Hydrocephalus and Shunts:

Is it just plumbing,
or is it more complicated?

Mark S. Dias, MD, FAANS, FAAP

Venous Sinus
 Hydrocephalus
• Imbalance between
production and
absorption of CSF
• In spina bifida, always
due to inadequate
absorption
– Blockage within ventricles
– Blockage in subarachnoid
space
• Not all or none - variable
degrees of blockage
VP and VA Shunts
 Shunt Valves
• Most are Differential Pressure Valves
– Open when the pressure above the valve (A) is
greater than the pressure below the valve (B) by a
certain amount (the valve opening pressure)

A B
A B
 Shunt Valves
• Most are Differential Pressure Valves
– Open when the pressure above the valve (A) is
greater than the pressure below the valve (B) by a
certain amount (the valve pressure)

A B
– Can be because the pressure above the valve (A) is
high or the pressure below the valve (B) is low
 Shunt Settings
• Shunts come in different
pressure settings
• Reflects how high the fluid
pressure would be before
the valve opens
• Measured in cm of water
pressure
• Higher setting reflects less
drainage, lower reflects
greater drainage
• Most commonly ranges
between 3 and 20 cm water
 The Problem with Shunts:
Siphoning
• When the child is
upright, there is a A

negative pressure
below the valve (B)
relative to the pressure
above the valve (A),
equal to the difference
(in cm) between the ear
B
and abdomen
 The Problem with Shunts:
Siphoning
• When upright, B is much
less than A, leading to
siphoning of CSF from the
head
• The valve will therefore
stay open all the time,
regardless of its pressure
setting
• In theory, this should
drain all of the CSF out of
the head!
• Doesn’t actually happen
 Problems with Overdrainage

• Overdrainage
headaches (usually
temporary)
• Subdural hematoma
• Repeated malfunction
• Slit ventricle syndrome
 Solutions for Overdrainage

• Siphon Control Devices

• Programmable Valves

• Gravitational Valves
 Siphon Control Devices
• Allow for direct
throughput when lying
down
• Direct fluid along
alternative pathway
with higher resistance
when upright
• Several different
designs
Programmable Valves
• Allow adjustment of the
valve setting (opening
pressure)
• Varies typically between
3-20 cm water pressure
• Higher and lower
pressures available from
some manufacturers
Programming the Valve
Programming the Valve
Medtronic Strata II valve
Medtronic Strata Valve
 Gravitational Assist Valve (GAV)
Aesculap
• Can select two separate pressures, one for
recumbent and one for upright posture (10/40)
 Gravitational Assist Valve (GAV)
• Six different gradations when going from
recumbent to upright
 ProGAV (Aesculap)
• Adds programmability to GAV
• Programmable Shunt Assist available
 So why doesn’t everyone have a
programmable valve?
• Expense
• No clear benefit over non-
programmable shunts
(Xu, 2013)
• More parts; increased potential
for malfunction
• Need for reprogramming after
MRI scans
• Magnetic fields can potentially
affect shunt setting (gaming
units, iPad, stereo headphones)
when close
Tactile Adjustments of
Programmable Shunts
Clinical Improvement Despite Increased
Ventricular Size after Shunt Revision
• Child comes to hospital with headaches,
vomiting, has increased ventricle size on CT
• Undergoes proximal shunt revision, with
blocked proximal catheter
• Complete relief of all symptoms post-op
• At routine follow up visit, new baseline CT
shows increased ventricle size!
Madenford CT pre- and post
Negative Pressure Hydrocephalus
• Child comes to hospital with headaches,
vomiting, CT shows increased ventricle size
• Undergoes routine shunt revision replacing
entire shunt
• Awakens with worse headaches, becomes
sleepy
• CT shows even larger ventricles
• Shunt tap shows negative pressures and
excellent proximal flow
Negative Pressure Hydrocephalus
• Shunt left in place and external drain inserted
on opposite side
• Drain placed at negative pressures, ventricles
gradually decrease in size over several days
• Drain removed, ventricles remain small and
child does well thereafter
• Illustration of “decreased brain turgor”,
“floppy brain” or “negative pressure
hydrocephalus (Pang, Rekate)
 Negative Pressure Hydrocephalus
• LaPlace’s Law: The greater
the radius of a sphere, the
less pressure is required to
keep the wall tension the
same
• Balloon analogy
• Not clear why this occurs in
some brains and not others
• Prior brain damage (trauma,
radiation) contributes
 Acquired Negative Pressure
Hydrocephalus
• Child with shunt presents with fevers
• Gets a spinal tap looking for infection
• Develops severe headaches and vomiting after spinal
tap (no headaches before)
• Headaches are much worse when upright, better when
recumbent
• CT shows increased ventricle size compared with
baseline
• Shunt tap shows negative pressures, excellent proximal
flow, withdrawing CSF makes headaches worse
 Acquired Negative Pressure
Hydrocephalus
• Spinal tap created leakage from the back
– Produced classic low pressure spinal headaches
• Global intracranial pressure is LOW
– lower than the shunt valve opening pressure
• Shunt valve can’t open because of low pressure
• Moreover, ventricular and subarachnoid spaces
don’t communicate
• Ventricles enlarge under negative pressure
(Dias, 1999)
 Double Armed Shunts
• Child undergoes shunt insertion, presents with
collapse of shunted ventricle but no change in
the other ventricle, which remains large
• Needs a shunt of the other ventricle
• Second shunt catheter inserted and is connected
to the existing shunt via T-connector to the
tubing just beyond the shunt valve
• Child gets worse, CT shows collapse of second
ventricle, enlargement of the first
 Double-Armed Shunts
• First catheter, above the
valve, drains only when
valve opening pressure
is exceeded
• Second catheter, below
valve, can drain freely at
lower pressure than
first
• First catheter won’t
drain until all fluid from
second ventricle is gone
 Double Armed Shunts
• All multi-shunt catheters should drain at the
same pressure setting
– Connected to each other above the valve or
– Shunted separately using the same shunt valve
setting for all of them
• Exception is the child with subdural collection
due to overdrainage of CSF, where preferential
drainage of the subdural fluid is preferable
 Shunted Hydrocephalus in Spina Bifida
• ANY deterioration in neurological function can
potentially be due to shunt malfunction
– Headaches, vomiting, lethargy/coma
– Seizures (rare, only about 7%)
– Significant change in behavior or school performance
– Changes in swallowing, vocal cord weakness (Chiari)
– Scoliosis (syringomyelia)
– Arm or leg weakness, changes in gait, ortho deformities
– Changes in urinary or bowel function
– Papilledema (swelling in optic nerve)
Thank You!
Thank you!