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Type:
Educational Exhibit
Keywords:
CT-Angiography, Catheter arteriography, Vascular, Neuroradiology brain, Diagnostic procedure, Embolism / Thrombosis, Ischaemia / Infarction
Authors:
E. Montaño Claure1, L. Valoyes Guerrero2, D. Rodriguez3, L. Aja Rodriguez1, A. Muntané Sánchez4, P. Mora Montoya1; 1Barcelona/ES, 2L'Hospitalet de Llobregat/ES, 3El Vendrell/ES, 4Hospitalet de Llobregat /ES
DOI:
10.1594/ecr2014/C-1646
Findings and procedure details
The main features of moyamoya disease are bilateral stenosis of the internal carotid
bifurcations and basal telangiectasias comprising dilated collateral lenticulostriate and thalamoperforating arteries.
Initial symptoms are due to either cerebral ischemia or intracranial hemorrhage.
The hemorrhage may be subarachnoid,
intraparenchymal,
or intraventricular and is sometimes associated with a saccular aneurysm (usually in the vertebrobasilar system).
In children the initial symptoms are usually due to ischemia whereas adults often have hemorrhagic manifestations.
Collateral circulation forms from a number of sources:
- via the abnormal moyamoya vessels
- pial collaterals from less affected vessels (especially PCA) : forming the so called ivy sign on contrast enhanced MRI
- transdural branches of the middle meningeal and other dural branches
Suzuki and Kodama classified the evolution of the disease using angiographic findings and defining six phases.
- Stage I
-
- "narrowing of the carotid fork"
- narrowed ICA bifurcation
- Stage II
-
- "initiation of the moyamoya"
- dilated ACA,
MCA and narrowed ICA bifurcation with moyamoya change
- Stage III
-
- "intensification of the moyamoya"
- further increase in moyamoya change of the ICA birfurcation and narrowed ACA and MCA
- Stage IV
-
- "minimization of the moyamoya"
- moyamoya change reducing with occlusive changes in ICA and tenuous ACA and MCA
- Stage V
-
- "reduction of the moyamoya"
- further decrease in moyamoya change with occlusion of ICA,
ACA and MCA
- Stage VI
-
- "disappearance of the moyamoya"
- ICA essentially disappeared with supply of brain from ECA
- the description in inverted commas is that of Suzuki in the original paper.
DSA is still considered necessary for a definite diagnosis although it has a risk of complications.
CTA allows visualization of intracranial vessels could be useful in the diagnosis,
classification and follow up of the disease.