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A 2-year-old girl recently had an ischemic stroke. Cerebral angiography confirmed the diagnosis of moyamoya disease. She presents for encephaloduroarteriosynangiosis (EDAS).

Laboratory: Thyroid-stimulating hormone 0.25

Physical examination: Right hemiparesis


  • Moyamoya disease is a rare cause of strokes in children, characterized by progressive stenosis of the distal internal carotid and basilar arteries. Affected children present with ischemic stroke, transient ischemic attacks (TIAs), and headache, and symptoms may be exacerbated by crying or straining; adults may present with intracranial hemorrhage.

  • Medical therapy with vasodilators such as calcium channel blockers and antiplatelet drugs such as aspirin does not prevent progression of the disease.

  • Surgical intervention aims to increase collateral cerebral blood flow (CBF) via either direct or indirect revascularization. In adults, direct revascularization usually is accomplished with a superficial temporal artery (STA) to middle cerebral artery bypass, but this is technically difficult in children. The most common indirect revascularization surgery in children is EDAS, in which the STA is dissected free and then sutured to the edges of the opened dura.

  • A history of frequent TIAs suggests decreased CBF and may predict perioperative complications. Medications should be taken until the day of surgery. Patients should be kept as hydrated as possible within nothing by mouth guidelines to maintain CBF.

  • Crying can lead to cerebral ischemia in patients with moyamoya disease, since crying leads to hyperventilation and hypocapnia, which in turn may cause cerebral vasoconstriction and decreased CBF. Therefore, premedication with an anxiolytic such as midazolam is recommended.


  • Avoid hyperventilation and hypotension with induction (whether inhalational or IV).

  • Minimize response to laryngoscopy and intubation with opioids and lidocaine to prevent increases in cerebral oxygen consumption (CMRO2). Avoid ketamine.

  • Maintain normocarbia to prevent cerebral vasoconstriction and ischemia.

  • Maintain normothermia; hyperthermia causes increased CMRO2 and potential cerebral ischemia, while hypothermia may cause cerebral vasospasm and stroke.

  • Maintain BP ≥ baseline: use invasive BP monitoring, aggressive hydration, and pressors as needed.

  • Crystalloid solutions should not contain glucose.

  • Maintain hematocrit >30% to prevent cerebral ischemia. Monitor urine output closely to assess volume status.


Pain control is critical to prevent increased cerebral metabolism and possible stroke; this usually is accomplished with narcotic patient-controlled analgesia.

DOs and DON’Ts

  • ✓ Do premedicate with midazolam to prevent crying and agitation on induction.

  • ⊗ Do not hyperventilate the patient during either induction or maintenance of anesthesia.

  • ✓ Do use opioids and lidocaine to attenuate the response to laryngoscopy and intubation.

  • ✓ Do keep the patient well hydrated and maintain BP ≥ baseline.

  • ✓ Do aggressively treat any postoperative pain.

  • ⊗ Do not allow the patient to become too warm or too cold.


  • Use of total intravenous anesthesia or inhalation anesthesia.


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