Moyamoya disease is a form of occlusive cerebrovascular disease. Patients develop progressive occlusion of the internal cerebral arteries at the skull base. The disease is idiopathic in most cases. The internal cerebral arteries are often asymmetrically affected. Patients are at risk for both hemorrhagic and ischemic stroke. On cerebral angiography, in more advanced stages of the disease, the hemispheric perfusion appears as a ‘puff of smoke’, from which the disease derives its name (Moyamoya= Puff of smoke in Japanese).
Intraoperative concerns for the neuroanesthesiologist focus on tight hemodynamic control as excessive hypertension may cause hemorrhage from friable vessels and hypotension may cause ischemic stroke from hypoperfusion. The typical neurosurgical treatment involves Superficial Temporal Artery to Middle Cerebral Artery (MCA) Anastamosis through a small craniotomy, if there is a suitable MCA target. If not, an encephalo-duro-arterio-synangiosis (EDAS) can be performed by opposing a Superficial Temporal Artery pedicle to the cortical surface; neovascularization occurs over time (months to years).
Typical Anesthetic Approach (this is not intended to specify the treatment of an individual patient; only to serve as a guide; no high level evidence exists to support any given practice): Superficial Temporal Artery-Middle Cerebral Artery Anastamosis for Moyamoya Disease or other Occlusive CV Disease: 1. Maintain CPP; 2. Target SBP at or above baseline throughout; 3. Pre-induction a-line; 4. Central venous line for pressors; 5. Start norepi or phenylephrine infusion prior to induction; 6. FiO2 1.0 (100%) throughout; 7. Propofol & Remifentanil TIVA due to avoid cerebral steal from inhalational agents (this is on theoretical grounds only).
Reference: Parray T, Martin TW, Siddiqui S. Moyamoya disease: a review of the disease and anesthetic management. J Neurosurg Anesthesiol. 2011 Apr;23(2):100-9. PMID: 20924291