Williams & Martin: MOYAMOYA DISEASE
999
the specific hazards associated with general anesthesia,
and allows continuous monitoring of cerebral func-
tion. It is important to titrate neuraxial blockade to
References
1 Suzuki J, Kodoma N. Moyamoya disease: a review.
8
Stroke 1983; 14: 104–9.
avoid hypotension and subsequent cerebral hypoper-
fusion. Patient reassurance can provide anxiolysis and
thereby prevent hyperventilation and potential cere-
bral ischemia.
2 Malley RA, Frost EAM. Moyamoya disease: pathophysi-
ology and anesthetic management. J Neurosurg
Anesthesiol 1989; 1: 110–4.
3 Numaguchi Y, Gonzalez CF, Davis PC, et al.
Moyamoya disease in the United States. Clin Neurol
Neurosurg 1997; 99: S26–30.
4 Aoki N, Mizutani H. Does moyamoya disease cause
subarachnoid hemorrhage? Review of 54 cases with
intracranial hemorrhage confirmed by computerized
tomography. J Neurosurg 1984; 60: 348–53.
5 Takeuchi S, Kobayashi K, Tsuchida T, Imamura H,
Tanaka R, Ito J. Computed tomography in Moyamoya
Disease. J Comput Assist Tomogr 1982; 6: 24–32.
6 Iwama T, Morimoto M, Hashimoto N, Goto Y, Todaka
T, Sawada M. Mechanism of intracranial rebleeding in
Moyamoya disease. Clin Neurol Neurosurg 1997; 99:
187–90.
7 D’Haese J, Christiaens F, D’Haens J, Camu F.
Combined Caesarean section and clipping of a rup-
tured cerebral aneurysm. J Neurosurg Anesthesiol
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8 Komiyama M, Yasui T, Kitano S, Sakamoto H, Fujitani
K, Matsuo S. Moyamoya disease and pregnancy: case
report and review of the literature. Neurosurgery
1998; 43: 360–9.
9 Tagawa T, Neritomi H, Mimaki T, Yabuuchi H,
Sawada T. Regional cerebral blood flow, clinical mani-
festations, and age in children with moyamoya disease.
Stroke 1987; 18: 906–10.
10 Kurokowa T, Chen YJ, Tomita S, Kishikawa T,
Kitamura K. Cerebrovascular occlusive disease with
and without the Moyamoya vascular network in chil-
dren. Neuropediatrics 1985; 16: 29–32.
General anesthesia allows optimal control of ventila-
tion but may be hazardous due to the risks of hyper-
tension at intubation, gastric aspiration and neonatal
depression. Traditionally, the choice of anesthetic
agents has consisted of a combination of potent volatile
anesthetic, nitrous oxide and intravenous narcotics.
Recent evidence offers support for continuous propofol
intravenous anesthesia in Moyamoya disease. Sato et al.
found that regional cortical blood flow diminished
under isoflurane-nitrous oxide general anesthesia com-
pared with total intravenous anesthesia using propo-
14
fol. Volatile anesthetics are known to have cerebral
vasodilating properties and it postulated that a steal
phenomenon analogous to hypercapnia occurs in
Moyamoya disease. Propofol anesthesia has been suc-
cessfully used for Cesarean section in a patient with
15
known Moyamoya disease. Continuous propofol infu-
sion reduces the hypertensive response to laryngoscopy
15,16
and intubation
which is important in Moyamoya
patients prone to intracranial hemorrhage. Neonatal
status as assessed by Apgar scores, cord acid base status
and the neurologic adaptive capacity scores are similar
when using intravenous propofol vs thiamylal-isoflurane
1
7
anesthesia. Therefore, propofol anesthesia appears to
be a safe alternative to “balanced anesthesia” for
Cesarean section, and may offer specific benefits in
Moyamoya patients.
Vaginal delivery can be performed under spinal or
epidural analgesia. Epidural anesthesia has been shown
to prevent hyperventilation and hypocapnia associated
with labour.1 8 In Moyamoya patients neuraxial blockade
can be used to help reduce hyperventilation and hyper-
tension during the first and second stages of labour.
11 Takeuchi S, Tanaka R, Ishii R, Tsuchida T, Kobayashi
K, Arai H. Cerebral hemodynamics in patients with
moyamoya disease. Surg Neurol 1985; 23: 468–74.
12 Brown SC, Lam AM. Moyamoya disease – a review of
clinical experience and anaesthetic management. Can J
Anaesth 1987; 34: 71–5.
Conclusion
Intracranial hemorrhage can be confused with preg-
nancy-induced hypertension because transient hyper-
tension and proteinuria can complicate ICH. The
specific diagnosis of ICH can only be made after non-
contrast CT scan has been performed.
In pregnant patients with known Moyamoya dis-
ease the principle aim of anesthesia is to maintain nor-
moventilation, normothermia and normotension
during delivery.
13 Khan-Ghori SN, Murshid WR, Samakandi AHH, Al-
Salman M, Salih MAM. Use of propofol and sevoflu-
rane in moyamoya disease – case reports and literature
review. MEJ Anesth 1999; 15: 73–83.
14 Sato K, Shirane R, Kato M, Yoshimoto T. Effect of inhala-
tional anesthesia on cerebral circulation in Moyamoya
disease. J Neurosurg Anesth 1999; 11: 25–30.
15 Furuya A, Matsukawa T, Ozaki M, Kumazawa T.
Propofol anesthesia for Cesarean section successfully
managed in a patient with moyamoya disease.
(Japanese) J Clin Anesth 1998; 10: 242–5.