The efficacy of partial versus total splenectomy in the treatment of hereditary spherocytosis in children: a systematic review and meta-analysis.

Objective: To explore the comparative postoperative efficacy of partial splenectomy (PS) and total splenectomy (TS) in the treatment of children with hereditary spherocytosis (HS).

Methods: The relevant HS studies from databases were searched and screened, comparing the differences in hemoglobin concentration, reticulocyte percentage, bilirubin concentration before and after TS and PS surgery, and during the follow-up period, as well as the incidence of postoperative adverse events. Statistical analysis was performed using Review Manager 5.4.

Results: A total of 5 studies were included in this meta-analysis, with a cumulative enrollment of 312 children, 130 in the PS group and 182 in the TS group. The meta-analysis results showed that both PS and TS groups had statistically significant differences in postoperative hematological outcomes compared to before surgery, with TS showing better improvement than PS. In the postoperative follow-up, the changes in hematological outcomes between PS and TS were statistically significant for hemoglobin concentration: within 1 year [MD = 1.85, 95%CI(1.09,2.60)], 1-2 years [MD = 1.74, 95%CI(0.25,3.24)], not statistically significant for 4-6 years [MD = 1.28, 95%CI(-1.75,4.32)]; for reticulocyte percentage: within 1 year [MD = 2.23, 95%CI(0.80,3.66)] was statistically significant, not statistically significant for 4-6 years [MD = 1.77, 95%CI(-2.04,5.59)]; for serum bilirubin concentration: within 1 year [MD = 1.55, 95%CI(0.91,2.18)] was statistically significant, not statistically significant for 1-2 years [MD = 1.77, 95%CI(-2.04,5.99)]. In the incidence of postoperative adverse events, the incidence of cholelithiasis [MD = 1.77, 95%CI(-2.04,5.99)] showed a statistically significant difference between PS and TS, while there were no statistically significant differences in other included events, such as postoperative infection rate, secondary surgery rate, thrombosis rate, postoperative hemorrhage rate, and transfusion therapy rate.

Conclusion: Splenectomy is a beneficial surgical strategy for children with moderate-to-severe HS, reducing; early hematological outcomes of TS are more robust than PS in the follow-up period, and there is no difference between the two in the later period; for postoperative adverse events, the incidence of cholelithiasis in children after PS is higher than after TS, and there is a risk of requiring a second surgery for total splenectomy due to hemolytic recurrences.