Evaluation of partial and total splenectomy in children with sickle cell disease using an Internet-based registry.

Background: Clinical outcomes of children with sickle cell disease (SCD) who undergo total or partial splenectomy (PS) are poorly defined. The purpose of this retrospective study was to initiate an Internet-based registry to facilitate analysis of clinical outcomes for these children. We hypothesized that both surgical procedures would be well tolerated and would eliminate risk of splenic sequestration.

Methods: We developed a web-based registry using the Research Electronic Data Capture (REDCap) platform. Children were included if they had SCD and underwent total splenectomy (TS) or PS between 2003 and 2010. Clinical outcomes were compared between cohorts, with follow-up to 1 year.

Results: Twenty-four children were included, TS (n = 15) and PS (n = 9). There were no differences in surgical time or intraoperative blood loss. The length of stay was longer after PS (4.1 ± 1.7 days) compared to TS, (2.4 ± 1.2 days, P = 0.02). Within 30 days of surgery, 2 (20%) patients had acute chest syndrome (ACS) following TS and 2 (15%) patients had ACS after PS. During 1-year follow-up, no patient in either cohort had recurrent splenic sequestration, venous thrombosis or overwhelming postsplenectomy sepsis. All children who were transfused preoperatively to prevent recurrent splenic sequestration successfully discontinued transfusions.

Conclusions: Both TS and PS result in favorable hematologic outcomes and low risk of adverse events for children with SCD. A REDCap-based registry may facilitate data entry and analysis of clinical outcomes to allow for comparison between different types of splenectomy.