PEDIATRIC
HYDROCEPHALUS
Sinan ÇAKIRER, M.D.
Kartal Research Hospital,
Dept. of Medical Imaging, Istanbul-TURKEY
18th European Congress of Radiology, March 3-7, 2006, Vienna-AUSTRIA
Embryology
• Closure of neural
hindbrain
midbrain
forebrain
tube by 28 days of
gestation.
• Certain portions of
central lumen
lateral
expand to form
3rd
basic pattern of
cerebral aqueduct
ventricular sytem.
4th
The fluid-filled cavities in the
developing neural tube of the
embryo form the ventricles of the
mature brain.
Ventricles
 The ventricular surfaces
body
ant
po
are lined by a single layer
of ependymal cells with
tight junctions.
in
 Ventricles are filled with
cerebrospinal fluid (CSF)
produced by the choroid
plexus.
Choroid Plexus
 Mesenchymal invagination (leading to the
formation of choroid plexuses) of the roof
of 4th ventricle (2nd gestational month).
 Followed by those of lateral and 3rd
ventricles.
 Choroid plexuses are initially larger,
gradually diminishing in size.
Choroid Plexus
Choroid Plexus
The choroid plexus
consist of highly
vascularized,
"cauliflower-like"
masses of pia mater
tissue that dip into
pockets formed by
ependymal cells.
CEREBROSPINAL FLUID
(CSF)
A specialized extracellular
fluid produced by choroid
plexi which bathes, supports
and protects the brain and
spinal cord.
CEREBROSPINAL FLUID
(CSF)
 CSF is produced by choroid plexi of lateral,
3rd and 4th ventricles.
 Normal total volume of CSF is about 130-150
ml.
 30-50 ml in the ventricles, 100 ml in the
subarachnoid space.
 CSF is produced/resorbed by the choroid
plexus/arachnoid granulations at a rate of
about 20 ml / hr or 500 ml / day.
CSF
Lateral ventricle
Foramen
of Monro
3rd ventricle
Aqueduct of Sylvii
4th ventricle
Foramen of
Luschka and
Magendi
Subarachnoid space
CSF
CSF then flows up
to the arachnoid
granulations
(groups of
arachnoid villi) in
the superior
sagittal sinus
where it passes
through
the one-way
valves into the
venous blood.
CSF
HYDROCEPHALUS
Increase in
ventricular
size due to an
imbalance
between the
production of
CSF and its
drainage by
the arachnoid
villi.
CLINICAL
MANIFESTATIONS




Headache,
nausea/vomiting,
Cognitive impairment,
decreased level of
consciousness,
Papilledema, decreased
vision and abducens
palsies,
Gait abnormalities,
CLINICAL MANIFESTATIONS





Dilated scalp veins,
Anterior fontanel wide open and bulging,
increased head circumference,
Setting sun sign,
Brisk tendon reflexes, spasticity
Clonus, Babinsky sign (+)
IMAGING STUDIES
 X-RAY PLAIN FILMS:



SEPARATION OF SUTURES
EROSION OF POSTERIOR CLINOIDS
INCREASED CONVOLUTIONAL
MARKINGS (BEATEN CUPPER
APPEAREANCE)
 ULTRASOUND
 CT
 MRI
HYDROCEPHALUS
Ventricular dilation and increased
intracranial pressure due to discrepancy
between CSF production and absorption
DDx: Atrophy (Ex vacuo dilatation of
ventricles)
DDx: Hydranencephaly
HYDROCEPHALUS
Commensurate dilatation of the
temporal horn with the bodies of
the lateral ventricles
ATROPHY
Temporal horn is not dilated
HYDRANENCEPHALY
 Cerebral hemispheres are virtually absent
 Replaced by membranous sacs of CSF
 Vascular injury: bilateral in utero internal
carotid artery infarction
 Large cystic mass, no cerebral cortex
 Midbrain, basal ganglia, and posterior fossa
are usually normal.
 Shunting not indicated (unlike extreme
hydrocephalus)
HYDRANENCEPHALY
Shunt ???
HYDROCEPHALUS
Dilatation of anterior and posterior
recesses of the 3rd ventricle;
decreased mamillo-pontine distance
HYDROCEPHALUS
Upward bowing of the corpus callosum
HYDROCEPHALUS
 Transependymal
CSF
resorption/migration
in the periventricular
white matter
HYDROCEPHALUS
The situation might be compensated or
noncompensated depending on the presence
of transependymal CSF migration.
CLASSIFICATION
Three major mechanisms account for the
development of hydrocephalus:
*Overproduction of CSF (communicating type),
*Obstruction to CSF flow (intraventricular,
non-communicating),
*Decreased absorption of CSF by arachnoid
villi (extraventricular, communicating)
OVERPRODUCTION OF CSF
Choroid plexus papillomas:
Large aggregations of
choroidal fronds,
Infancy,
Large amounts of CSF,
Atria of the lateral ventricles
are the most common
locations.
Courtesy of Prof. A. Cila
NON-COMMUNICATING
HYDROCEPHALUS
HYDROCEPHALUS SECONDARY TO INTRAVENTRICULAR
OBSTRUCTION OF CSF
Mechanism: Obstruction to CSF flow within the ventricular system,
common at narrow points such as foramen or aqueduct
*Tumors (benign and malignant),
* Foraminal gliosis/stenosis,
*Aqueductal stenosis/web/gliosis,
*Congenital anomalies (Chiari malformations, Dandy-Walker
malformations,…)
NON-COMMUNICATING
HYDROCEPHALUS
Lateral ventricle
Foramen Monro
3rd ventricle
*Stenosis/gliosis,tumor,colloid cyst
Courtesy of Prof. A. Cila
NON-COMMUNICATING
HYDROCEPHALUS
Lateral ventricle
Foramen Monro
3rd ventricle
*Stenosis/gliosis,tumor,colloid cyst
NON-COMMUNICATING
HYDROCEPHALUS
Lateral ventricle
Foramen Monro
3rd ventricle
*Stenosis/gliosis,tumor,colloid cyst
NON-COMMUNICATING
HYDROCEPHALUS
3rd ventricle
Aqueduct of Sylvius
4th ventricle
*Stenosis/gliosis,tumor,infection
NON-COMMUNICATING
HYDROCEPHALUS
3rd ventricle
Aqueduct of Sylvius
4th ventricle
*Stenosis/gliosis,tumor,infection
NON-COMMUNICATING
HYDROCEPHALUS
3rd ventricle
Aqueduct of Sylvius
4th ventricle
*Stenosis/gliosis,tumor,infection
Courtesy of Prof. A. Cila
NON-COMMUNICATING
HYDROCEPHALUS
3rd ventricle
Aqueduct of Sylvius
4th ventricle
*Stenosis/gliosis,tumor,infection
NON-COMMUNICATING
HYDROCEPHALUS
3rd ventricle
Aqueduct of Sylvius
4th ventricle
*Stenosis/gliosis,tumor,infection
NON-COMMUNICATING
HYDROCEPHALUS
CONGENITAL MALFORMATIONS
Vein of Galen Malformation
3 month-old baby boy with enlarging head
circumference, and an audible cranial bruit.
NON-COMMUNICATING
HYDROCEPHALUS
CONGENITAL MALFORMATIONS
Chiari I
NON-COMMUNICATING
HYDROCEPHALUS
CONGENITAL MALFORMATIONS
Chiari II
NON-COMMUNICATING
HYDROCEPHALUS
CONGENITAL MALFORMATIONS
Chiari III
NON-COMMUNICATING
HYDROCEPHALUS
CONGENITAL MALFORMATIONS
DANDY-WALKER
COMMUNICATING
HYDROCEPHALUS
HYDROCEPHALUS SECONDARY TO
EXTRAVENTRICULAR OBSTRUCTION OF CSF
Mechanism: Decreased absorption of CSF by
arachnoid villi
*subarachnoid and intraventricular bleeding,
*inflammatory conditions (meningitis),
* tumoral seeding to subarachnoid spaces,
*venous hypertension.
COMMUNICATING
HYDROCEPHALUS
*Subarachnoid and intraventricular bleeding
COMMUNICATING
HYDROCEPHALUS
*Inflammatory conditions (meningitis)
COMMUNICATING
HYDROCEPHALUS
*Inflammatory conditions (meningitis)
COMMUNICATING
HYDROCEPHALUS
* Tumoral seeding to subarachnoid spaces
Courtesy of Prof. A. Cila
TREATMENT OF HYDROCEPHALUS
Persistent compression of the brain
Gliosis, cortical distortion, neuronal
damage, axonal tearing along
longitudinal fibers (internal capsule),
and myelin destruction
Treatment is mandatory
TREATMENT OF HYDROCEPHALUS
•Ventricular shunting (VP-VA shunt):
CSF diversion
• Endoscopic third ventriculostomy
(E3V); endoscopic aqueductoplasty
(EA)
TREATMENT OF HYDROCEPHALUS
• Ventricular shunting (VP-VA shunt)
Assess the fxn of shunt
by determining the
position of tip of
ventriculostomy tube
(around f. Monro), and
the reduction in
ventricular size.
TREATMENT OF HYDROCEPHALUS
• Ventricular shunting
complications
-Shunt malfxn-nonfxn (due to occlusion of ventricular
catheter, disconnection of the shunt components, …)
-Infection (ventriculitis)
-Subdural hematomas
-Post-shunt dural fibrosis
-Slit ventricle syndrome
-Abdominal complications (ascites, pseudocyst formation,
perforation of a viscus,…)
TREATMENT OF HYDROCEPHALUS
• Ventricular shunting (VP shunt) complications
TREATMENT OF HYDROCEPHALUS
• Ventricular shunting (VP shunt) complications
TREATMENT OF HYDROCEPHALUS
• Ventricular shunting (VP shunt) complications
TREATMENT OF HYDROCEPHALUS
•Endoscopic third ventriculostomy (E3V)
Courtesy of Prof. Y. Ersahin
TREATMENT OF HYDROCEPHALUS
•Endoscopic third ventriculostomy (E3V)
Courtesy of Prof. Y. Ersahin
TREATMENT OF HYDROCEPHALUS
•Endoscopic aqueductoplasty (EA)
Courtesy of Prof. Y. Ersahin
TREATMENT OF HYDROCEPHALUS
•Endoscopic aqueductoplasty (EA)
Courtesy of Prof. Y. Ersahin
BENIGN INFANTILE
ENLARGEMENT OF
SUBARACHNOID SPACES
Infants with macrocephaly or rapid head growth, no underlying abnormality,
usually normal development after 2nd year
THANK YOU FOR YOUR KINDEST
ATTENDANCE…
18th European Congress of Radiology, March 3-7, 2005, Vienna-AUSTRIA