Moyamoya Disease Cases

Moyamoya Disease Cases

Patients with Moyamoya Disease have progressive cerebrovascular disease which often leads to cerebral infarctions and death. Limited treatments are available. Some treatment options to restore cerebral blood supply include EC-IC bypass and EDAS (encephaloduroarteriosynangiosis), a procedure in which superficial temporal artery is sutured to the surface of the brain. No one knows what the ideal anesthetic regimen is, but clearly low blood pressure and hyperventilation may lead to strokes. For all Moyamoya cases, please review the anesthesia technique for EC-IC Bypass cases, and in addition please follow the following guidelines as closely as possible.

1) Maintain mean arterial pressure (MAP) above awake baseline MAP values at all times. Use phenylephrine. Have a phenylephrine infusion available prior to induction of anesthesia.
2) Insert an arterial catheter before induction of anesthesia (to achieve #1)
3) Use an anesthetic technique which allows SSEP and EEG monitoring, including baseline values.
4) Maintain arterial pCO2 around 40 mmHg (no hyperventilation).
5) Insert a CVP catheter (postinduction is OK). The goal will be to maintain adequate hydration to maximize CBF (suggested CVP 10-12 mmHg). Consider moderate fluid loading before induction of anesthesia (500 ml Lactated Ringer’s)
6) Mannitol will only be administered on surgeon’s request.
7) Surgeons and anesthesiologists should communicate regarding the surgery/anesthesia plan before the operation.

Following these parameters may not be possible in all patients due to their coexisting disease. Naturally that would indicate further communication and decision making.


Last revised 05/2013 Talke

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This information is meant to serve as an educational resource. Clinicians should use their own professional judgment in the care of any individual patient as the guidance contained in this document may not be appropriate for all patients or all situations.