Impact of hydroxyurea on perioperative management and outcomes in children with sickle cell anemia.

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.