האנטומיה של מערכת העצבים

Transcription

האנטומיה של מערכת העצבים
Neurovascular cases
Vein of Galen Aneurysmal
Malformation (VGAM)
Dr.. Guy Raphaeli
Dr
Neurology department
Interventional Neuroradiology unit
Rabin Medical Center,
Beilinson hospital, Petach Tikva
Illustrative case
A 15 YO male patient, history unremarkable, chronic
headaches, first-ever generalized seizure
CT (-)
T2
FLAIR
Left VA
ONYX Embolization of feeders
Control DSA 21 days
Control DSA 21 days
 The vein of Galen is located under the cerebral
hemispheres and drains the anterior and central regions of
the brain into the sinuses of the posterior cerebral fossa.
 Called also as The great cerebral vein is one of the large
blood vessels in the skull draining the cerebrum
 The veins of the deep structures of the brain normally drain
through the Galenic system
 Named for its discoverer, the Greek physician Galen.
 Aelius Galenus or Claudius Galenus (b. 129 AD, d. circa
200 AD), better known as Galen of Pergamon was a
prominent Roman (of Greek ethnicity) physician, surgeon
and philosopher.
 Galen
contributed
numerous
scientific
greatly
to
the
disciplines,
understanding
including
of
anatomy,
physiology, pathology, pharmacology, and neurology, as
well as philosophy and logic.
 Direct communication between a cerebral artery and a
cerebral
vein
results
from
a
congenital
vascular
malformation. The vein of Galen abnormality is the most
frequent arteriovenous malformation in neonates.
 It commonly presents in the neonatal period, although it
may present later, in early childhood.
 Typically, in the neonatal period, VGAM presents with
congestive heart failure, a cranial bruit, and marked
carotid pulses.
 Vein of Galen aneurysmal malformations (VGAM) are the
most common form of symptomatic cerebrovascular
malformation in neonates and infants
 The
VGAM
is
a
choroidal
type
of
arteriovenous
malformation involving the vein of Galen and is distinct
from an arteriovenous malformation with venous drainage
into a dilated, but already formed, vein of Galen.
 Aneurysmal malformations of the vein of Galen typically
result in high-output congestive heart failure or may
present with developmental delay, hydrocephalus, and
seizures
 High-output heart failure in the newborn resulting from the
decreased resistance and high blood flow in the lesion.
 Associated findings include cerebral ischemic changes
such as strokes or steal phenomena that result in
progressive hemiparesis.
 Hemorrhage from the malformation can occur, although
this is not a common finding. Finally, the malformation may
result in mass effects, causing progressive neurological
impairment. Alternatively, the malformation may cause
obstruction
of
hydrocephalus.
the
CSF
outflow
and
result
in
Epidemiology
 The incidence of the vein of Galen malformation is
unknown.
Mortality/Morbidity
 Infants often die if the high-output congestive heart failure
is the presenting feature.
 Macrocephaly usually improves following shunting for
hydrocephalus.
 A recent study of 21 neonates demonstrated that poor
outcomes included neurological manifestations at birth,
focal cerebral parenchymal changes, calcification of
cerebral tissue, tonsillar herniation, and two or more
arterial feeders.
 In infants with these features, consideration could be given
to withholding aggressive intervention
Geibprasert S et al. Childs Nerv Syst. Jan 2010
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