S-Space College of Medicine/School of Medicine (의과대학/대학원) Internal Medicine (내과학전공) Journal Papers (저널논문_내과학전공)
Pediatric Moyamoya Disease: An Analysis of 410 Consecutive Cases
- Kim, Seung-Ki; Cho, Byung-Kyu; Phi, Ji Hoon; Lee, Ji Yeoun; Kim, Ki Joong; Kim, In-One; Lee, Joongyub; Wang, Kyu-Chang; Lee, Dong Soo; Hwang, Yong-Seung; Chae, Jong Hee
- Issue Date
- ANNALS OF NEUROLOGY; Vol.68 1; 92-101
- Objective: Moyamoya disease (MMD) is a cerebrovascular occlusive disease of the bilateral internal carotid arteries that causes a compensatory abnormal vascular network at the base of brain. The rare incidence and various surgical techniques applied have limited the clinical research on MMD. Methods: We conducted a retrospective analysis of the surgical outcome of 410 pediatric MMD patients. All patients were treated in a relatively uniform scheme at a single institution. The surgical procedures consisted of bilateral encephaloduroarteriosynangiosis augmented by bifrontal encephalogaleo-/periosteal synangiosis. Logistic regression analyses were applied to reveal the prognostic factors for surgical outcome. Results: The overall clinical outcome was excellent in 66%, good in 15%, fair in 15%, and poor in 4% of the patients. Therefore, 81% of the patients had a favorable clinical outcome (excellent and good). Multivariate analyses revealed that infarction on presentation was associated with unfavorable clinical outcome (odds ratio [OR], 2.85; 95% confidence interval [CI], 1.49-5.46; p < 0.01) and decreased vascular reserve only on single-photon emission computerized tomography (OR, 0.07; 95% CI, 0.01-0.52; p < 0.01), with favorable clinical outcome. Interpretation: Our results indicate that an early diagnosis and active intervention before establishment of irreversible hemodynamic change are essential to achieve a favorable clinical outcome in children with MMD. ANN NEUROL 2010;68:92-101
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