Moya Moya

FACTS

  • progressive bilateral occlusion of supraclinoid ICAs → collateral vascular network via hypertrophy of lenticulostriate arteries
  • pathophysiology: intimal thickening with no inflammation
  • can be associated with aneurysms (2/2 aberrant flow) and AVMs (rare)
  • ischemic and hemorrhagic components
  • NOTE: Moyamoya syndrome/phenomenen is when underlying etiology (e.g. atherosclerosis, radiation, SCD produces uni/bilateral steno-occlusive disease with associated moyamoya type collatoerals
  • Genetic associations: Trisomy 21, NF1, collagen vascular disorders
  • Adults: F > M
  • stereotypical presentations
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Asian
White
Black
Children
ischemia
-
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Adults
hemorrhage
ischemia
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HPI

  • bleed history
  • stroke history
  • sickle cell anemia
PHYSICAL EXAM
standard neuro exam
+ attention to stroke symptoms (speech, facial weakness)
IMAGING
CTA: evaluate the entire course of the ICA
general diminutive caliber of L ICA intracranially is demonstrated
general diminutive caliber of L ICA intracranially is demonstrated
abrupt cutoff of L ICA around paraclinoid segment is demonstrated DSA:
abrupt cutoff of L ICA around paraclinoid segment is demonstrated

DSA:
L ICA occlusion on same patient
L ICA occlusion on same patient
 
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ICA
Collaterals
1
narrowing of ICA apex
none
2
ICA stenosis
moyamoya collateral initiation
3
ICA progression of stenosis
moyamoya collateral intensification
4
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ECA intiation
5
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intensification of ECA, reduction of moyamoya
6
total occlusion of ICA
disappearance of moyamoya collaterals
 
notion image
A/P
  • CTH/CTA/CTP
  • MRI Brain w/o
  • DSA
  • Bypass
  • Surgical treatments:
    • STAMCA
    • EDAS (encephaloduraarteriosynangiosis)
    • Multiple skull burr holes
 
answers 1D, 2B, 3C, 4B