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Clinical and Hematologic Benefits of Partial Splenectomy for Congenital Hemolytic Anemias in Children

Rice, Henry E. MD*; Oldham, Keith T. MD‡; Hillery, Cheryl A. MD§; Skinner, Michael A. MD*; O’Hara, Sara M. MD¶; Ware, Russell E. MD, PhD†

Original Articles

Objective: To assess the role of partial splenectomy for symptomatic children with various congenital hemolytic anemias.

Summary Background Data: The use of total splenectomy for symptomatic children with congenital hemolytic anemias is restricted by concern of postsplenectomy sepsis. A partial splenectomy is an alternative procedure, although its utility remains incompletely defined.

Methods: This longitudinal cohort study followed 25 symptomatic children with various congenital anemias who underwent partial splenectomy. Sixteen children had hereditary spherocytosis (HS), and nine children had other erythrocyte disorders. Outcome measures were clinical and laboratory hemolysis, splenic phagocytic and immune function, and splenic regrowth as measured by ultrasonography. Discrete parameters were compared using the Student t test.

Results: Partial splenectomy was successful in all 25 children, with minimal morbidity. Follow-up ranged from 7 months to 6 years (mean 2.3 ± 1.5 years). Following surgery, children with HS had increased hemoglobin values, decreased reticulocyte and bilirubin levels, and preserved splenic function. Most children without HS had decreased symptoms of hypersplenism and splenic sequestration. Over time, variable rates of splenic regrowth were noted, although regrowth did not necessarily correlate with recurrent hemolysis.

Conclusions: In children with hereditary spherocytosis, a partial splenectomy appears to control hemolysis while retaining splenic function. In children with other congenital hemolytic anemias, a partial splenectomy appears to control symptoms of hypersplenism and splenic sequestration.

Congenital hemolytic anemias frequently lead to severe hemolysis due to splenic sequestration of abnormal erythrocytes. 1 For children with hereditary spherocytosis (HS), a total splenectomy eliminates the main source of erythrocyte destruction. For children with other hemolytic anemias, a splenectomy eliminates splenic sequestration and reduces symptoms of hypersplenism.

The use of total splenectomy in children is restricted by concern of overwhelming postsplenectomy sepsis. 2–5 In children less than 5 years of age, the risk of overwhelming postsplenectomy sepsis may be increased 60- to 100-fold compared to children who have not had a splenectomy. 4 Although the risk of overwhelming postsplenectomy sepsis is reduced by use of immunizations to Streptococcus pneumoniae, Meningococcus, and Haemophilus influenzae as well as postoperative antibiotic prophylaxis, its risk is never eliminated. 5 Moreover, concerns persist of incomplete protection by pneumococcal vaccinations, antibiotic resistance, and poor compliance with antibiotic prophylaxis. 5,6

For children with congenital hemolytic anemias, a partial splenectomy has been proposed as an alternative to total splenectomy, with the goal of removing enough spleen to gain a desired hematologic effect while preserving splenic immune function. 7–10 However, the use of partial splenectomy is limited because of technical difficulties and concerns of splenic regrowth. 7,8,11,12 Recently several groups have renewed an interest in partial splenectomy. 7–10

To assess the role of partial splenectomy in symptomatic children with various congenital hemolytic anemias, we have followed 25 children with various congenital hemolytic anemias who underwent partial splenectomy. We examined the effects of partial splenectomy on hemolysis, splenic phagocytic and immune function, and splenic regrowth. Our results suggest that partial splenectomy may be beneficial for a variety of congenital hemolytic anemias.

From the Departments of *Surgery and

†Pediatrics, Duke University Medical Center, Durham, North Carolina; Departments of

‡Pediatric Surgery and

§Pediatrics, Children’s Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin; and

¶Department of Radiology, Children’s Medical Center of Cincinnati, Cincinnati, Ohio

Correspondence: Henry E. Rice, MD, Division of Pediatric Surgery, Box 3815, Duke University Medical Center, Durham, NC, 27710.

E-mail: rice0017@mc.duke.edu

Accepted for publication June 13, 2002.

© 2003 Lippincott Williams & Wilkins, Inc.