Résumé
To evaluate the clinical and hematological response to subtotal splenectomy in children with hereditary spherocytosis [HS]. The authors analyzed the main clinical and hematological features in 18 patients with HS treated by subtotal splenectomy. The average age at the time of operation was 4 years. The indications for subtotal oplenetomy were hypersplenism and severe anemia. The diagnosis of HS was made according to standard methods. The lower three fourth of the spleen were removed and the upper pole was preserved. The effect on hemolytic rate was assessed by comparing the presurgical and postsurgical values of hemoglobin, reticulocyte number and RBC life span. The residual splenic phagocytic function was assessed using technetium 99m scan and number of pitted red cells. The splenic regrowth were measured by ultrasonography. There were no complications related to the surgical procedure in any of the 18 children. The mean follow up period was 30 +/- 8 months. At the end of follow up hemoglobin increased on the average by 3 gm/dl, reticulocyte count decreased by 300x10[6]/L, and bilirubin level decreased. Normal technetium uptake was noted in the splenic remnant. Splenic regrowth were noted in 4 patients, two of them still need more than 2 transfusion/ year. No postoperative overwhelming infection occurred. Subtotal splenectomy can be performed without major blood loss in patients with HS. Up to 95% of the spleen can be safely removed. It is effective in decreasing the hemolytic rate while maintaining residual splenic function