Myocardial necrosis following general anesthesia in hemoglobin SC disease

A 4-year-old girl with hemoglobin SC disease died following general anesthesia. Autopsy showed widespread intravascular sickling; staining with hematoxylin-basic fuchsin-picric acid demonstrated newly developed massive myocardial necrosis, a rarely documented finding. Anesthesia may produce conditions that provoke "crises" in patients with sickle hemoglobinopathies. Preoperative identification of sickle states and careful attention to hydration and oxygenation may minimize anesthetic risks in these patients.