Perioperative management of children with sickle hemoglobinopathy.

During the 11 years between July, 1967 and June, 1978, 35 children with a major hemoglobinopathy (SS, SC, S-thalassemia) underwent 46 operations without any mortality or unusual morbidity. All of these children were prepared for surgery by preoperative transfusion of 15-20 cc/kg packed erythrocytes and a hematocrit of at least 36%. In urgent and emergent situations, the operation was performed as soon as the transfusion was completed. For elective patients, the operation was the following morning. The ease of application and the absence of morbidity and mortality suggest that this method of preoperative transfusion preparation of children with a major hemoglobinopathy is more desirable than a two-volume exchange or transfusions beginning 10-15 days prior to surgery.