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JOURNAL ARTICLE

Pediatric moyamoya disease: An analysis of 410 consecutive cases

Seung-Ki Kim, Byung-Kyu Cho, Ji Hoon Phi, Ji Yeoun Lee, Jong Hee Chae, Ki Joong Kim, Yong-Seung Hwang, In-One Kim, Dong Soo Lee, Joongyub Lee, Kyu-Chang Wang
Annals of Neurology 2010, 68 (1): 92-101
20582955

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.

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