IMPORTANT: We currently have a number of bugs related to image cropping and are actively trying to resolve them. In the meantime, we have disabled cropping. Apologies for any inconvenience. Stay informed: radiopaedia.org/chat

Moyamoya syndrome with background sickle cell disease

Case contributed by Kauv Paul
Diagnosis almost certain

Presentation

Follow up on patient with sickle cell disease.

Patient Data

Age: 30 years

There is no flow in cavernous segment of the right ICA with reconstitution of the flow in supraclinoid segment and distal branches. There is also narrowing of the supraclinoid segment of the left ICA and MCAs bilaterally with reduced number and caliber of the cortical branches of MCAs. Vertebrobasilar system is relatively spared.

There is a network of multiple small collateral branches at the base of the brain, ambient cisterns and intra-axial bilateral basal ganglia region in keeping with moyamoya collaterals. Characteristic "puff of smoke" appearance in MIP of TOF.

Absence of recent ischemic lesion. Leukoaraiosis in cortical watershed territories.

Case Discussion

Moyamoya syndrome in a patient with sickle cell disease and with evidence of chronic ischemia in cortical watershed zones in deep corona radiata regions.

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.