Hematologic outcomes after total splenectomy and partial splenectomy for congenital hemolytic anemia

Abstract

Purpose The purpose of this study was to define the hematologic response to total splenectomy (TS) or partial splenectomy (PS) in children with hereditary spherocytosis (HS) or sickle cell disease (SCD). Methods The Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium registry collected hematologic outcomes of children with CHA undergoing TS or PS to 1 year after surgery. Using random effects mixed modeling, we evaluated the association of operative type with change in hemoglobin, reticulocyte counts, and bilirubin. We also compared laparoscopic to open splenectomy. Results The analysis included 130 children, with 62.3% (n = 81) undergoing TS. For children with HS, all hematologic measures improved after TS, including a 4.1 g/dl increase in hemoglobin. Hematologic parameters also improved after PS, although the response was less robust (hemoglobin increase 2.4 g/dl, p < 0.001). For children with SCD, there was no change in hemoglobin. Laparoscopy was not associated with differences in hematologic outcomes compared to open. TS and laparoscopy were associated with shorter length of stay. Conclusion Children with HS have an excellent hematologic response after TS or PS, although the hematologic response is more robust following TS. Children with SCD have smaller changes in their hematologic parameters. These data offer guidance to families and clinicians considering TS or PS.

Laparoscopic Splenectomy Alone for Sickle Cell Disease: Account of 50 paediatric cases

“…In the operating theatre, patients were placed in a semi-decubitus position with their left side elevated. 3,5,14 A total of 25 mg/kg of cefuroxime was administered as an induction dose. The positioning of the ports during the splenectomy is shown in Figure 1.…”

“…All patients underwent total splenectomies as there is currently a lack of evidence regarding the benefits of partial splenectomies in improving SCD haematological parameters; moreover, partial splenectomies are associated with greater intraoperative blood loss and higher rates of conversion to open surgery. 14,15 Postoperatively, patients were managed by a physician specialising in paediatric haematology. One month after discharge, the patients were followed-up in the paediatric surgery outpatient department.…”

“…3 The dissection of the hilar vessels is an important aspect of a laparoscopic splenectomy; to this end, various dissections tools are available, including linear staplers and clipping and harmonic devices. 1,3,4,14 Although it shortens the operative time, en bloc stapling of the hilum can increase the risk of the patient developing arteriovenous fistulae, injuries to the pancreatic tail and splenic or portal vein thrombosis. 4 In a small study of 17 patients, Vargün et al established a one-year follow-up as a safe period of time for evaluation following en bloc stapling; however, this procedure has not yet been adopted at SQUH.…”

“…However, the superiority of a laparoscopic approach over an open technique has been previously wellestablished. [1][2][3]12,14 Nevertheless, this study presents only the results of laparoscopic surgeries for patients with a single haematological condition; hence, the results should be interpreted carefully. Studies documenting the quality of life of SCD patients and…”

Understanding splenectomy for children with hereditary spherocytosis

“…As the data in this area are mostly of an observational nature and limited to small case series, this is an entirely appropriate tool to summarize the existing literature in this area and communicate it in a manner that is understandable for readers . Most of the research in this area remains limited to several groups, including the leading work by Tchernia in France, Stoehr in Germany, and our own Splenectomy in Children With Congenital Hemolytic Anemia (SICHA) consortium in the United States …”