Evaluation of partial and total splenectomy in children with sickle cell disease using an internet‐based registry

Abstract

Background Clinical outcomes of children with sickle cell disease (SCD) who undergo total or partial splenectomy 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 partial splenectomy (PS) between 2003 to 2010. Clinical outcomes were compared between cohorts, with follow-up to one year. Results Twenty-four children were included, total splenectomy (n=15) and partial splenectomy (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 following TS and 2 (15%) patients had acute chest syndrome after PS. During one year follow-up, no patient in either cohort had recurrent splenic sequestration, venous thrombosis or overwhelming post-splenectomy 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.

Clinical outcomes of splenectomy in children: Report of the splenectomy in congenital hemolytic anemia registry

“…There were 8 eligible children reported to the central coordinating center whose parents declined enrollment, although the total number of eligible children who were not enrolled was not collected. To test feasibility of this registry, we pilot tested it at a single site and previously reported these findings in 24 children with SCD [11]. Based on this pilot experience, we eliminated extraneous variables, refined technical aspects of the data entry, and expanded inclusion to multiple types of CHA prior to rollout as a multicenter registry.…”

Recommendations regarding splenectomy in hereditary hemolytic anemias

“…Limited evidence is available about any possible increased incidence of pulmonary arterial hypertension or pain frequency in SCD patients. 97 …”

Bacterial Infections Following Splenectomy for Malignant and Nonmalignant Hematologic Diseases

“…Splenectomy has been considered for a long time at high risk of infections, early and late, and of death in children and adult with SCA, 3 , 4 particularly in children of 4 years or below. Recently, after the introduction of conjugate vaccines 103 , 104 and prophylactic antibiotics, 105 splenectomy is considered in developed countries feasible at all age with a moderate risk, 31 , 106 , 107 which, in any case, is superior to that of other non-malignant hematological diseases. 92 Data from low-income countries are scarce; splenectomy is considered only in urgency, and then a comparison cannot be done.…”