Postoperative neurological deterioration in pediatric moyamoya disease: watershed shift and hyperperfusion

Clinical article

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Young patients with moyamoya disease frequently exhibit extensive cerebral infarction at the time of initial presentation, and even in the early postoperative period. To investigate clinical characteristics in the early postoperative period, the authors prospectively analyzed findings of MR imaging, MR angiography, and SPECT before and after surgery. The authors focused in particular on how postoperative neurological deterioration occurred.


Between August 2005 and June 2009, 22 patients younger than 18 years of age with moyamoya disease were treated at Miyagi Children's Hospital. The mean patient age (± SD) was 8.58 ± 4.55 years (range 2–17 years). Superficial temporal artery–middle cerebral artery bypass and indirect bypass of encephalosynangiosis between the brain surface and the temporal muscle, galea, and dura mater were performed in 35 hemispheres. Magnetic resonance imaging and MR angiography were performed before surgery, at 7 days postoperatively, and 3–6 months after surgery. A 123I-isopropyl iodoamphetamine SPECT scan was also obtained pre- and postoperatively.


During the postoperative period, neurological deterioration was observed after 15 operations (10 cases of motor paresis, 1 of aphasia, and 4 of sensory disturbance) in 13 patients. All symptoms had resolved by the time of discharge, except in 2 patients who suffered cerebral infarction. All patients exhibited disappearance (94.3%) or reduction (5.7%) of transient ischemic attacks (TIAs) during the follow-up period. Perioperative studies revealed 2 different types of radiological findings, focal uptake decrease on SPECT indicative of cerebral ischemia due to dynamic change in cerebral hemodynamics caused by bypass flow, the so-called watershed shift, and perioperative edematous lesions on MR imaging due to cerebral hyperperfusion. The frequent occurrence of preoperative TIAs was significantly associated with watershed shift, whereas preoperative MR imaging findings and preoperative SPECT findings were not. Age at operation was the only factor significantly associated with postoperative hyperperfusion.


In young patients, moyamoya disease exhibits rapid progression, resulting in poor clinical outcome. The risk of postoperative neurological deterioration in very young moyamoya patients with frequent TIAs should be noted. The findings in this study showed that direct bypass is not completely safe in patients with moyamoya disease because it causes dynamic change in postoperative cerebral hemodynamics.

Abbreviations used in this paper: ACA = anterior cerebral artery; ICA = internal carotid artery; IMP = isopropyl iodoamphetamine; MCA = middle cerebral artery; PCA = posterior cerebral artery; STA = superficial temporal artery; TIA = transient ischemic attack.

Article Information

Address correspondence to: Toshiaki Hayashi, M.D., Ph.D., Department of Neurosurgery, Miyagi Children's Hospital, 4-3-17 Ochiai, Aoba-ku, Sendai, 989-3126 Japan. email:

© AANS, except where prohibited by US copyright law.



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    Preoperative (upper) and postoperative (lower) images obtained in a 3-year-old girl. Preoperative MR angiography study showed minimal moyamoya vessels with poor depiction of the MCA in the right hemisphere. Postoperative SPECT study obtained 3 days after surgery revealed improvement of right cortical uptake. On postoperative T2-weighted MR imaging and MR angiography studies obtained 1 week after surgery, the right STA–MCA bypass and the branch of the right MCA were well visualized, and no significant postoperative lesion was seen.

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    Preoperative (upper) and postoperative (lower) images obtained in a 3-year-old girl, demonstrating watershed shift. Preoperative MR imaging revealed cerebral infarction in the right hemisphere. Preoperative MR angiography revealed marked moyamoya vessels, with good depiction of the MCA in the left hemisphere. Postoperative SPECT study obtained 3 days after left direct and indirect bypass was performed revealed improvement of left occipitotemporal uptake, with focal hyperperfusion and marked decrease in uptake in the left frontal region. On postoperative T2-weighted MR imaging obtained 1 week after surgery, an ischemic change is noted in the left frontal subcortical area. Postoperative MR angiography revealed increased signal intensity in the lower branch of the left MCA (black arrowheads) and decreased signal intensity in the upper branch of the left MCA (white arrowheads).

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    Preoperative and postoperative findings in a 5-year-old girl. Preoperative MR angiography revealed marked moyamoya vessels with poor depiction of the MCA in the left hemisphere (e). Preoperative SPECT study revealed a mild decrease in uptake bilaterally in the frontal area (a). Postoperative SPECT performed 3 days after left direct and indirect bypass revealed a decrease in uptake in the left frontal region (b). Follow-up SPECT study performed 3 months after surgery revealed marked improvement of left cortical uptake (c). Follow-up MR imaging (f) performed 3 months after surgery revealed no significant lesion other than the preoperative one (d). Signal intensity of the left STA–MCA bypass (white arrowhead) and depiction of the left MCA were more prominent on follow-up MR angiography (g) than on preoperative MR angiography (e). Decreased depiction of moyamoya vessels was also noted.

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    Preoperative (a), perioperative (b), and follow-up (c) images obtained in a 17-year-old girl who had previously undergone bypass surgery in the right hemisphere and who this time underwent left direct and indirect bypass. Preoperative T2-weighted MR imaging revealed no significant cerebral lesion (a). Preoperative MR angiography revealed minimal moyamoya vessels, with poor depiction of the MCA in the left hemisphere. Postoperative T2-weighted MR imaging performed 7 days after surgery revealed a hyperintense area in the left parietal region (arrowhead; b). An MR angiography study disclosed an apparently patent STA–MCA bypass (arrow) with increased intensity in the left MCA. A SPECT study performed 3 days after surgery revealed improvement of uptake in the left hemisphere, with an area of focal hyperperfusion in the left parietal region corresponding to the hyperintense area. Follow-up studies performed 3 months after surgery revealed disappearance of the hyperintense area and focal hyperperfusion (c). Signal intensity of the STA–MCA bypass (arrow) was less prominent than in the acute postoperative period.

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    Schematic drawings of postoperative hemodynamic changes. A: Ideal increase in MCA flow with direct bypass. B: Excessive flow from direct bypass causes hyperperfusion. C: The graft, by reversing flow patterns, induces relative hypoperfusion in the remote territory of the MCA.



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