Pediatric moyamoya disease: An analysis of 410 consecutive cases.
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.


CONCLUSIONS
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.
Kim
Seung-Ki
SK
Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea.
Cho
Byung-Kyu
BK
Phi
Ji Hoon
JH
Lee
Ji Yeoun
JY
Chae
Jong Hee
JH
Kim
Ki Joong
KJ
Hwang
Yong-Seung
YS
Kim
In-One
IO
Lee
Dong Soo
DS
Lee
Joongyub
J
Wang
Kyu-Chang
KC
D000293:Adolescent; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D020520:Brain Infarction / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000601:surgery; D002536:Cerebral Arteries / Q000473:pathology / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D042241:Early Diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009072:Moyamoya Disease / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery*; D019635:Neurosurgical Procedures / Q000379:methods; D011379:Prognosis; D011877:Radionuclide Imaging; D012189:Retrospective Studies; D016896:Treatment Outcome