Prognostic Factors of Moyamoya Disease Evaluated by Activity of Daily Living

Park, Jun Bum; Ra, Young Shin; Ahn, Jae Sung; Kwun, Byung Duk; Lee, Jung Kyo

Abstract

BACKGROUNDS: Moyamoya disease is a progressive occlusive cerebrovascular disease which has characteristics of distal ICA stenosis and basal collateral vessels. Various methods of surgical treatment are recommended in the literatures but surgical strategies and outcome are not well established yet. AIMS AND METHODS: The aims of study is to evaluate surgical outcomes of moyamoya disease and to establish surgical indications. Total 155 patients diagnosed with moyamoya disease since 1990, were analyzed retrospectively. Female was more predominant by 1.5 : 1. There were two peaks of age of onset at the 1st decade (39.0%) and 4th (15.9%). Familial occurrence was 6.8% (n=17). The mean duration of follow-up was 41.1 months (12-156 months). Moyamoya syndrome was excluded in this study. Surgical outcomes were measured by grading activity of daily living (ADL) and prognostic factors were analyzed statistically with SAS. RESULT: The most common clinical presentations were transient ischemic attacks (69.1%), followed by cerebral infarction (26.0%) and hemorrhage (4.9%) in pediatric patients, but in adult hemorrhage (49.2%) was the most prevalent. Recurrence of symptoms developed in 8 patients (11.4%) among 70 of cerebral infarction with mean intervals of 30.8 months and 7 patients (21.9%) among 32 of hemorrhage with mean interval of 42.3 months. Forty five (29.0%) of 155 patients showed stenosis of posterior cerebral arteries on cerebral angiography at the age of diagnosis. The surgical treatment were performed at 183 hemispheres of 115 patients, direct bypass surgery was done in 10 hemispheres, indirect bypass surgery in 169 hemispheres (106 EDAMS, 14 EDAS, 40 frontal EDS or burr hole, 6 EDAMS and EDAS, 3 others), combined direct and indirect bypass surgery in 4 hemispheres. The improvement of ADL was not so different between 68 (59.1%) of 115 patients treated with surgery and 23 (57.5%) in 40 patients with conservative care. The initial and final grade of ADL of pediatric patients were better than those of adult (p<0.01). The grades of ADL of patients with transient ischemic attack were better than those with infarction or hemorrhage (p<0.01). Those patients with recurrent hemorrhage were poorer in the outcome than recurrent infarction (p<0.05). The incidence of recurrent infarction in the surgical cares was lower than that in conservative care (5.9% vs 26.3%, p<0.05), but there was no statistical difference between two groups with regard to rebleeding (8.3% vs 30.0%, p>0.1). Significant prognostic factors affecting outcomes of moyamoya disease age at onset, clinical features, and initial grade of ADL.
CONCLUSION
Children with transient ischemic attack were the best, but adults with recurrent hemorrhage were the worst in outcomes. Surgical treatment for moyamoya disease should be carefully tailored according to age of onset and clinical features regardless of surgical methods. Further prospective study is indicated to determine optimal treatment guideline for moyamoya disease.