Anesthetic management in a child with moya-moya disease and sickle cell anemia: Case report.

OBJECTIVE: The objective of this case report is to describe the anesthetic management in a child with moya-moya disease and sickle cell anemia provided in a tertiary hospital. CASE: A 6 year-old patient, diagnosed with moya-moya disease and sickle cell anemia, both conditions associated with a greater incidence of intracranial ischemic events, with a history of two strokes of the ischemic subtype, was submitted to general anesthesia for the execution of multiple cranial burr holes in order to produce the neovascularization in poorly perfused regions. There were no complications in the perioperative period and the child was discharged from the hospital on the second postoperative day.
CONCLUSION: Although scarcely described in the medical literature, the anesthetic management in a patient with moya-moya disease must ensure the maintenance of cerebral blood flow, normocapnia and the appropriate mean arterial pressure. In a patient with sickle cell disease, an adequate tissue perfusion, adequate oxygenation and hydration and strict pain control are to be primarily expected. The anesthesiologist is expected to know the physiopathology of both conditions to provide the best outcome for these patients.