259. Longterm Outcome of Treatment for Juvenile Patients with Moyamoya Disease Based on Hemodynamic Measurement.

Authors: Tadashi Nariai, MD, PhD, IFAANS; Yoji Tanaka, MD, PhD; Shoko Murayama-Hara, MD, PhD; Yosuke Ishii, MD, PhD; Taketoshi Maehara, MD, PhD (Tokyo, Japan)


We are applying perfusion imaging with MRI for all juvenile patients with moyamoya disease to determine the surgical indication and procedure. We analyzed if this protocol is appropriate to obtain good clinical outcome.


From 2001 to present, 205 juvenile patients (0-18 y/o) were treated in our institute. All of them underwent at least one of perfusion MRI techniques, DSC and/or ASL to determine surgical indication and the area to be operated. After the treatment, all were followed up with periodical MR hemodynamic study. 


1) Of all, 150 were treated after the initial diagnosis and were followed up for more than 3 years. Present status of 135 (90%) were confirmed. (mean follow up 9.8 years). 2) Using MR data, 115 (85.2%) were determined to be operated and 20 (14.8%) were conservatively followed up. 3) All surgically treated patients received indirect bypass surgery. 53 (46%) received bilateral surgery, and 62 (54 %) received unilateral surgery as initial operation. 4) 33 of 62 (53.2%) who received unilateral surgery later received surgery in the other side. 11 of 86 ( 12.7 %) who completed bilateral surgery received additional surgery to the posterior part of brain. 5) 90.4 % of surgically treated and 100 % of conservatively treated patients are now in good conditions (mRS = 0-2) , and 9.6 % of surgically treated patients need social assistance due to mental retardation (mRS=3). None of patients have severe physical disability. 6) None experienced a subsequent infarction. In one, intra-ventricular hemorrhage occurred 16 years after the initial surgery, but recovered well (mRS=2).


Hemodynamic measurement have beed usefully applied for the treatment of moyamoya disease of juvenile onset by selecting appropriate surgical candidate and optimal surgical method, and by obtaining improved clinical outcome than previously published (Mukawa et al. JNSP 2012).