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Moyamoya disease is one of several conditions generally described as
"cerebrovascular insufficiency". Moyamoya disease affects both adults
and children. It is characterized by progressive narrowing of the
internal carotid artery as it enters the base of the brain. This
narrowing results in decreased blood flow to the brain. The decreased
blood flow leads to strokes or transient ischemic attacks (TIA’s or
"mini-strokes"). Typically medical treatment of this condition with
drugs is not effective. At Johns Hopkins, patients with moyamoya disease
and other cerebrovascular insufficiency conditions undergo an extensive
evaluation by a team of neurosurgeons, neurologists, and
neuroradiologis. If the problem cannot be treated with medicine then
surgery is considered.
During surgery, new blood flow to the brain can be accomplished using
one of three techniques. We typically use all three techniques during
one surgery. The first surgical technique is the microsurgical
extracranial-to-intracranial (EC-IC) bypass surgery. In general this is
done using the superficial temporal artery (STA) which is anastomosed to
the middle cerebral artery (MCA). This is referred to as an STA-MCA
bypass, which is defined as a “direct” revascularization technique.
When a direct revascularization procedure such as the STA-MCA bypass
cannot be carried out, we perform two "indirect" bypass procedures.
These consist of (1) transferring an arteriovenous pedicle of the
superficial temporal artery to the surface of the brain and (2) bringing
the temporalis muscle in contact with the brain. The first procedure is
referred to as the encephaloduroarteriosynangiosis (EDAS) and the second
as the encephaloduromyosysnangiosis (EDMS). Typically these two are done
together and then the procedure is described as the
encephaloduroarteriomyosynangiosis (EDAMS). With time new vessels grow
into the brain from the arteriovenous STA pedicle or the temporalis muscle.
Johns Hopkins is one of the few centers where these procedures are
performed. The results are typically outstanding. |
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