Moyamoya Disease in a Patient With Sickle Cell Disease: A Case Report and Review of the Literature

Abstract

Moyamoya disease (MMD) is a relatively rare, progressively worsening steno-occlusive condition primarily characterized by a progressive narrowing of the intracranial arteries, causing hypoperfusion and consequent cerebral ischemia and infarction. This case report discusses the rare presentation of a patient who was known to have sickle cell disease and MMD. Various investigations have revealed a typical presentation of such a disease through radiological findings. Our report highlights this rare disease and its possible association with other comorbidities, as well as the medical treatment options that patients may undergo with the option of surgical treatment.

Keywords: cerebrovascular disorder; epilepsy; genetic disease; moyamoya disease; sickle cell disease.

Figure 1. Multiple bilateral cerebral acute / subacute ischemic infarctions and right internal carotid artery occlusion (black arrow).

Figure 2. Examination of the intracranial vascular channels using 3D Time Of Flight (TOF) magnetic resonance angiography (MRA)

TOF MRA shows no evidence of flow signal in the intracranial portion of the right internal carotid artery likely representing right internal carotid artery occlusion and refilling of the right middle cerebral artery and anterior cerebral artery from the contralateral side is noted. Both also show mildly attenuated caliber. "puff off smoke" phenomenon which is typical for MMD (black square).