Pediatric moyamoya disease: An analysis of 410 consecutive cases

Authors

  • Seung-Ki Kim MD, PhD,

    1. Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
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  • Byung-Kyu Cho MD, PhD,

    1. Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
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  • Ji Hoon Phi MD,

    1. Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
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  • Ji Yeoun Lee MD,

    1. Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
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  • Jong Hee Chae MD, PhD,

    1. Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
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  • Ki Joong Kim MD, PhD,

    1. Division of Pediatric Neurology, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
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  • Yong-Seung Hwang MD, PhD,

    1. Division of Pediatric Neurology, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
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  • In-One Kim MD, PhD,

    1. Division of Pediatric Radiology, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
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  • Dong Soo Lee MD, PhD,

    1. Department of Nuclear Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
    2. Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
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  • Joongyub Lee MD,

    1. Medical Research Collaborating Center, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
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  • Kyu-Chang Wang MD, PhD

    Corresponding author
    1. Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
    • Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, 101 Daehangno, Jongno-gu, Seoul 110-744, Republic of Korea
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Abstract

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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