Anesthesia for cerebral revascularization for adult moyamoya syndrome associated with sickle cell disease

We report the anesthetic management of an adult patient undergoing cerebral revascularization surgery for moyamoya syndrome complicating sickle-cell disease (SCD). We present a 25 year-old male of African ethnicity with homozygous SCD who was experiencing worsening ischemic neurologic symptoms culminating in intraventricular hemorrhage from rupture of moyamoya vessels. Despite an extracranial-intracranial superficial temporal artery-middle cerebral artery bypass that was angiographically patent postoperatively, he subsequently required an intracranial omental transplant to improve cerebral blood flow to the anterior cerebral artery territory. Prior to both cerebral revascularization procedures, the patient had continued with his regularly scheduled red blood cell exchange transfusion. The importance of normothermia, normocarbia, normotension, and normovolemia is emphasized in the neuroanesthetic management. We conclude that the safe and efficacious operative treatment of moyamoya disease, using both direct and indirect revascularization procedures, is being increasingly described, and therefore anesthesiologists are likely to encounter similar cases in the future and need to be aware of the surgical procedures and perioperative implications. The overall principles of safe anesthesia (normotension, normocarbia, good oxygenation, normothermia, normovolemia) for patients with SCD also applies to patients with moyamoya. During a craniotomy, certain deviations from these are needed (hyperventilation and mannitol diuresis for brain volume reduction, induced hypothermia or manipulations of arterial blood pressures) but they can be safely used with careful monitoring of the patient.