Hemorrhagic Moyamoya Disease in Children: Clinical, Angiographic features, and Long-Term Surgical Outcome

Background and purpose: Here, we describe the clinical, angiographic characteristics, and long-term surgical outcome of hemorrhagic moyamoya disease in children.

Methods: We retrospectively collected 374 consecutive children with moyamoya disease (hemorrhagic 30 and ischemic 344) between 2004 and 2012 in our hospital. The clinical and radiological characteristics of the hemorrhagic patients were retrospectively described and analyzed. All the hemorrhagic patients underwent encephalo-duro-arterio-synangiosis procedure. Digital subtraction angiography was performed to evaluate the efficacy of vascularization. Clinical follow-up outcomes were obtained through clinical visits, telephone, or letter interview.

Results: In our study, the ratio of female to male patients in the hemorrhagic group was significantly higher than the ischemic group (2:1 versus 0.9:1; P<0.05). The most frequent hemorrhagic location was intraventricular hemorrhage (n=22, 73%). In addition, significantly greater dilatation of the anterior choroidal artery and the posterior communicating artery were seen in the hemorrhagic group (P<0.05). Good or fair vascularization were observed in all the 15 children with digital subtraction angiography follow-up. Clinical outcomes showed that 25 of 30 (83%) patients had no disability (modified Rankin scale score, 0 and 1); 1 patient (3.3%) died of recurrent hemorrhagic stroke.

Conclusions: The presence of anterior choroidal artery and posterior communicating artery dilation may be associated with the bleeding episode in the children with hemorrhagic moyamoya disease. The encephalo-duro-arterio-synangiosis surgery can effectively increase the cerebral blood flow in children, which may decrease the incidence of recurrent hemorrhage.