Anaesthetic management of a child with hemoglobinopathy

Perioperative management of a child with sickle-cell disease requires close collaboration between hematologist, surgeon and anesthetist. The level of preoperative preparation must consider both the surgical risk and the impact of the disease. Preoperative hydration and blood transfusion are the most important part of preoperative management. Anesthetic technique is not as important as preoperative preparation, management of stress and anxiety, and optimization of intraoperative physiological parameters (oxygenation, acid-base balance, tissue perfusion, normothermia). Pain management must be optimal for these children with increased needs of analgesic. Preoperative assessment of a child with thalassemia must include evaluation of the impact of chronic hemolytic anemia and iron overload induced by repeated transfusions. The most important factor in the management of a child with glucose-6-phosphate dehydrogenase deficiency is to avoid exposure to oxidative stressors. Outpatients should be informed of the risk of hemolytic crisis, and free hemoglobin in the urine should lead to discontinuation of drugs associated with hemolysis and to maintenance of urine output to prevent acute renal failure.