Prognosis of moyamoya disease
There have been not so many reports on the prognosis of moyamoya disease. When we talk on the prognosis of moyamoya disease, those for pediatric patients and adults patients should be discriminated because of the essential differences between the two.
It is believed that the bypass surgery is beneficial for the patients with ischemic presentation. However, there has been no blinded, randomized study to compare the prognosis of the patients treated with bypass surgery and that of the conservatively treated patients. In this sense, there is no evidence that bypass surgery provides better prognosis than the conservative treatment does. Such a study cannot be performed today in Japan because presumed benefit of bypass surgery in comparison to the non-surgical treatment. The situation for the patients with hemorrhagic presentation is different because it is still in doubt about the benefit of bypass surgery for the patients with hemorrhagic presentation.
The following summaries are about the prognosis of pediatric patients who present with ischemic symptoms.
Kurokawa et al reported on the prognosis of 27 patients aged between 11 months and 4 years and 11 months. Observation periods ranged within 4 years (13 patients), 5-9 years (5 patients), and 10-15 years (9 patients). Eleven patients underwent surgical treatment and their prognosis before surgery was included. Transient ischemic attacks were frequently observed in the first 4 years, but decreased thereafter. Intellectual deterioration and motor impairment increased with time. There was no sexual differences. Outcome included normal in five (19%), occasional transient ischemic attacks or headache alone in nine (33%), mild intellectual and/or motor impairment in seven (26%), requirement for special school or care by parents or institutions after reaching the teen years in three (11%), continuous 24-hour care in two (7%), and death in one (3%). Poor prognosis was correlated with an early age at onset and hypertension.
Fukuyama et al reported on the long-term prognosis of 19 moyamoya children with transient ischemic attacks (6 males and 15 females, mean age 22 years old). Residual clinical symptoms, neurological deficits, activity of daily living, and neurological signs were evaluated in nine patients. Mean follow-up period was 16.5 years. Only three patients (27.3%) under conservative treatment showed good prognosis. Four among six patients with surgical treatment showed clinical improvement. These results suggested that surgical treatment might be appropriate in children with transient ischemic attacks under conservative treatment, who shows residual clinical symptoms.
Outcome after surgical anastomosis
Moyamoya disease and Brain death