Abstract
BACKGROUND: Moyamoya disease is a chronic progressive cerebrovascular disorder. A proportion of 10-20% of patients with sickle cell disease have associated moyamoya disease and may require surgical revascularization as definitive treatment. CASE PRESENTATION: A 22-year-old African lady with sickle cell disease and moyamoya disease, with extensive cerebral vasculopathy, was scheduled for elective extracranial to intracranial bypass surgery. She presented with right-sided weakness secondary to a hemorrhagic stroke of the left lentiform nucleus. She required a multidisciplinary team approach for preprocedural optimization. Her preoperative hemoglobin SS levels were reduced to less than 20%, with preoperative red blood cell transfusion to avoid sickling. We maintained normal physiology and optimal analgesia perioperatively. She was extubated after the successful surgical procedure and was transferred to Intensive care unit (ICU) for invasive monitoring, with subsequent discharge to the ward several days later. CONCLUSION: Optimal preprocedural optimization can decrease complications in patients with critically comprised cerebral circulation booked for extensive surgery such as ECIC bypass. We believe the presentation of anesthetic management of a patient with moyamoya disease and sickle cell disease may prove helpful.