Pediatric Sickle Cell Disease Neurological

Etiology: Sickle cell disease
Imaging:
DDX:
Complications: 11% of children with sickle cell disease not receiving primary stroke prevention therapy will have a stroke by 20 years old
Treatment:
Clinical: Most common inherited blood disorder in USA

Radiology Cases of Sickle Cell Disease Neurological

MRI and MRA of sickle cell disease resulting in occlusion of the right middle cerebral artery — Axial T1 MRI without contrast of the brain (above left) shows diffuse volume loss in the gray matter and white matter of the right cerebral hemisphere. 3D MRA of the brain viewed from above (above right) shows overall decreased vascularity in the right middle cerebral artery territory when compared to the left middle cerebral artery territory. 3D MRA viewed from the front (below) shows complete occlusion of the M1 segment of the right middle cerebral artery just distal to its origin with collateral perfusion of the distal right middle cerebral artery being provided by the right posterior cerebral artery.

MRA of sickle cell disease causing near total occlusion of the left internal carotid artery — AP MRA of the neck with contrast (above) shows near total occlusion of the left internal carotid artery. AP MRA of the brain with contrast (below) shows the left internal carotid artery to be small in caliber and to have a beaded appearance. The left middle carotid artery is reconstituted throug the anterior communicating artery.