Partial splenectomy for hereditary spherocytosis: A multi-institutional review

Keely L. Buesing, Elisabeth T. Tracy, Colleen Kiernan, Aimee C. Pastor, Laura D. Cassidy, J. Paul Scott, Russell E. Ware, Andrew M. Davidoff, Frederick J. Rescorla, Jacob C. Langer, Henry E. Rice, Keith T. Oldham

Research output: Contribution to journalArticle

42 Scopus citations

Abstract

Background/Purpose: Partial splenectomy has emerged as a surgical option for selected children with hereditary spherocytosis, with the goal of reducing anemia while preserving splenic function. This multi-institutional study is the largest series to date examining outcomes data for partial splenectomy in patients with hereditary spherocytosis. Methods: Data were collected retrospectively from 5 North American pediatric hospitals. Sixty-two children underwent partial splenectomy for hereditary spherocytosis between 1990 and 2008. Results: At 1 year following partial splenectomy, mean hemoglobin significantly increased by 3.0 ± 1.4 g/dL (n = 52), reticulocyte count decreased by 6.6% ± 6.6% (n = 41), and bilirubin level decreased by 1.3 ± 0.9 mg/dL (n = 25). Patients with poor or transient hematologic response were found to have significantly more splenic regeneration postoperatively compared with patients with a durable clinical response (maximal spleen dimension, 9.0 ± 3.4 vs 6.3 ± 2.2 cm). Clinically significant recurrence of anemia or abdominal pain led to completion splenectomy in 4.84% of patients. No patients developed postsplenectomy sepsis. Conclusions: Our multi-institutional review indicates that partial splenectomy for hereditary spherocytosis leads to sustained and clinically significant improvement in hematologic profiles and clinical symptoms in most patients. Our data support partial splenectomy as an alternative for selected children with hereditary spherocytosis.

Original languageEnglish (US)
Pages (from-to)178-183
Number of pages6
JournalJournal of pediatric surgery
Volume46
Issue number1
DOIs
StatePublished - Jan 2011

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Keywords

  • Hereditary spherocytosis
  • Multi-institutional review
  • Outcomes data
  • Partial splenectomy

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

Buesing, K. L., Tracy, E. T., Kiernan, C., Pastor, A. C., Cassidy, L. D., Scott, J. P., Ware, R. E., Davidoff, A. M., Rescorla, F. J., Langer, J. C., Rice, H. E., & Oldham, K. T. (2011). Partial splenectomy for hereditary spherocytosis: A multi-institutional review. Journal of pediatric surgery, 46(1), 178-183. https://doi.org/10.1016/j.jpedsurg.2010.09.090