcome- most likely symptom to improve with shunting is incontinence,
then gait disturbance, and lastly dementia
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Markers for good candidates for
improvement with shunting
• Presence of the classic triad
• LP: OP > 100 mm H2O
• Continuous CSF pressure recording: pressure > 180 mm H2O or
frequent Lundberg B waves
• CT or MRI: large ventricles with flattened sulci (little atrophy)
• When symptoms have been present for a shorter time

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Treatment of Hydrocephalus
Medical treatment of hydrocephalus
HCP remains a surgically treated condition
1. Diuretic therapy
Temporizing measure mainly in pediatrics age < 1 year
Acetazolamide + furosemide + tricitrate
Watch for electrolyte imbalance and acetazolamide side effects
Weekly U/S or CT scan
Maintain therapy for a 6 month trial, then taper dosage over 2–4 weeks

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2. Spinal taps
o Serial taps (ventricular or LP) may temporize until resorption resumes
o If reabsorption does not resume when the protein content of the CSF is <100 mg/dl,
then it is unlikely that spontaneous resorption will occur in the near future
3. Surgical
Goals are optimum neurologic function and a good cosmetic result
Surgical options
 Third ventriculostomy
 Shunting : The techniques of shunt placement are covered for VP shunts , for VA
shunt , for ventriculopleural shunts, and for LP shunt
 Eliminating the obstruction: e.g. opening a stenosed sylvian aqueduct
 Choroid plexectomy (choroid plexus coagulation)
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1. Endoscopic third ventriculostomy
Indications
Obstructive HCP
Shunt infection
For patients who developed subdural hematomas after shunting
Indicated for slit ventricle syndrome
Contraindications
No absolute contraindication

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Complications
• Hypothalamic injury: may result in hyperphagia
• Injury to pituitary stalk or gland
• Transient 3rd and 6th nerve palsies
• Injury to basilar artery
• Uncontrollable bleeding
• Cardiac arrest
• Traumatic basilar artery aneurysm

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2. Shunts
Types of shunts
1. Ventriculoperitoneal (VP) shunt:
Most commonly used
Lateral ventricle is the usual proximal location
2. Ventriculo-atrial (VA) shunt (“vascular shunt”):
 Shunts ventricles through jugular vein to superior vena cava, so-called “ventriculo-atrial”
 Shorter length of tubing results in lower distal pressure
3. Torkildsen shunt:
Shunts ventricle to cisternal space
Rarely used
Effective only in acquired obstructive HCP

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4. Miscellaneous: various distal projections used historically or in patients who have had
significant problems
5. Lumboperitoneal (LP) shunt
6. Cyst or subdural shunt : from arachnoid cyst or subdural hygroma cavity, usually to
peritoneum
Disadvantages/complications of various shunts
Obstruction
Disconnection at a junction, or break at any point
Infection
Hardware erosion through skin
Seizures (ventricular shunts only)
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Act as a conduit for extra neural metastases of certain tumors
Silicone allergy
Neurological deficit
Overdrainage
Underdrainage
Bleeding
Wound breakdown
CSF leakage
Hernias
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PATIENT OUTCOMES
Hydrocephalus can be successfully treated surgically with reversal of
the initial neurological deficits.
Patients exhibit variable improvement after successful CSF diversion,
even in the context of proven hydrocephalus and raised ICP
Follow-up at 3, 6, and 12 months; careful evaluation of
nonresponders for shunt malfunction; and the use of objective scales
for measurement of improvement

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References:
1. Hand book of Neurosurgery, Eighth Edition
2. Principles of Neurosurgery, Second Edition
3. Youmans Neurological Surgery, Sixth Edition
4. UpToDate

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 Thank you!!!

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