Hydrocephalus

Ventriculo-peritoneal shunt
Diana Tănase
Ventricular system
 Hydrocephalus
Etiology

• subnormal CSF reabsorption

• rarely CSF overproduction

…which result in accumulation of CSF in the

ventricular system
 FUNCTIONAL CLASSIFICATION
Two main functional
subdivisions of
hydrocephalus (HCP)
1. obstructive (AKA non-
communicating): block
proximal to the arachnoid
granulations(AG).

2. communicating (AKA
non-obstructive): CSF
circulation blocked at
level of AG
 SPECIAL FORMS OF
HYDROCEPHALUS AND
"PSEUDOHYDROCEPHALUS"
1. conditions that are not actually hydrocephalus
"pseudohydrocephalus"
A. hydrocephalus ex vacuo: cerebral atrophy
B. otitic hydrocephalus
C. external hydrocephalus
D. Hydranencephaly

2. normal pressure hydrocephalus (NPH)

3. entrapped fourth ventricle

4. arrested hydrocephalus
 Normal pressure hydrocephalus
-adult form of hydrocephalus-

As originally described, the hydrocephalus of NPH was

considered to be idiopathic.
"secondary NPH“ causes:
1. post-SAH
2. post-traumatic
3. post-meningitis
4. following posterior fossa surgery
5. tumors
6. also seen in some patients with Alzheimer's disease (AD)
7. deficiency of the arachnoid granulations
8. aqueductal stenosis
Clinical triad
Hakim triad

1. gait disturbance
2. dementia: primarily
memory impairment
with bradyphrenia
(slowness of thought)
and bradykinesia
3. urinary incontinence
 Other clinical features
Age usually> 60 yrs.
Slight male preponderance.

Differential diagnosis
- Alzheimer's disease
- Parkinson's disease
Hydrocephalus Classification

• Congenital
• Acquired
 Congenital, usually paediatric
A. Chiari Type 2 malformation and/or
myelomeningocele (MM)
B. Chiari Type 1 malformation
C. primary aqueductal stenosis of Sylvius
aqueduct
D. secondary aqueductal gliosis
E. Dandy Walker malformation: atresia of
foramina of Luschka & Magendie
F. X-linked inherited disorder: rare
 Acquired
A. infectious
1. post-meningitis
2. cysticercosis
B. post-hemorrhagic
1. post-SAH
2. post-intraventricular hemorrhage (IVH)
C. secondary to masses
1. non neoplastic: e.g. vascular malformation
2. neoplastic: e.g. Medulloblastoma, pituitary tumor
D. post-op: following p-fossa tumor removal.
E. Neurosarcoidosis
F. "constitutional ventriculomegaly": asymptomatic. No treatment
required.
G. associated with spinal tumors
 DIFFERENTIAL DIAGNOSIS OF
HYDROCEPHALUS

Conditions that may mimic HCP but are not due to

inadequate CSF absorption include:
1. atrophy: sometimes referred to as
"hydrocephalus ex vacuo"
2. hydranencephaly
3. developmental anomalies where the ventricles
appear enlarged:
A. agenesis of the corpus callosum
B. septa-optic dysplasia
 SIGNS AND SYMPTOMS OF
ACTIVE HCP
In young children
1. cranium enlargement
2. irritability, poor head control, NN
3. fontanelle full and bulging
4. enlargement and engorgement of scalp veins
5. Macewents sign: cracked pot sound on percussing over dilated
ventricles
6. 6th nerve (abducens) palsy
7. "setting sun sign" (upward gaze palsy)
8. hyperactive reflexes
9. irregular respirations with apneic spells
10. splaying of cranial sutures

In older children/adults with rigid cranial vault

Symptoms of increased ICP, including:
- papilledema,
- HI A,
- NN,
- gait changes,
- upgaze and/or abducens palsy.
Slowly enlarging ventricles may initially be asymptomatic.
CHRONIC HCP
Features indicative of chronic hydrocephalus
(as opposed to acute hydrocephalus):
1. beaten copper cranium on plain skull x-ray.

2. 3rd ventricle herniating into sella (seen on

CT or MRI)
3. erosion of sella turcica
4. the temporal horns may be less prominent on
CT than in acute HCP
5. Macrocrania
6. atrophy of corpus callosum
7. in infants
A. sutural diastasis
B. delayed closure of fontanelles
C. failure to thrive or developmental delay
 CT/MRI CRITERIA OF
HYDROCEPHALUS
Hydrocephalus HCP is
suggested when either:
A. both TH ≥ 2 mm in
width
OR
B. both TH ≥ 2 mm
+ ratio FH/ID > 0.5

TH= temporal horns

FH= the largest width of
the frontal horns
ID= the internal diameter
 Other features suggestive of hydrocephalus:

1. ballooning of frontal
horns of lateral ventricles 2. periventricular low density
("Mickey Mouse" on CT
ventricles) and/or 3rd
ventricle
3. used alone, the ratio FH/ID
<40% normal
40-50% borderline
>50% suggests hydrocephalus
4. Evans ratio (or index): ratio of FH to BPD measured in the same CT slice:
> 0.3 suggests hydrocephalus
5. sagittal MRI may show thinning and/or upward bowing of the corpus callosum

BPD= maximal biparietal diameter

 Treatment of hydrocephalus
MEDICAL
Acetazolamide may be helpful for temporizing.
Diuretic therapy may be tried in premature infants with bloody CSF.
This should only be considered as an adjunct to definitive treatment or as a
temporizing measure.
• acetazolamide (a carbonic anhydrase inhibitor): 25 mg/kg/day
• simultaneously start furosemide: 1 mg/kg/day
• to counteract acidosis:
+ tricitrate 4 ml/kg/day
+ measure serial electrolytes, and adjust dosage to maintain serum HCO3 >
18mEq/L
• watch for electrolyte imbalance and acetazolamide side effects: lethargy,
tachypnea, diarrhea, paresthesias (e.g. tingling in the fingertips)
• perform weekly U/S or CT scan and insert ventricular shunt if progressive
ventriculomegaly occurs. Otherwise, maintain therapy for a 6 month trial,
then taper dosage over 2-4 weeks. Resume 3-4 mos of treatment if
progressive HCP occurs
 SPINAL TAPS
HCP after intraventricular hemorrhage may be only
transient Serial taps (ventricular or LP) may
temporize until resorption resumes but LPs can
only be performed for communicating HCP.
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
(i.e. a shunt will usually be necessary).
 SURGICAL
Goals of therapy:
Normal sized ventricles is not the goal of therapy. Goals are optimum
neurologic function and a good cosmetic result.

Options include:
1. choroid plexectomy: for communicating hydrocephalus
May reduce the rate but does not totally halt CSF production (only a
portion of CSF is secreted by the choroid plexus, other sources
include the ependymal lining of the ventricles and the dural sleeves
of spinal nerve roots).

2. eliminating the obstruction: e.g. opening a stenosed sylvian

aqueduct.

3. third ventriculostomy

4. shunting
 Third ventriculostomy
Indications:
- used in patients with obstructive HCP
- an option in managing shunt infection
- an option for patients who developed subdural hematomas after shunting
- for slit ventricle syndrome.
Contraindications: Communicating hydrocephalus

Complications:
1. hypothalamic injury
2. transient 3rd and 6th nerve palsies
3. uncontrollable bleeding
4. cardiac arrest
5. traumatic basilar artecy aneurysm

Success rate:
~56% Highest maintained patency rate is with previously untreated acquired AqS.
There is a low success rate (only ~20% of TVs will remain patent) if there is pre-existing
pathology including:
1. tumor
2. previous shunt
3. previous SAH
4. previous whole brain radiation
5. adhesions
 TYPES OF SHUNTS
SHUNT TYPE BY CATEGORY
1. ventriculoperitoneal (VP) shunt:
A. most commonly used shunt
B. lateral ventricle is the usual proximal location

2. ventriculo-atrial (VA) shunt ("vascular shunt"):

A. ventricles -> jugular vein -> superior vena cava
B. treatment of choice when abdominal abnormalities are present

3. Torkildsen shunt:
A. shunts ventricle to cisternal space
B. rarely used
C. effective only in acquired obstructive HCP
 TYPES OF SHUNTS
4. miscellaneous: used historically or in patients who have
had significant problems with traditional shunt locations:
A. pleural space (ventriculopleural shunt): not a first
choice, but a viable alternative if the peritoneum is not
available.
B. gall bladder
C. ureter or bladder: causes electrolyte imbalances

5. lumboperitoneal (LP) shunt

- only for communicating HCP

6. cyst or subdural shunt: from arachnoid cyst or subdural

hygroma cavity, usually to peritoneum
 Shunts
Ventricular catheterization
Most common insertion sites:
1. occipital-parietal region: commonly used for
CSF shunt
A. entry site: a number of means have been
described, including:
1. Frazier burr hole: placed
prophylactically before p-fossa crani for
emergency ventriculostomy in event of
post-op swelling.
Location: 3-4 cm from midline, 6-7 cm
above inion
2. parietal boss
3. follow point from mid-pupillary line
parallel to sagittal suture until it intersects
line extending posteriorly from the top of the
pinna
4. ~3 cm above and ~3 cm posterior
to top of pinna

B. trajectory: insert the catheter parallel to

skull base:
- aim for middle of forehead
C. insertion length: ideally, the tip should be
just anterior to the foramen of Monro in the
frontal horn.
 3. Dandy's point: 2 cm
from midline, 3 cm
above inion (may be
more prone to damage
visual pathways than
above)

2. Keen's point (posterior

parietal): (placement in
trigone) 2.5-3 cm
posterior and 2.5-3 cm
superior to pinna
4. Kocher's point (coronal): places
catheter in frontal horn. The right
side is usually used. Commonly
employed for ICP monitors

A. entry site: 2-3 cm from midline which is

approximately the mid-pupillary line with
forward gaze, 1 cm anterior to coronal
suture (to avoid motor strip)
B. trajectory: direct catheter perpendicular to
surface of brain, aiming in coronal plane
towards medial canthus of ipsilateral eye
and in AP plane towards EAM
C. insertion length: advance catheter with
stylet until CSF is obtained
(should be < 5-7 cm depth; this may be 3-
4 cm with markedly dilated ventricles).
Advance catheter without stylet 1 cm
deeper.
CAUTION: if CSF is not obtained until
very long insertion length (e.g. ≥8 cm) the
tip is probably in a cistern (e.g. prepontine
cistern) which is undesirable
 Ventriculostomy/ICP monitor
AKA intraventricular catheter (IVC) or external
ventricular drainage (EVD).

INSERTION TECHNIQUE
The right (non-dominant) side is preferred.
Five minute Betadine prep.
Site: approximately Kocher's point.
To avoid motor strip, enter 1-2 cm anterior to
coronal suture
*estimated position of coronal suture: follow line up
midway between lateral canthus and EAM, and to
avoid the sagittal sinus, 2-3 cm lateral to midline.

Incision oriented in sagittal plane;

elevate periosteum;
place self-retaining retractor;
make twist drill hole.
Bone-wax edges to stop bone bleeding;
cauterize dura with bipolar coagulator;
incise dura in cruciate fashion with #11 scalpel blade;
cauterize incised dural edges and then pia/arachnoid
with bipolar.
For ventriculostomy:
insert catheter perpendicular to brain surface to a depth of 5-7 cm.
With any ventricular enlargement, CSF should flow at least by 3-4 cm
depth.
If no CSF is encountered here and the catheter is passed further until
CSF is obtained, it is unlikely to be due to catheterization of frontal
horn of lateral ventricle.
If unsuccessful after a maximum of three attempted passes, then place
a subarachnoid bolt or intraparenchymal monitor.
REMOVAL
Patients receiving anticoagulants need to have normal coagulation and
platelet function before discontinuing the catheter to reduce the risk
of intracranial hemorrhage.
For heparin and LMW heparin, stop the drug 24 hours prior to
discontinuing the drain.

SUMP DRAINAGE
The tip of a 25 gauge butterfly may be bent at a 90° angle, and inserted
into a subcutaneous reservoir for prolonged ventricular drainage.
The use of a one-way valve, continuous antibiotics (ampicillin and
cloxacillin) and meticulous technique was credited for the lack of
infection.
 Ventricular shunts
1. position: supine with shoulder roll
2. Implants
3. equipment:
A. C-arm for ventriculo-atrial shunts
B. endoscopic display (e.g. If NeuroPen is used)
C. image guided navigation system (infrequently used)
4. consent (in lay terms for the patient- not all-inclusive):
A. procedure: surgery to insert a permanent drainage tube from the brain to the
abdomen, outside of the lungs, vein near the heart (as appropriate) to drain excess
cerebrospinal fluid
B. alternatives: nonsurgical management (rarely effective for hydrocephalus), third
ventriculostomy (for certain cases)
C. complications: infection, suboptimal position which might require re-operation,
failure to relieve hydrocephalus/symptoms, subdural hematoma, bleeding in the brain,
shunts are mechanical devices and will eventually fail (break, block up, move ... ) and
need repair/replacement (sometimes sooner rather than later). Abdominal shunts:
risk of bowel injury (which could require further surgery)
 Ventricular catheter
Occipital burr hole is used in most cases for insertion site of ventricular catheter.
Some prefer a frontal burr hole (Kocher's point) citing a lower incidence of failure from
choroid plexus occlusion.

An inverted "J" shaped incision is used to keep hardware from lying directly under the skin
incision (minimizes risk of skin breakdown and also creates additional barrier to infection
of subjacent hardware).
CSF should be sent for culture at the time of insertion since it has been estimated that in
3% of patients the CSF is already infected.
4 mg of preservative-free gentamicin may be instilled into the ventricular catheter by the
technique of barbotage.
If you think the catheter is in the ventricle, but you don't get CSF flow, it may be due to low
pressure, you can compress the jugular veins or lower the head of the bed to try and
induce CSF flow.

Connectors
If a connector must be used near the clavicle, placing it below the clavicle increases the risk
of disconnection vs. placing the connector above the clavicle.
DISTAL CATHETER PLACEMENT

The general order of preference for distal catheter

placement:
1. peritoneal cavity
2. pleural space
3. right atrium or superior vena cava
4. less frequently used distal shunt sites
A. gall bladder
B. internal jugular vein
C. superior sagittal sinus
 VENTRICULOPERITONEAL
SHUNT
Peritoneal catheter
For small children, use at least 30 cm length of
intraperitoneal tubing to allow for continued
growth (120 cm total length of peritoneal tubing
recommended).
A silver clip is placed at the point where the
catheter enters the peritoneum so that the
amount of residual intraperitoneal catheter can
be determined on later films.
Distal slits on the peritoneal catheter may increase
the risk of distal obstruction, and some authors
recommend that they be trimmed off.
 Open technique: A vertical incision lateral and superior to the umbilicus
is one of several choices.

Layers
1. subcutaneous fat
2. anterior rectus sheath
3. abdominis rectus
muscle fibers: should
be split longitudinally
4. posterior rectus sheath
5. preperitoneal fat
6. peritoneum
Trocar technique:
1. place a Foley catheter to decompress the bladder
2. 1 cm skin incision above and lateral to the umbilicus
3. pull abdominal skin anteriorly (away from patient)
4. insert trocar aiming toward the ipsilateral iliac crest
5. feel 2 "pops" of penetration: 1 = anterior rectus sheath, 2 = posterior rectus
sheath/peritoneum
6. peritoneal catheter should feed easily through trocar
 VP SHUNT, POST-OP ORDERS
(ADULT)
1. flat in bed
2. if peritoneal end is new or revised, do not
feed until bowel sounds resume (at least
24 hrs)
3. shunt series (AP & lateral skull, and
chest/ abdominal x-ray) as baseline for
future comparison
 Disadvantages/complications of
various shunts
Those that may occur with any shunt:
A. obstruction: the most common cause of shunt malfunction
- proximal: ventricular catheter (the most common site)
- valve mechanism
- distal
B. disconnection at a junction, or break at any point
C. infection
D. hardware erosion through skin, usually only in debilitated patients
E. seizures (ventricular shunts only)
Seizure risk is questionably higher with frontal catheters than with parieto-occipital.
F. act as a conduit for extraneural metastases of certain tumors (e.g. medulloblastoma).
Low risk.
G. silicone allergy: rare.
May require fabrication of a custom silicone-free device (e.g. polyurethane)
Disadvantages/complications of VP
shunts:
A. inguinal hernia
B. need to lengthen catheter with growth
C. obstruction of peritoneal catheter
D. peritonitis from shunt infection
E. hydrocele
F. CSF ascites
G. tip migration
- into scrotum
- perforation of a viscus: stomach, bladder
- through the diaphragm
H. intestinal obstruction (as opposed to
perforation): rare
I. volvulus
J. intestinal strangulation
K. overshunting: more likely than with VA shunt.
Some recommend LP shunt for communicating
hydrocephalus.
 MISCELLANEOUS SHUNT
HARDWARE
1. tumor filter: used to prevent
peritoneal or vascular seeding in
tumors that may metastasize
through CSF (e.g. medulloblastoma,
PNETs, ependymoma); may
eventually become occluded by
tumor cells and need replacement;
2. antisiphon device: prevents
siphoning effect when patient is
erect
3. "horizontal-vertical valve" (H-V valve)
used with LP shunts to increase the
valve resistance when the patient is
vertical to prevent overshunting
4. variable pressure valves that may be
externally programmed
5. on-off device: used to open or
occlude shunt system by external
manipulation of shunt
Programmable shunt valves

– Components
• Inlet occluder
• Reservoir
• Outlet occluder

One-way valve
Pressure settings
 Shunt problems
• Problems associated with shunt insertion
• Problems in patients with established CSF
shunt
 Problems associated with shunt
insertion
1. intraparenchymal or intraventricular
hemorrhage
2. Seizures
3. malposition
A. of ventricular catheter
B. of distal catheter
4. infection
 Problems in patients with
established CSF shunt
Shunt "problems" usually involve one or more of the following:
1. Undershunting
2. infection
3. overshunting: slit ventricle syndrome, subdural hematomas
4. seizures
5. problems related to the distal catheter
A. peritoneal
B. atrial
6. skin breakdown over hardware: infection or silicone allergy
7. hemorrhage at time of insertion: uncommon in pediatrics
 Overshunting -subdural
-slit ventricles- hematomas-
 TAPPING A SHUNT
aka aspirating a shunt
Indications to tap a shunt or ventricular access device include:
1. to obtain CSF specimen
A. to evaluate for shunt infection
B. for cytology
C. to remove blood
2. to evaluate shunt function
A. measuring pressures
B. contrast studies:
3. as a temporizing measure to allow function of a distally occluded shunt
4. to inject medication
A. antibiotics: for shunt infection or ventriculitis
B. chemotherapeutic (antineoplastic) agents
5. for catheters placed within tumor cyst (not a true shunt):
A. periodic withdrawal of accumulated fluid
B. for injection of radioactive liquid (usually phosphorous) for ablation