Moyamoya disease with EDAMS

Case contributed by Dr Vinay V Belaval

Presentation

Known case of Moyamoya disease. Underwent EDAMS (encephaloduroarteriomyosynangiosis) procedure one year back.

Patient Data

Age: 4 years
Gender: Male
MRI

Large areas of gliosis are seen in bilateral cerebral hemispheres, predominantly in bilateral temporo-occipital lobes - representing extensive ischemic changes.  There is ex-vacuo dilatation of bilateral lateral and 3rd ventricles.

Post operative changes are seen in the form of bilateral temporalis muscle flaps along with bilateral superficial temporal artery branches on bilateral temporal lobes - consistent with encephaloduroarteriomyosynagniosis. There is collateralization between few bilateral middle cerebral artery branches and bilateral superficial temporal artery branches.

Thin bilateral fronto-parietal extradural collections are noted.

Intracranial bilateral internal carotid arteries are diffusely small in caliber measuring 1.8mm on right and 2.0mm on left. There is severe abrupt narrowing of bilateral supraclinoid internal carotid arteries.

There is non visualization of bilateral middle cerebral arteries.  

A1 segment of left anterior cerebral artery is not visualized. Rest of the left anterior cerebral artery is small in caliber.   

Right anterior cerebral artery is not visualized.

Multiple small leptomeningeal collaterals are seen in basal cisterns.

Bilateral intracranial vertebral arteries are normal in course and caliber.

Basilar artery and both posterior cerebral arteries appear normal.  

Annotated image

Annotated coronal MIP image of the MR angiogram shows normal appearence of posterior circulation, hypoplastic bilateral internal carotid arteries and middle cerebral arteries. Bilateral superficial temporal artery branches are noted adjacent to bilateral temporal poles. 

Case Discussion

Moyamoya disease is a progressive vasculopathy affecting bilateral distal internal carotid arteries and circle of Willis. Children present with acute or chronic ischemic changes. 

Multiple surgical revascularization techniques are present, comprising of direct and indirect methods of extracranial- to intracranial artery anastamosis. 

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Case information

rID: 72783
Published: 17th Dec 2019
Last edited: 17th Dec 2019
Inclusion in quiz mode: Included
Institution: Belcity Scan and Diagnostic Centre

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