Pragmatic anaesthetic approach for extracranial to intracranial (ECIC) bypass surgery in a patient with Moyamoya disease and Sickle cell disease.

Background: Moyamoya disease is a chronic progressive cerebrovascular disorder. 10–20% of patients with sickle cell disease have associated moyamoya disease (MMD) and may require surgical revascularisation as definitive treatment. Case presentation: A patient with sickle cell disease and moyamoya disease with extensive cerebral vasculopathy was scheduled for elective ECIC bypass surgery. She presented with right-sided weakness secondary to a haemorrhagic stroke of the left lentiform nucleus. She required a multidisciplinary team approach for pre-procedural optimisation. Her preoperative HbSS levels were reduced to less than 20% with preoperative red blood cell transfusion to avoid sickling. We maintained normal physiology and optimal analgesia peri-operatively. She was extubated after the successful surgical procedure and was transferred to ICU for invasive monitoring, with subsequent discharge to the ward several days later. Conclusion Optimal pre-procedural optimisation can decrease complications in patients with critically comprised cerebral circulation booked for extensive surgery such as ECIC bypass. We believe the presentation of anaesthetic management of a patient with moyamoya disease and sickle cell disease may prove helpful.