Childs Nerv Syst (2007) 23:1195–1198
DOI 10.1007/s00381-007-0361-2

CASE REPORT

Symptomatic hyperperfusion after superficial temporal
artery–middle cerebral artery anastomosis in a child
with moyamoya disease
Miki Fujimura & Tomohiro Kaneta & Hiroaki Shimizu &
Teiji Tominaga

Received: 24 April 2006 / Revised: 20 February 2007 / Published online: 8 May 2007
# Springer-Verlag 2007

Abstract
Object Surgical revascularization for moyamoya disease
prevents cerebral ischemic attacks by improving cerebral
blood flow (CBF). It is undetermined, however, how rapid
increase in CBF affects ischemic brain at acute stage,
especially in children.
Case report A 4-year-old girl with moyamoya disease
underwent right superficial temporal artery–middle cerebral
artery (STA–MCA) anastomosis. She suffered temporary
left facial palsy 5 days after surgery. Postoperative Nisopropyl-p-[123I]iodoamphetamine single-photon emission
computed tomography (123I-IMP-SPECT) revealed focal
intense increase in CBF at the sites of anastomosis.
Magnetic resonance imaging/angiography showed the apparently patent STA–MCA anastomosis as a thick high signal
without ischemic changes. Her symptom improved 9 days
after surgery, and single-photon emission computed tomography (SPECT) 2 months later showed normalization of CBF.
Surgical revascularization completely relieved the transient
ischemic attack on her left hand that was seen before surgery.
Conclusion We demonstrated, for the first time, that
delayed focal neurological deficit after STA–MCA anastoM. Fujimura (*) : T. Tominaga
Department of Neurosurgery,
Tohoku University Graduate School of Medicine,
1-1 Seiryo-machi, Aoba-ku,
Sendai 980-8574, Japan
e-mail: fujimur@nsg.med.tohoku.ac.jp
T. Kaneta
Department Radiology, Tohoku University School of Medicine,
Sendai, Japan
H. Shimizu
Department of Neurosurgery, Kohnan Hospital,
Sendai, Japan

mosis can be caused by focal hyperperfusion in childhood
moyamoya disease.
Keywords Moyamoya disease . Superficial temporal
artery–middle cerebral artery anastomosis . Hyperperfusion .
Single-photon emission computed tomography

Introduction
Moyamoya disease is a chronic, occlusive cerebrovascular
disease with unknown etiology characterized by bilateral
steno-occlusive changes at the terminal portion of the
internal carotid artery and an abnormal vascular network
at the base of the brain [19]. Surgical revascularization for
moyamoya disease is believed to prevent cerebral ischemic
attacks by improving cerebral blood flow (CBF), and
superficial temporal artery–middle cerebral artery (STA–
MCA) anastomosis with or without indirect bypass is
generally employed as the standard surgical treatment for
moyamoya disease [6, 8, 16].
Cerebrovascular reconstruction surgery including carotid
endarterectomy or STA–MCA anastomosis in patients with
atherosclerotic cerebral steno-occlusive diseases can cause
a rapid increase in CBF in the chronic ischemic brain,
resulting in complications such as ‘cerebral hyperperfusion
syndrome.’ ‘Cerebral hyperperfusion syndrome’ is characterized by unilateral headache, face and eye pain, seizures,
and focal symptoms that occur secondary to cerebral edema
or intracerebral hemorrhage [14, 17, 18]. Due to the
relatively low flow revascularization obtained by surgery
for moyamoya disease, postoperative hyperperfusion syndrome has been considered to be less common in this entity,
except for limited reports [4, 12], compared to perioperative
transient ischemic attack [5, 7, 15]. However, the exact

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effect of STA–MCA anastomosis on ischemic brain of
moyamoya disease at the acute stage is undetermined due
to the lack of comprehensive CBF study immediately after
revascularization for this entity.
To clarify this critical issue, we prospectively performed
both N-isopropyl-p-[123I] iodoamphetamine single-photon
emission computed tomography (123I-IMP-SPECT) and
magnetic resonance (MR) imaging within 1 week after
surgery during the past 2 years [3]. Among the 78
consecutive surgeries, we had an experience of a 4-year-old
girl manifesting delayed transient facial palsy due to hyperperfusion 5 to 9 days after single STA–MCA anastomosis.
Anatomical location and temporal profile of hyperperfusion
were completely in accordance with her symptom. The
reversibility of both her symptom and the SPECT finding
further convinced the central role of hyperperfusion in her
postoperative neurological deterioration.

Case material
A 4-year-old girl with moyamoya disease presented with
transient ischemic attach on her left hand. MR imaging and
MR angiography demonstrated steno-occlusive changes at the
terminal portions of the bilateral internal carotid arteries and
the presence of abnormal network-like vessels at the bilateral
basal ganglia, which satisfied the diagnostic criteria for
moyamoya disease [19] according to the criteria of the

Fig. 1 123I-IMP-SPECT scans before surgery (a) and 1 (b), 7 (c), and
63 days (d) after right STA–MCA anastomosis. The focal intense
increase in CBF at the site of anastomosis (arrows in c) was evident
7 days after surgery, when the patient suffered left facial palsy. Two

Childs Nerv Syst (2007) 23:1195–1198

Research Committee on Spontaneous Occlusion of the Circle
of Willis of the Ministry of Health, Labor, and Welfare,
Japan. Single STA–MCA anastomosis with encephalo-duromyo-synangiosis (EDMS) and dural pedicle insertion [16, 21]
were performed on the right. After exploring the parietal
branch of the left STA, fronto-temporo-parietal craniotomy
was performed. The recipient artery at the M4 segment of the
MCA was then explored and anastomosis was performed
between the stump of the STA (0.8 mm in diameter) and the
proximal portion of the M4 segment (0.8 mm in diameter)
that supplied the fronto-parietal lobe. Then EDMS and dural
pedicle insertion were performed. She showed no neurological deficit immediately after surgery. The 123I-IMP-SPECT
1 day after surgery showed no apparent change in CBF at the
site of anastomosis (Fig. 1b) compared to the preoperative
findings (Fig. 1a). Postoperative diffusion-weighted MR
imaging 2 days after surgery showed no evidence of ischemic
change (Fig. 2a). MR angiography showed the apparently
patent STA–MCA bypass (Fig. 2c, arrow). She suffered left
facial palsy and numbness on her left hand 5 days after
surgery. The SPECT 7 days after surgery showed focal
intense increase in CBF at the sites of anastomosis (Fig. 1c).
MR imaging showed no ischemic changes, and MR
angiography showed the thick high signal of STA (Fig. 2d,
arrow) compared to that seen 2 days after surgery (Fig. 2c).
We suspected the involvement of hyperperfusion in her
neurological deterioration, and we kept her under intensive
blood pressure control with the intravenous administration of

months later, normalization of CBF at the territory of the right MCA
was evident (d) in accordance with the recovery of her neurological
deficit

Childs Nerv Syst (2007) 23:1195–1198

1197

Fig. 2 Postoperative diffusion-weighted magnetic resonance images
2 days after surgery showing no evidence of ischemic change (a).
Magnetic resonance angiogram before (b) and after surgery (c, d).
Magnetic resonance angiogram 2 days after surgery showing the

apparently patent STA–MCA bypass (arrow in c). Signal intensity of
the STA–MCA bypass became more prominent 9 days after surgery
(arrow in d)

free radical scavenger. Her facial palsy and numbness
recovered completely 9 days after surgery. She was discharged without neurological deficit 11 days after surgery.
One month later, she underwent surgical revascularization on
the left side and was discharged without neurological deficit
after an uneventful course. The SPECT performed 63 days
after initial surgery showed normalization of CBF at the
territory of the right middle cerebral artery (Fig. 1d). Surgical
revascularization completely relieved her transient ischemic
attack that was seen before surgery.

profile of hyperperfusion, as shown by time sequential
SPECT after surgery, were completely in accordance with
her postoperative course. The reversibility of both her
symptom and the SPECT finding further convinced the
central role of hyperperfusion in her postoperative neurological deterioration.
The clinical manifestation due to hyperperfusion in our case
was distinct from the ‘cerebral hyperperfusion syndrome’ seen
after carotid endarterectomy or STA–MCA anastomosis for
atherosclerotic cerebral occlusive disease, which is characterized by unilateral headache, face and eye pain, seizures, and
focal symptoms secondary to cerebral edema or intracerebral
hemorrhage [14, 17, 18]. The clinical presentation of our case
was characterized by simple focal neurological deficit without
headache and seizure. Also in adult patients with moyamoya
disease, we observed similar transient neurological deficit
without headache due to postoperative focal hyperperfusion
in our same series [3].
The underlying mechanism of such specific manifestations
of hyperperfusion in patients with moyamoya disease remains
undetermined. Certain specific biological conditions, such as
differences in reactive oxygen species (ROS) production after
vascular reconstruction, vulnerability to ROS, and the expression of antioxidant enzymes in the chronic ischemic cortex
may be involved in these intrinsic responses to vascular
reconstruction against chronic ischemic brain in moyamoya
disease because ROS has been implicated in cerebral ischemia/

Discussion
Postoperative hyperperfusion syndrome has been considered to be less common in patients with moyamoya disease
[5, 7, 15] due to the relatively low flow revascularization
obtained by surgery for moyamoya disease. Furthermore, it
was totally unclear how the rapid increase in CBF affects
ischemic brain in childhood moyamoya disease, although
substantial number of children with moyamoya disease, as
much as 59.3% of patients with STA–MCA anastomosis,
were reported to suffer transient neurological deterioration
due to unknown mechanism [20]. We demonstrate, for the
first time, a 4-year-old girl manifesting delayed transient
facial palsy due to hyperperfusion 5 to 9 days after single
STA–MCA anastomosis. Anatomical location and temporal

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reperfusion injury [1, 2]. Antioxidant agent is reported to
prevent hyperperfusion syndrome after carotid endarterectomy
in patients with atherosclerotic occlusive disease and markedly
affected cerebrovascular reserve capacity [11]. Therefore, we
treated her with edaravone, a novel free radical scavenger, to
ameliorate the unfavorable effects of hyperperfusion on the
affected brain.
Long-term follow-up of the neurological function including cognitive functions and the histological changes in
the affected brain is needed because exposure to sublethal
levels of ROS can affect organelles, and thus lead to
delayed neuronal damage by apoptosis [2]. Postoperative
cerebral hyperperfusion may be associated with impairment
of cognitive function in patients undergoing carotid
endarterectomy [13]. These characteristics of moyamoya
disease remain to be elucidated. Identification of the
predictors for cerebral hyperperfusion in patients with
moyamoya disease is clinically important. Preoperative
cerebrovascular reserve capacity [10], intraoperative ischemic period [9], difference in vascular anatomy, and patient
age may affect postoperative cerebral hyperperfusion.
Investigation of a larger number of patients is required.

Conclusion
We demonstrated, for the first time, that transient focal
neurological deficit after STA–MCA anastomosis can be
caused by focal hyperperfusion in a child with moyamoya
disease. Routine CBF measurement by SPECT is recommended for the differential diagnosis between hyperperfusion and transient ischemic attack because the treatments
for these conditions are contradictory.

References
1. Chan PH (1996) Role of oxidants in ischemic brain damage.
Stroke 27:1124–1129
2. Fujimura M, Tominaga T, Chan PH (2005) Neuroprotective effect
of antioxidant in cerebral ischemia: role of neuronal apoptosis.
Neurocrit Care 2:59–66
3. Fujimura M, Shimizu H, Tominaga T (2006) Transient focal
neurological deficit due to hyperperfusion after superficial
temporal artery–middle cerebral artery anastomosis in patients
with moyamoya disease. Surgery for Cerebral Stroke 34:37–41
4. Furuya K, Kawahara N, Morita A, Momose T, Aoki S, Kirino T
(2004) Focal hyperperfusion after superficial temporal artery–
middle cerebral artery anastomosis in a patient with moyamoya disease. Case report. J Neurosurg 100:128–132
5. Houkin K, Ishikawa T, Yoshimoto T, Abe H (1997) Direct and
indirect revascularization for moyamoya disease: surgical techniques and peri-operative complications. Clin Neurol Neurosurg 99(2):S142–S145

Childs Nerv Syst (2007) 23:1195–1198
6. Houkin K (2001) Direct bypass surgery. In: Loftus CM (ed)
Moyamoya disease. AANS Press, Rolling Meadows, Illinois,
pp 157–165
7. Houkin K, Nonaka T, Baba T (2005) Peri-operative complications
in surgical treatment for moyamoya disease. In: Annual Report 2004
by the Research Committee on Spontaneous Occlusion of the Circle
of Willis (Moyamoya Disease), pp 47–50
8. Ishikawa T, Houkin K, Kamiyama H, Abe H (1997) Effects of
surgical revascularization on outcome of patients with pediatric moyamoya disease. Stroke 28:1170–1173
9. Komoribayashi N, Ogasawara K, Kobayashi M, Saitoh H,
Terasaki K, Inoue T, Ogawa A (2005) Cerebral hyperperfusion
after carotid endarterectomy is associated with preoperative
hemodynamic impairment and intraoperative cerebral ischemia. J Cereb Blood Flow Metab 26(7):878–884
10. Ogasawara K, Yukawa H, Kobayashi M, Mikami C, Konno H,
Terasaki K, Inoue T, Ogawa A (2003) Prediction and monitoring
of cerebral hyperperfusion after carotid endarterectomy by
using single-photon emission computerized tomography scanning. J Neurosurg 99:504–510
11. Ogasawara K, Inoue T, Kobayashi M, Endo H, Fukuda T, Ogawa
A (2004) Pretreatment with the free radical scavenger edaravone prevents cerebral hyperperfusion after carotid endarterectomy. Neurosurgery 55:1060–1067
12. Ogasawara K, Komoribayashi N, Kobayashi M, Fukuda T, Inoue
T, Yamada K, Ogawa A (2005) Neural damage caused by cerebral
hyperperfusion after arterial bypass surgery in a patient with
moyamoya disease: case report. Neurosurgery 56:E1380
13. Ogasawara K, Yamadate K, Kobayashi M, Endo H, Fukuda T,
Yoshida K, Terasaki K, Inoue T, Ogawa A (2005) Postoperative
cerebral hyperperfusion associated with impaired cognitive
function in patients undergoing carotid endarterectomy. J
Neurosurg 102:38–44
14. Piepgras DG, Morgan MK, Sundt TM Jr, Yanagihara T, Mussman
LM (1988) Intracerebral hemorrhage after carotid endarterectomy.
J Neurosurg 68:532–536
15. Sakamoto T, Kawaguchi M, Kurehara K, Kitaguchi K, Furuya H,
Karasawa J (1997) Risk factors for neurologic deterioration
after revascularization surgery in patients with moyamoya
disease. Anesth Analg 85:1060–1065
16. Shirane R, Yoshida Y, Takahashi T, Yoshimoto T (1997)
Assessment of encephalo-galeo-myo-synangiosis with dural
pedicle insertion in childhood moyamoya disease: characteristics of cerebral blood flow and oxygen metabolism. Clin
Neurol Neurosurg 99(2):S79–S85
17. Solomon RA, Loftus CM, Quest DO, Correll JW (1986) Incidence
and etiology of intracerebral hemorrhage following carotid
endarterectomy. J Neurosurg 64:29–34
18. Sundt TM Jr, Sharbrough FW, Piepgras DG, Kearns TP, Messick
JM Jr, O’Fallon WM (1981) Correlation of cerebral blood flow
and electroencephalographic changes during carotid endarterectomy: with results of surgery and hemodynamics of cerebral
ischemia. Mayo Clin Proc 56:533–543
19. Suzuki J, Takaku A (1969) Cerebrovascular “moyamoya” disease.
Disease showing abnormal net-like vessels in base of brain. Arch
Neurol 20:288–299
20. Taki W, Tanaka M, Miyamoto S, Nagata I, Kikuchi H (1995)
Postoperative transient neurological deficit in children with
moyamoya disease. In: Annual Report 1994 by the Research
Committee on Spontaneous Occlusion of the Circle of Willis
(Moyamoya Disease), pp 88–93
21. Yoshida YK, Shirane R, Yoshimoto T (1999) Non-anastomotic
bypass surgery for childhood moyamoya disease using dural
pedicle insertion over the brain surface combined with
encephalogaleomyosynangiosis. Surg Neurol 51:404–411