Vein of Galen Malformations

FACTS

  • typically presents before 5 years age
  • pathophysiology: can be thought of as AVF draining to median prosencephalic vein (MPV) (embryonic precursor to VOG)
Definitions
  • Vein of Galen aneurysmal malformation (VGAM): AVFs draining to MPV
    • Mural:
      • located in either velum interpositum cistern or quadrigeminal cistern
      • single or multiple fistulas at wall of dilated posterior portion of MPV
      • fewer fistulas than choroidal and typically more round dilation of MPV
      • later in infancy usually
    • Choroidal: multiple fistulas to embryonic median prosencephalic vein mainly located in velum interpositum cistern medial to choroidal fissure
      • arterial feeders: choroidal arteries, pericallosal arcade (ACA) +/- quadrigeminal arteries, thalamoperforators
  • Vein of Galen aneurysmal dilatation (VGAD): group of malformations associated with dilation of VOG caused by pial / dural AV shunts draining into True VOG (matured)
    • relevant difference is the midline ectatic vein is the true VOG and receives drainage from normal brain and malformation too
  • Vein of Galen varix: dilated vein of Galen with no AV shunts at all
 
Good article: https://www.medlink.com/articles/vein-of-galen-malformations
 

HPI

Close to birth: congestive high flow heart failure 2/2 AV shunting
Infancy/Childhood: progressive hydro and developmental delay, FTT, macrocephaly

PHYSICAL EXAM

IMAGING

 
A/P
  • Elective DSA when a patient is old / heavy enough
  • definitive treatment is embolization
 

Vein of Galen Malformation (Mural Subtype) with Shunting Youman’s 247.7

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Vein of Galen Dilatation (Youman’s 247.9)

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No shunting seen here, simply aneurysmal dilatation