Abstract

To compare the effectiveness of different types of splenectomy in children with congenital hemolytic anemias. We constructed key questions that addressed outcomes relevant to clinicians and families on effects of partial or total splenectomy, including hematologic effect, splenic function, and the risk of adverse events. We identified from Pubmed and Embase 703 studies that evaluated different types of splenectomy and accepted 93 studies that satisfied entry criteria. We graded the quality of each report and summarized the overall strength of research evidence for each key question. We did not identify any randomized clinical trials. All types of splenectomy have favorable clinical outcomes in most diseases. We did not identify any hematologic advantage of laparoscopy compared with laparotomy. Adverse events are uncommon in most studies and are minimized with use of laparoscopy. There is a need for randomized clinical trials and improved data collection of different types of splenectomy in congenital hemolytic anemias. Outcomes studied should address the concerns of families and clinicians to assess the risks and benefits of various treatments.

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