Total splenectomy (TS) and partial splenectomy (PS) are used for children with congenital hemolytic anemia (CHA), although the long‐term outcomes of these procedures are poorly defined. This report describes long‐term outcomes of children with CHA requiring TS or PS.

We collected data from children ages 2‐17 with hereditary spherocytosis (HS) or sickle cell disease (SCD) requiring TS or PS from 1996 to 2016 from 14 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a prospective, observational patient registry. We summarized hematologic outcomes, clinical outcomes, and adverse events to 5 years after surgery. Hematologic outcomes were compared using mixed effects modeling.

Over the study period, 110 children with HS and 97 children with SCD underwent TS or PS. From preoperatively compared to postoperatively, children with HS increased their mean hemoglobin level by 3.4 g/dL, decreased their mean reticulocyte percentage by 6.7%, and decreased their mean bilirubin by 2.4 mg/dL. Hematologic improvements and improved clinical outcomes were sustained over 5 years of follow‐up. For children with SCD, there was no change in hemoglobin after PS or TS following surgery, although all clinical outcomes were improved. Over 5 years, there was one child with HS and five children with SCD who developed postsplenectomy sepsis.

For children with HS, there are excellent long‐term hematologic and clinical outcomes following either PS or TS. Although hemoglobin levels do not change after TS or PS in SCD, the long‐term clinical outcomes for children with SCD are favorable.