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RADIOLOGYSubj ect

PEDIATRIC NEURO RADIOLOGYTopic
DR. BANDONGLecturer
2ND SHIFTING/ SEPT 25,2008 Shifting /Date
COFFEE LOVERS Trans group

PEDIATRIC NEURORADIOLOGY
STAGE 1: DORSAL INDUCTION
\u2013 Formation and closure of the Neural
Tube
\u2022
3-4 weeks
\u2022
THREE PHASES:
a) Neurulation
b) Canalization
c) Retrogressive differentiation
ANENCEPHALY
-
Failure of brain and skull development
-
Most severe anomaly
-
UTZ diagnosis as early as 20 weeks
-
Polyhydramnios, high alpha-fetoprotein
-
Death
CEPHALOCELE
-

Defect in the dura and cranium with associated extracranial herniation felt to be related to abnormal closure of the neural tube

-
TWO TYPES:
a)Meningocele \u2013 herniation of meninges
with CSF
b)Encephalocele \u2013 herniation of brain and
meninges
-
Usually midline
-
Occipital location = US
-
Frontal ethmoidal = Asia
-
Often the herniated brain is dysgenic and
nonfunctional
CHIARI I
-
Low tonsils
-
Small fourth ventricle
-
Hydromyelia
-
Klippel-Feil
-
Occipitilization of the
atlas
-
syrinx
CHIARI II
-
Myelomeningocele
-
Low tentorium
-
Medully kink
-
Hydrocepahalus
-
Beaked tectum
-
Corpus
callosum
agenesis
-
Polymicrogyria
-
Syringomyelia
-
Lacunar skull
CHIARI III
-
Cervical
occipital
encephalocele
that
contains cerebellum

MARY YVETTE ALLAIN TINA RALPH SHERYL BART HEINRICH PIPOY KC JAM CECILLE DENESSE VINCE HOOPS CES XTIAN LAINEY RIZ KIX EZRA GOLDIE BUFF MONA AM MAAN ADI KC
PENG KARLA ALPHE AARON KYTH ANNE EISA KRING CANDY ISAY MARCO JOSHUA FARS RAIN JASSIE MIKA SHAR ERIKA MACKY VIKI JOAN PREI KATE BAM AMS HANNAH MEMAY PAU
RACHE ESTHER JOEL GLENN TONI

Subject:
Topic:
Page 2 of 8
SPINAL DYSRAPHISM
STAGE 2: VENTRAL INDUCTION
- Formation of the Brain segments and
Face
\u2022
5-10 weeks
\u2022

Three vesicles (prsencephalon, mesencephalon, & rhombencephalon) form the cerebrum, midbrain, cerebellum, and lower brainstem

\u2022
Division into two hemispheres
HOLOPROSENCEPHALIES
-
Failure to separate into hemispheres
-
TYPES:
a) ALOBAR
\ue000Complete
failure
\ue000No falx
\ue000Single mono-
ventricle
\ue000Fused
thalami
b) SEMI LOBAR
\ue000Partial separation
of the posterior
occipital
and
temporal lobes
\ue000Frontal brain is
fused
\ue000Thalami partially
fused
\ue000Ace of spades
configuration of
the ventricles
c) LOBAR
\ue000Fusion of only
anterior

inferior frontal lobes so no faux in

that
location
\ue000Otherwise the

brain appears to be quite normal except for

lack

of
septum
pellucidum

d) SEPTAL OPTIC DYSPLASIA
\ue000Most mild form in w/c ther is no septum
pellucidum and the optic nerves are
very atrophic.
\ue000Schizencephaly may be present in 50%
of these cases
\ue000Corpus Callosum agenesis may also be
seen in this entity
\u2022
The optic tracts are thinned bilaterally

The absent septum pellucidum on this coronal T2 image results in a boxlike configuration of the anterior horn of the lateral ventricles. Also note the thin optic tracts.

Coronal
T1
image
demonstrates
corpus
callosum
Subject:
Topic:
Page 3 of 8
CORPUS CALLOSUM AGENESIS
\u2022
Growth anterior to posterior starting at the genu.
Myelination from posterior to anterior
\u2022
Association with Chiari II, Dandy Walker,
Holoprosencephaly and lipomas.
\u2022

Splaying of the anterior horns (Bulls horn appearance) due to realignment of the Probst bundles.

\u2022
High third ventricle
DANY WALKER
\u2022
Defective development of the roof of the fourth
ventricle.
\u2022
Posterior fossa cyst; hydrocephalus often.
\u2022
Large posterior fossa; high torcula; absent falx in
the posterior fossa
\u2022
Partial Dandy Walker lacks high torcula.
Represents a form of cerebellar hypoplasia
\u2022
Mildest aspect of this spectrum would be mega
cisterna magna.
\u2022
DDX: arachnoid cyst, mega cisterna magna
Dandy Walker Variant
\u2022
Open communication of the posteroinferior 4th
ventricle and posterior CSF density cyst.
\u2022
Large CSF density cyst occupies much of the
posterior fossa which is not enlarged
\u2022

Hypoplastic cerebellar hemispheres are \u201cwinged\u201d anterolaterally by the cyst which stops at the lateral angle of the cerebellar hemispheres and does not extend anterior to them (demonstrate a hypoplastic vermis which is anteriorly and superiorly located).

FACIAL ANOMALIES
STAGE
3:
MIGRATION
AND
HISTOGENESIS
\u2022
Neuronal migration from germinal matrix to the
cortex
\u2022
Cortical organization
\u2022
Months: 2-5
\u2022
Disorders:
o
Heterotopias,

agyria-pachygyria, polymicrogyria, vascular malformations, teratomas, phakomatosis

GRAY MATTER HETEROTOPIAS
\u2022
Interruption of normal migration of neural blast
from the general matrix to the cortex
\u2022

Various degrees range from simple nodular gray matter heterotopias to band heterotopias to schizencephaly

to
lissencephaly
and
to
polymicrogyria.
\u2022
Seizures, mental retardation
\u2022

Association with corpus callosum agenesis, Chiari malformations, Tuberous Sclerosis, septo- optic dysplasia.

SCHIZENCEPHALY
\u2022
Gray matter extension from the ventricle to the
cortex.
\u2022

Two types: closed lip (mild, no CSF within) and open lip (contains CSF, severe with cortical defects and large ventricles).

\u2022
Association with septo-optic dysplasia and optic
atrophy.
LISSENCEPHALY (AGYRIA-PACHYGYRIA)
\u2022

Most severe form of neuronal migrational anomalies. Patients often have small brains, mental retardation, spasticity, seizures.

\u2022

Agyria (complete lissencephaly) presents with smooth brain and is identified by figure eight configuration with clefts extending to the sylvian fissure.

\u2022
Pachygyria (incomplete lissencephaly) has
broad, shallow gyre. More mild form the agyria.
Type I Lissencephaly
\u2022
Lack of gyri and alternating layers of grey and
white matter.
\u2022
With a thin outer cortical layer and
\u2022
A thicker deep layer of neurons
of 00

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