A Pediatric Case of Aplastic or Twig-like Middle Cerebral Artery Presenting with Intracerebral Hemorrhage

Abstract

Patients presenting with angiographic demonstration of missing main trunk of middle cerebral artery (MCA) from the vicinity of its origin and multiple plexiform arterial networks along the normal route of M1 segment of MCA have been reported as “Spontaneous middle cerebral artery occlusion with moyamoya phenomenon (MCAO-Mo),” MCA aplasia/dysplasia, or twig-like MCA. It has been suggested that such vascular conditions are distinct from moyamoya disease and degenerative steno-occlusive diseases of MCA such as atherosclerosis. More recently, aplastic or twig-like MCA (Ap/T-MCA) has been proposed as a term that comprehensively defines a diagnosis for such anomalous conditions.
We recently experienced a pediatric case of Ap/T-MCA presenting with intracerebral and intraventricular hemorrhage, which was successfully treated with ventricular drainage and conservative management for Ap/T-MCA. Since this juvenile case had neither angiographic findings that suggested arterial dissection nor a medical history that might cause such a cerebrovascular condition, this case would support the above-mentioned putative congenital etiology of Ap/T-MCA.
We also summarized the data from reported cases of MCAO-Mo and Ap/T-MCA, and evaluated their clinical/radiological characteristics and disease, including eight pediatric cases, to identify characteristics findings for these anomalous conditions that might point to an optimal therapeutic approach.