Clinical features and long-term outcomes of moyamoya disease: a single-center experience with 528 cases in China

Abstract

OBJECT The aim of this study was to describe the baseline clinical features and long-term outcomes of patients with moyamoya disease (MMD) based on a 25-year period at a single center in China. METHODS Data obtained in 528 consecutive patients with MMD treated at the authors' hospital from 1984 to 2010 were reviewed retrospectively. Events of transient ischemic attack, new infarction, and hemorrhage were included. The Kaplan-Meier risk of stroke was calculated. RESULTS The mean (± SD) patient age was 26 ± 13 years (range 2–67 years), and the female/male ratio was 0.9:1. There were 332 cases of ischemia and 196 hemorrhages. Adults had a higher rate of bleeding than children (50.7% vs 14.0%, respectively; p < 0.001). One hundred twenty-two patients were treated conservatively, and 406 patients underwent revascularization procedures. Of 528 patients, 331 (62.7%) had at least 1 year of follow-up (median 39.5 months) and data from these patients were analyzed. Rebleeding and mortality rates in patients with hemorrhagic MMD (n = 104) were higher than in those with ischemic MMD (n = 227) (26.9% vs 2.2% [p < 0.001] and 4.8% vs 0.4% [p < 0.05], respectively). Twenty-five of 60 (41.7%) conservatively treated patients and 8 of 271 (2.9%) surgically treated patients experienced rebleeding events, a difference that was significant in the Kaplan-Meier curve of rebleeding (p < 0.01). An improvement in perfusion was found in 164 of 224 (73.2%) surgically treated patients 1 month after discharge. However, there was no significant difference in the rate of ischemic events in the surgical and conservative groups (18.8% and 28.3%, respectively; p = 0.09). Among the 104 hemorrhagic cases, rebleeding attacks were observed in 25 patients in the nonsurgical group (n = 60) and 3 patients in the surgical group (n = 44) (41.7% and 6.8%, respectively; OR 9.7 [95% CI 2.7–35.0]; p < 0.01). CONCLUSIONS There was no difference in the sex distribution of Chinese patients with MMD. Patients with hemorrhagic MMD had a much higher rate of rebleeding and poorer prognosis than those with the ischemic type. Surgical revascularization procedures can improve cerebral perfusion and have a positive impact in preventing rebleeding in patients with hemorrhagic MMD.

Genotype-Phenotype Correlation in Long-Term Cohort of Japanese Patients with Moyamoya Disease

“…1 ticular, recurrent hemorrhage was observed in 5 patients. In several past large studies, the incidence of recurrent stroke after revascularization was reported to be approximately 5-20% (annual risk: 0.4-6.7%) [11][12][13][14][15][16]. It is difficult to directly compare these incidences because the included patients' characteristics, follow-up rate, or management made a difference to the incidence of recurrent stroke in our study.…”

The Efficacy of Surgical Treatment for the Secondary Prevention of Stroke in Symptomatic Moyamoya Disease

“…Then, 8 records were excluded for no data. Finally, 16 articles were included, 7,8,11–24 among which 8 articles compared the efficacy of conservative versus surgical treatment in 961 patients, 12 studies compared the recurrent stroke rate of indirect and direct bypass surgery in 837 patients and 11 papers (1 paper without complication data was excluded) focused on surgical complications after indirect and direct bypass surgery in 1071 patients. Additionally, the mean NOS score was 6.9 indicating that the methodological quality of studies was good (Table 1).…”

“…Some previous studies have suggested that surgery is superior to drugs alone. 7,8 In contrast, Sundaram et al 9 reported that conservative treatment results in a lower stroke recurrence rate and good functional outcomes.…”

Peek through the smoke: a report of moyamoya disease in a 32-year-old female patient presenting with ischaemic stroke

“…The evidence favours surgical treatment in ischaemic moyamoya and to a lesser extent haemorrhagic moyamoya in which there is an argument that revascularisation may increase the risk of recurrent haemorrhage 42. Available data, however, suggest that revascularisation reduces the risk for recurrent haemorrhage 43. Studies show no significant difference between indirect and direct plus combined techniques 30.…”