Long-term follow-up of surgically treated juvenile patients with moyamoya disease

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>Surgical revascularization is considered an effective treatment for juvenile patients with moyamoya disease (MMD). Yet the long-term outcome in surgically treated patients still needs to be clarified. More than 30 years have passed since the authors' department started intensively treating pediatric patients with MMD using indirect anastomosis techniques. In this study the authors surveyed the current status of these patients.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>Activities of daily living (ADLs) were surveyed and present clinical status was assessed based on the modified Rankin Scale (mRS). Cerebrovascular events subsequent to surgical treatment were also recorded.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>Since 1979, 208 patients younger than 19 years of age with MMD were surgically treated and followed up for &gt; 3 years. Data were available on 172 patients (83%), who had been followed up for a mean of 14.3 years (range 3–32 years). Activity of daily living outcomes were as follows: 138 patients (80.2%) had mRS scores of 0–2, 29 (16.9%) a score of 3, 1 (0.6%) a score of 4, 1 (0.6%) a score of 5, and 3 (1.7%) a score of 6. Cerebrovascular events occurred 8 or more years after surgery in 6 patients (3.4%), that is, 6 hemorrhages and 3 infarctions. The cumulative risk of late-onset stroke at 10, 20, and 30 years after surgical intervention was 0.8%, 6.3%, and 10.0%, respectively.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>This long-term survey demonstrated that most surgically treated pediatric patients with MMD maintain good ADL outcomes. However, a significant number of new cerebrovascular events occurred more than 10 years after the initial surgery. Additional follow-up will help to identify which events may occur during the adult years of patients treated as children.</jats:p></jats:sec>

Clinical Features, Surgical Treatment, and Long-Term Outcome in Pediatric Patients with Moyamoya Disease in China

“…In children, direct revascularization is frequently technically not feasible, whereas the response to indirect revascularization is excellent, although 1 or 2 weeks are required for stabilization of symptoms. Although objective evidence from randomized controlled clinical trials suggesting improved function following these procedures is still lacking, several studies examining the long-term outcome in children with MMD following revascularization surgery strongly suggest that surgical revascularization improves cerebral hemodynamics and reduces the incidence of subsequent ischemic events [21,22,23,25,26,27,28]. Similar to previous studies, TIAs rapidly decrease or disappear and strokes rarely recur after surgery in pediatric patients of our cohort.…”

“…Despite the varied clinical associations observed in this series, the modes of presentation, age at onset of symptoms, familial occurrence were similar to those reported in the Japanese and South Korean literature [17,19,21,22,23]. However, the ratio of female to male patients was 1:1 by chance in our Chinese cohort.…”

Impact of posterior cerebral artery involvement on long-term clinical and social outcome of pediatric moyamoya disease

“…Our results indicating that more than 80% of patients had a favorable social outcome appear comparable to those of past studies, in which the frequency of patients with a normal intelligence quotient or capable of independent daily activities remained 63.5%-87%. 13,17,25,31 Nevertheless, our results, along with those of several pioneering studies, shed light on the salient issue of social adaptation in pediatric moyamoya disease. Nakashima et al reported that approximately 10% of patients had severe difficulty in social or school life because of intellectual impairment.…”

Treatment of moyamoya disease by multipoint skull drilling for indirect revascularization combined with mobilization of autologous bone marrow stem cells

“…These repair the damaged brain tissue via multiple mechanisms. The GM-CSF treatment can promote growth of collateral arterial branches in the pia mater and reduce the area of cerebral infarction due to arterial occlusion in the brain, thus protecting the nerves Mukawa et al, 2012). Previous studies on cerebral ischemia have shown that multipoint skull drilling combined with mobilization of autologous BMSCs have a synergistic effect.…”