27 July 2011

MoyaMoya Child

Three years old child that suffered from a major infarct at the age of 7 months. MRI of the brain shows large porencephaly of the left hemisphere with compensatory larger right hemisphere corresponding with "age" of the infarct. Note thickness of the bone on second image. But why would a 7 months old child suffer from such extensive infarction? There are some clues when you look at the vessels of the Circle of Willis. Note tapering (progressively narrowing) of the distal Internal Carotid Arteries (ICA). Also you might notice extensive small collateral vessels centrally in the brain (mostly lenticulostriate). This is a case of MoyaMoya disease that is also known as Idiopathic Progressive Arteriopathy of Childhood.

Above images show source and reconstructions of the flow based Time of Flight (ToF) MR Angiography (MRA). You can see tapering of the distal ICA (arrows) as well as multiple small collateral arteries (arrow heads). Those collaterals would show as "puff of smoke" cloud on the conventional angiography. This gives a name - MoyaMoya in Japanese. You might also check my previous case of MoyaMoya in adult.