Clinical Features, Surgical Treatment, and Long-Term Outcome in Children with Hemorrhagic Moyamoya Disease

Abstract

Background: The objective of this study was to elucidate the clinical features, surgical treatment, and long-term outcome in pediatric patients with moyamoya disease (MMD).

Methods: We retrospectively reviewed 46 pediatric patients with hemorrhagic MMD at Beijing Tiantan Hospital. Clinical features, radiological findings, and outcomes were analyzed.

Results: The mean age at diagnosis was 13.6 ± 3.6 years. The ratio of girls to boys was 1.0:1.1. Familial occurrence was 4.3%. The primary symptoms at initial presentation were intraventricular hemorrhage (IVH) (47.8%), intracerebral hemorrhage (ICH) (37.0%), ICH with IVH (13.0%), and subarachnoid hemorrhage (2.2%). Most patients presented with Suzuki stage 3 or 4 MMD. Posterior cerebral artery involvement was observed in 22 (18.4%) patients. During the average follow-up of 126.1 ± 96.0 months, 11 of 53 (20.8%) conservatively treated hemispheres and 1 of 36 (2.8%) surgically treated hemispheres experienced a stroke event (P < .05). There was a difference in the Kaplan-Meier curve of stroke between the 2 groups (log rank test, P < .05). What is more, the rate of perfusion improvement in surgically treated patients was higher than in those conservatively treated patients 3 months after discharge (P < .05).

Conclusions: Revascularization surgery has a role in the prevention of recurrent strokes and can improve cerebral perfusion in pediatric patients with hemorrhagic MMD. Further study is needed to determine which bypass surgery is more beneficial for pediatric patients with hemorrhagic MMD.

Keywords: Pediatric; conservative treatment; hemorrhage; moyamoya disease; stroke; surgical revascularization.