Development of a de novo cerebral arteriovenous malformation in a child with sickle cell disease and moyamoya arteriopathy

The authors report the unique case of a 6-year-old African-American girl with sickle cell disease (SCD) and an associated moyamoya arteriopathy who developed a de novo arteriovenous malformation (AVM) of the cerebral circulation. Based on preoperative cerebral angiography, computerized tomography angiography, and magnetic resonance imaging, the incidentally discovered lesion was originally thought to be a direct arteriovenous fistula with an associated varix. At surgery, however, a 1.5-cm AVM was identified adjacent to the deep surface of the varix, and it was successfully resected. The diagnosis of cerebral AVM was then confirmed histopathologically. Based on a review of the literature, no published correlation between cerebral AVMs and SCD exists. In addition to reporting this case, the authors provide a description of AVM pathogenesis, with particular emphasis on acquired AVMs of the cerebral circulation.