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Partial splenectomy for hereditary spherocytosis: a multi-institutional review. J Pediatr Surg 2011 Jan;46(1):178-83 PMID: 21238662

Pubmed ID

21238662

DOI

10.1016/j.jpedsurg.2010.09.090

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.

Author List

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

Authors

Laura Cassidy PhD Professor in the Institute for Health and Equity department at Medical College of Wisconsin
Keith T. Oldham MD Professor in the Surgery department at Medical College of Wisconsin
John Paul Scott MD Professor in the Pediatrics department at Medical College of Wisconsin




Scopus

2-s2.0-78751481305   36 Citations

MESH terms used to index this publication - Major topics in bold

Adolescent
Bilirubin
Child
Child, Preschool
Female
Follow-Up Studies
Hospitals, Pediatric
Humans
Infant
Male
Regeneration
Reticulocyte Count
Spherocytosis, Hereditary
Spleen
Splenectomy
Treatment Outcome
jenkins-FCD Prod-332 f92a19b0ec5e8e1eff783fac390ec127e367c2b5