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Technical note
Abstract
Moyamoya disease can have devastating eects on paediatric patients as a result of cerebral ischaemia. Several direct and indirect
surgical methods have been devised in order to facilitate revascularisation. Debate has long ensued about which methods are most ecacious and yet safe. The authors describe their experience with a straightforward method for performing the EDAMS (encephaloduro-arterio-myo-synangiosis) technique.
2005 Elsevier Ltd. All rights reserved.
Keywords: Moyamoya; Surgery; Revascularisation; EDAMS; EDAS; Children; Indirect bypass
1. Introduction
Moyamoya disease is a rare condition, rst described by
Takeuchi in the 1950s, as bilateral hypoplasia of the internal carotid arteries.1 Today, moyamoya disease is more
specically characterised by progressive spontaneous
occlusion of the supraclinoid internal carotid arteries, usually bilaterally, with secondary formation of a collateral
capillary network at the base of the brain.
Stroke is rare in the paediatric population. Moyamoya
disease is an important consideration in the dierential
diagnosis of stroke in children. Disease course is extremely
variable: some reports describe rapid progression from
diagnosis to multiple strokes over 12 years, whereas others report symptom-free intervals of up to 8 years. However, some characteristics that signal worse prognosis
include early age of onset, severity of clinical presentation,
rapid progression, and advanced angiographic ndings at
presentation.2
*
Corresponding author. Present address: Division of Neurosurgery,
UCSD Medical Center, 200 W. Arbor Drive, Suite 8893, San Diego, CA
92103-8893, USA. Tel.: +1 619 543 5540; fax: +1 619 543 2769.
E-mail address: hearyan@ucsd.edu (H.E. Aryan).
0967-5868/$ - see front matter 2005 Elsevier Ltd. All rights reserved.
doi:10.1016/j.jocn.2005.04.008
Although, adjuvant medical treatments exist, the mainstay of treatment is surgery. Various techniques aim to
augment the intracranial cortical blood ow using extracranial sources. It is well known that burr holes alone
can lead to revascularisation in patients with moyamoya
disease. Kawaguchi and his associates reported that this
technique was clinically eective.3 The other end of the
surgical spectrum is a supercial temporal artery to middle cerebral artery (STA-MCA) bypass. Although STAMCA anastomosis oers the greatest potential collateral
ow, it has its limitations. The technique was rst used
by Yasargil and was subsequently reported by Krayenbuhl in 1975 to have excellent angiographic and clinical results.4 A signicant limitation to performing a direct
bypass of this sort is the size of the parent and recipient
vessels in children. The diameter of the STA in children
is often 0.5 mm or less, with branches of the MCA even
smaller. Additional issues include the risk of temporary
MCA occlusion potentiating intraoperative stroke or
haemorrhage.
Between these extremes of the surgical spectrum lie
intermediate revascularisation options. EMS (encephalomyosynangiosis) is a technique initially developed by Henschen and initially performed by Karasawa in 1977.5 EDAS
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slightly anterior to the external auditory meatus. The incision curves upward, slightly posterior, and then wraps
anteriorly along the linea temporalis. The incision is made
with a no. 15 blade. In the regions away from the marked
STA branches, surgical scissors are used to perform a supragaleal opening. In this way, the branches of the STA remain adherent to the underlying galea. The scalp ap is
then reected inferiorly and held in place using a moistened
sponge.
Separate incisions using a no. 15 blade are then placed
through the galea, in between the branches of the STA
(Fig. 3). Through these incisions, the underlying temporalis
fascia and muscle is identied. Next, the temporalis muscle
is incised midway between the external auditory meatus
and the lateral canthus with a no. 15 blade (Fig. 4). The
incision is taken down to the underlying bone, and a subsequent incision is made to the most posterior aspect of
the temporalis muscle. The incision is taken superiorly,
leaving an approximately 1 cm cu to the linea temporalis.
Now the temporalis muscle aps can be further freed from
107
Fig. 6. Cortical exposure and punctate pial incisions into the cortex.
Fig. 4. Temporalis fascia and muscle incisions.
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Acknowledgement
The authors would like to acknowledge the artistic contributions of Mr Bill Winn of Atlanta, GA, USA.
References
3. Conclusion
Moyamoya disease can be devastating to its victims.
There continues to be erce debate about whether direct
STA-MCA bypass is necessary, or if one of the various
forms of indirect revascularisation techniques leads to