Hydroxyurea has enhanced the treatment for children with
sickle cell anemia. The objectives of this study were to compare perioperative transfusions and outcomes for children taking
hydroxyurea versus those not taking
hydroxyurea. We retrospectively reviewed perioperative management and outcomes for 51 children with
sickle cell anemia (HbSS genotype) who underwent surgery in our center between January 2003 and April 2008. Of the 51 patients, 30 (59%) were taking
hydroxyurea and 21 (41%) were not taking
hydroxyurea. Eight of 30 (27%) in the
hydroxyurea group were not transfused preoperatively, 12 of 30 (40%) received a single transfusion and 10 of 30 (33%) received serial transfusions, compared with 1 of 21 (5%) children in the nonhydroxyurea group who was not transfused, 2 of 21 (10%) who received a single transfusion and 18 of 21 (85%) who received serial transfusions or
pheresis (P=0.004; for comparison across groups). One patient not taking
hydroxyurea developed a delayed hyperhemolytic
transfusion reaction, and 2 children taking
hydroxyurea developed
acute chest syndrome. Overall, children taking
hydroxyurea had substantially fewer transfusions compared with children not taking
hydroxyurea. Both groups of children had a low complication rate. Further research should be done to optimize perioperative management of children taking
hydroxyurea.