Moyamoya disease

Keyword

Moyamoya disease

Definition

Moyamoya is the Japanese term for “ puff of smoke”

Progressive stenosis of intracranial vessels로 인해 secondary development of an anastomotic capillary network가 발생,

그 결과 나타나는 작은 비정상 혈관들이 밀집해있는 angiographic finding을 표현한 명칭

Increased frequency - Intracranial aneurysms, symptoms of ischemia(ex.TIA, Cerebral infarcts) in children, hemorrhagic complication in adults

Treatment

Aimed at decreasing ischemic symptoms ; vasodilators and anticoagulants

Surgical treatment ; Extracranial-intracranial bypass or other indirect revascularization procedures

Management of anesthesia

Anticoagulant or antiplatelet drug therapy should be discontinued

Goal of induction and maintenance of anesthesia

• Ensuring hemodynamic stability

(hypotension - risk of ischemia, hypertension - risk of hemorrhage)

• Avoiding factors that lead to cerebral or peripheral vasoconstriction

(Avoid hypocapnia or phenylephrine; hypotension시 ephedrine 이나 dopamine 사용)

• Facilitating a rapid emergence from anesthesia ;