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Pediatric moyamoya disease: An analysis of 410 consecutive cases
Author(s) -
Kim SeungKi,
Cho ByungKyu,
Phi Ji Hoon,
Lee Ji Yeoun,
Chae Jong Hee,
Kim Ki Joong,
Hwang YongSeung,
Kim InOne,
Lee Dong Soo,
Lee Joongyub,
Wang KyuChang
Publication year - 2010
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.21981
Subject(s) - moyamoya disease , medicine , confidence interval , odds ratio , logistic regression , surgery , internal carotid artery , radiology
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