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Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease

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Abstract

Although most children with Hirschsprung disease ultimately do well, many experience a variety of ongoing problems after pull-through surgery. The most common include obstructive symptoms, soiling, enterocolitis and failure to thrive. The purpose of this guideline is to present a rational approach to the management of postoperative obstructive symptoms in children with Hirschsprung disease. The American Pediatric Surgical Association Board of Governors established a Hirschsprung Disease Interest Group. Group discussions, literature review and expert consensus were then used to summarize the current state of knowledge regarding causes, methods of diagnosis, and treatment approaches to children with obstructive symptoms following pull-through for Hirschsprung disease. Causes of obstructive symptoms post-pull-through include mechanical obstruction; persistent or acquired aganglionosis, hypoganglionosis, or transition zone pull-through; internal sphincter achalasia; disordered motility in the proximal intestine that contains ganglion cells; or functional megacolon caused by stool-holding behavior. An algorithm for the diagnosis and management of obstructive symptoms after a pull-through for Hirschsprung disease is presented. A stepwise, logical approach to the diagnosis and management of patients experiencing obstructive symptoms following pull-through for Hirschsprung disease can facilitate treatment.

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Authors and Affiliations

  1. Division of General and Thoracic Surgery, Department of Surgery, Hospital for Sick Children, University of Toronto, Toronto, Canada

    J. C. Langer

  2. Primary Children’s Hospital, University of Utah School of Medicine, Salt Lake City, UT, USA

    M. D. Rollins

  3. Department of Pediatric Surgery, Center for Colorectal and Pelvic Reconstruction, Nationwide Children’s Hospital, Columbus, OH, USA

    M. Levitt

  4. Division of Pediatric Surgery, Department of Surgery, Le Bonheur Children’s Hospital, Children’s Foundation Research Institute, University of Tennessee Health Science Center, Memphis, TN, USA

    A. Gosain

  5. Colorectal Center for Children at Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA

    L. de la Torre

  6. Department of Laboratories, Seattle Children’s Hospital and University of Washington, Seattle, WA, USA

    R. P. Kapur

  7. Section of Pediatric Surgery, Department of Surgery, Yale University School of Medicine, New Haven, CT, USA

    R. A. Cowles

  8. Madigan Army Medical Center, Tacoma, WA, USA

    J. Horton

  9. Department of Pediatric Surgery, Women and Children’s Hospital of Buffalo, Buffalo, NY, USA

    D. H. Rothstein

  10. Department of Pediatric Surgery, Harvard Medical School, Massachusetts General Hospital, Boston, MA, USA

    A. M. Goldstein

  11. Division of Pediatric General and Thoracic Surgery, Hospital for Sick Children, Rm 1524, 555 University Ave, Toronto, ON, M5G 1X8, Canada

    J. C. Langer

Authors
  1. J. C. Langer
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  2. M. D. Rollins
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  3. M. Levitt
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  4. A. Gosain
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  5. L. de la Torre
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  6. R. P. Kapur
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  7. R. A. Cowles
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  8. J. Horton
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  9. D. H. Rothstein
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  10. A. M. Goldstein
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Consortia

On behalf of the American Pediatric Surgical Association Hirschsprung Disease Interest Group

Corresponding author

Correspondence to J. C. Langer.

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Langer, J.C., Rollins, M.D., Levitt, M. et al. Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int 33, 523–526 (2017). https://doi.org/10.1007/s00383-017-4066-7

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  • DOI: https://doi.org/10.1007/s00383-017-4066-7

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