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 Rescorla, Jacob C. Langer, Henry E. Rice, Keith T. Oldham

Research output: Contribution to journalArticle

38 Citations (Scopus)

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
Pages (from-to)178-182
Number of pages5
JournalJournal of Pediatric Surgery
Volume46
Issue number1
DOIs
StatePublished - Jan 2011

Fingerprint

Hereditary Spherocytosis
Splenectomy
Anemia
Reticulocyte Count
Pediatric Hospitals
Bilirubin
Abdominal Pain
Regeneration
Sepsis
Hemoglobins
Spleen
Recurrence

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., ... Oldham, K. T. (2011). Partial splenectomy for hereditary spherocytosis: A multi-institutional review. Journal of Pediatric Surgery, 46(1), 178-182. https://doi.org/10.1016/j.jpedsurg.2010.09.090

Partial splenectomy for hereditary spherocytosis : A multi-institutional review. / Buesing, Keely L.; Tracy, Elisabeth T.; Kiernan, Colleen; Pastor, Aimee C.; Cassidy, Laura D.; Scott, J. Paul; Ware, Russell E.; Davidoff, Andrew M.; Rescorla, Frederick; Langer, Jacob C.; Rice, Henry E.; Oldham, Keith T.

In: Journal of Pediatric Surgery, Vol. 46, No. 1, 01.2011, p. 178-182.

Research output: Contribution to journalArticle

Buesing, KL, Tracy, ET, Kiernan, C, Pastor, AC, Cassidy, LD, Scott, JP, Ware, RE, Davidoff, AM, Rescorla, F, Langer, JC, Rice, HE & Oldham, KT 2011, 'Partial splenectomy for hereditary spherocytosis: A multi-institutional review', Journal of Pediatric Surgery, vol. 46, no. 1, pp. 178-182. https://doi.org/10.1016/j.jpedsurg.2010.09.090
Buesing, Keely L. ; Tracy, Elisabeth T. ; Kiernan, Colleen ; Pastor, Aimee C. ; Cassidy, Laura D. ; Scott, J. Paul ; Ware, Russell E. ; Davidoff, Andrew M. ; Rescorla, Frederick ; Langer, Jacob C. ; Rice, Henry E. ; Oldham, Keith T. / Partial splenectomy for hereditary spherocytosis : A multi-institutional review. In: Journal of Pediatric Surgery. 2011 ; Vol. 46, No. 1. pp. 178-182.
@article{9f2fa02340b04078b9d60aa4f383ca0d,
title = "Partial splenectomy for hereditary spherocytosis: A multi-institutional review",
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.",
keywords = "Hereditary spherocytosis, Multi-institutional review, Outcomes data, Partial splenectomy",
author = "Buesing, {Keely L.} and Tracy, {Elisabeth T.} and Colleen Kiernan and Pastor, {Aimee C.} and Cassidy, {Laura D.} and Scott, {J. Paul} and Ware, {Russell E.} and Davidoff, {Andrew M.} and Frederick Rescorla and Langer, {Jacob C.} and Rice, {Henry E.} and Oldham, {Keith T.}",
year = "2011",
month = "1",
doi = "10.1016/j.jpedsurg.2010.09.090",
language = "English",
volume = "46",
pages = "178--182",
journal = "Journal of Pediatric Surgery",
issn = "0022-3468",
publisher = "W.B. Saunders Ltd",
number = "1",

}

TY - JOUR

T1 - Partial splenectomy for hereditary spherocytosis

T2 - A multi-institutional review

AU - Buesing, Keely L.

AU - Tracy, Elisabeth T.

AU - Kiernan, Colleen

AU - Pastor, Aimee C.

AU - Cassidy, Laura D.

AU - Scott, J. Paul

AU - Ware, Russell E.

AU - Davidoff, Andrew M.

AU - Rescorla, Frederick

AU - Langer, Jacob C.

AU - Rice, Henry E.

AU - Oldham, Keith T.

PY - 2011/1

Y1 - 2011/1

N2 - 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.

AB - 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.

KW - Hereditary spherocytosis

KW - Multi-institutional review

KW - Outcomes data

KW - Partial splenectomy

UR - http://www.scopus.com/inward/record.url?scp=78751481305&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=78751481305&partnerID=8YFLogxK

U2 - 10.1016/j.jpedsurg.2010.09.090

DO - 10.1016/j.jpedsurg.2010.09.090

M3 - Article

VL - 46

SP - 178

EP - 182

JO - Journal of Pediatric Surgery

JF - Journal of Pediatric Surgery

SN - 0022-3468

IS - 1

ER -