Vein of Galen malformation Flashcards
VOGM or VOG anurysum , what is it and when develop .?
Congenital
Developer befor 3 month embryo stead
Pathology or cause
medial vein of the prosencephalon
The vein of Galen is formed by the confluence of the internal cerebral veins ++ the basal vein of Rosenthal.
🔺 AVM vs VOGM
VOGM retrograte filing of internal cerebral vein in the former.6
VOGM feeding artery
medial and lateral choroidal, circumferential, mesencephalic, anterior choroidal, pericallosal, and meningeal arteries.
Agenesis of the straight sinus may be an associated finding.
Presentation
CHF ❤️ ▶️ high flow
HC ▶️ compresse Sylvian aqueduct by enlarge VOg
Prominent scalp vein⏯️ high venous pressure
Classification depend on the location
- pure internal fistulae: single or multiple
- fistulae between thalamoperforators and the VOG
- mixed form: the most common
- plexiform AVMs
Outcome
Mortality 100% ⏩️ 1-12 m
Poor prognosis 😭
YAŞARGIL classification
Type 3 MCC
Yasargil classification is probably the most descriptive one so far proposed, with application toward open neurosurgery
Type IVA: Pure plexiform nidus in the parenchyma of thalamus Type IVB: Pure plexiform nidus in the parenchyma of mesencephalon 5 Type IVC: Nidus within the parenchyma combined with fistulous cisternal nidus (type I)
LASJEUNAS
Lasjaunias system is more applicable to endovascular approaches.
Choroidal type = Yasargil type I.( more complex and common CHF ♥️ )
Mural = Yasargil type 2 ➡️ Due to the smaller number of fistulas and more outflow obstruction, they are associated with more severe dilation of the median prosencephalic vein and manifest later in infancy as macrocephaly
tX VOGM
Embolization ➡️ Trans-arterial embolization is the primary choice of treatment.
Mural 2 session
Choroidal multiple session
BICERe score VOGM
Lasjaunias (Biceˆtre) score
Embryology ➡️ median prosencephalic vein of Markowski usually regresses at
11th week of gestation, and by 3 months of gestation, the posterior part of it joins the internal cerebral veins and basal veins to form the vein of Galen.
VOG 📍
quadrigeminal cistern.
VOG drain
thalamus, the
medial temporal lobes, the occipital lobes, and the superior cerebellar
vermis.
Classification
Straight Sinus In VOG
Absent most of the time
Melting brain syndrome
- fetuses, neonates, and infants, but it is not observed in adults.
Due to ⬇️ CBF 🩸 and ⬆️ venous HTN ➡️ white matter destroy
Associated with all AV fistula ex pial AVM and dural sinus
bilaterally
Gold stander for diagnosis
DSA
target sign. CT
central thrombus and peripheral circulating blood along the wall of the sac can produce the so-called “target sign.”
Target sign 🪧
‘central thrombus and peripheral circulating blood along the wall of the sac can produce the so-called “target sign.”
keyhole sign US 🔐
Prenatal ultrasonography shows the characteristic midline tubular anaechoic structure superior to the thalamus, which is contiguous with the dilated sagittal sinus (comet tail or keyhole sign).
DDX of VOG
arachnoid cyst, cavum vergi, cavum septum pellucidum, and quadrigeminal cistern
Embolization should be avoided for at least a few days after placement of a ventricular shunt t
avoid the risk for upward cerebellar herniation secondary to a rapid decrease in supratentorial pressure.
Indication for tx
Unstable or progressive cardiac failure despite adequate medical treatment.
5 Chronic venous ischemia of the brain induces subcortical white matter
calcification.
Microsurgery intervention Yasargil stage
Yasargil type I and less complex types II and III.
How much contrast can tolerate
In most neonates, up to 8 mL/kg body weight of contrast material is well tolerated.
Surgery for VOGM indication and approach
Yasargil 1 and mild 2-3
subtemporal, transcallosal, or transtentorial approaches.
The posterior interhemispheric approach is most commonly used to adequately access a vein of Galen malformation and expose the feeding arteries.
of Galen Varix
VGV indicates dilation of the vein of Galen without the presence of an AV shunt and it is usually asymptomatic.
Two type
1➡️ transient asymptomatic dilation of the vein of Galen in neonates with cardiac failure from a cause other than VGAM. T
2 ➡️ as an anatomic variation in which venous drainage of the brain converges toward the deep venous system. It is also asymptomatic, but this arrangement of venous drainage may predispose to future venous thrombosis and resultant ischemic symptoms because of the lack of compliance.
Trans arterial CI
occlusion of the venous dilation in the VGAD is contraindicated because it may produce hemorrhage or venous infarction of the deep cerebral structures as a result of occlusion of the outflow of these veins