Partial splenectomy for hereditary spherocytosis: a multi-institutional review.

Published

Journal Article

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.

Full Text

Duke Authors

Cited Authors

  • Buesing, KL; Tracy, ET; Kiernan, C; Pastor, AC; Cassidy, LD; Scott, JP; Ware, RE; Davidoff, AM; Rescorla, FJ; Langer, JC; Rice, HE; Oldham, KT

Published Date

  • January 2011

Published In

Volume / Issue

  • 46 / 1

Start / End Page

  • 178 - 183

PubMed ID

  • 21238662

Pubmed Central ID

  • 21238662

Electronic International Standard Serial Number (EISSN)

  • 1531-5037

Digital Object Identifier (DOI)

  • 10.1016/j.jpedsurg.2010.09.090

Language

  • eng

Conference Location

  • United States