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Pediatric: Hirschsprung's, Anorectal Malformations, and Other Conditions

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The ASCRS Textbook of Colon and Rectal Surgery

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Abstract

Hirschsprung’s disease (congenital megacolon) is an anomaly characterized by functional partial colonic obstruction caused by the absence of ganglion cells. It occurs in approximately 1 in 5000 births. Boys are more frequently affected than girls and it is more common in Caucasians. A deletion in the long arm of chromosome 10 has been found.1 The functional disturbances in this condition are attributed to the absence of ganglion cells from the Auerbach’s myenteric plexus (located between the circular and longitudinal layers of smooth muscle of the intestine), the Henle’s plexus (located in the submucosa), and the Meissner’s plexus (in the superficial submucosa). The absence of these cells probably produces uncoordinated contractions of the affected colon. This is translated into a lack of relaxation of the colon that results in partial colonic obstruction.

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Authors
  1. Alberto Peña
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  2. Marc Sher
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Editor information

Editors and Affiliations

  1. Professor of Surgery, Mayo Clinic College of Medicine, Chairman, Division of Colon and Rectal Surgery, Mayo Clinic Foundation, Rochester, MN, USA

    Bruce G. Wolff MD, FASCRS (Senior Editors) (Senior Editors)

  2. Professor of Surgery, Chief, Section of Colorectal Surgery, Washington University School of Medicine, St. Louis, MO, USA

    James W. Fleshman MD, FASCRS (Senior Editors) (Senior Editors)

  3. Chairman, Department of Colon and Rectal Surgery, Ochsner Clinic Foundation, New Orleans, LA, USA

    David E. Beck MD, FASCRS (Senior Editors) (Senior Editors)

  4. Professor of Surgery, Mayo Clinic College of Medicine, Consultant, Colon and Rectal Surgery, Mayo Clinic and Mayo Foundation, Rochester, MN, USA

    John H. Pemberton MD, FASCRS (Senior Editors) (Senior Editors)

  5. Chief of Staff, Cleveland Clinic Florida, Weston, FL, USA

    Steven D. Wexner MD, FASCRS (Senior Editors) (Senior Editors)

  6. Victor W. Fazio Chair of Colorectal Surgery, Department of Colorectal Surgery, Cleveland Clinic Foundation, Cleveland, OH, USA

    James M. Church BSc, MBChB, MMed Sci, FASCRS (Associate Editors) (Associate Editors)

  7. Professor of Surgery, Chief, Section of Colon and Rectal Surgery, Department of Surgery, University of California, San Francisco, San Francisco, CA, USA

    Julio Garcia-Aguilar MD, PhD, FASCRS (Associate Editors) (Associate Editors)

  8. Department of Colon and Rectal Surgery, Lahey Clinic, Burlington, MA, USA

    Patricia L. Roberts MD, FASCRS (Associate Editors) (Associate Editors)

  9. Professor of Surgery, Head, Section of Colon and Rectal Surgery, Rush University Medical Center, Chicago, IL, USA

    Theodore J. Saclarides MD, FASCRS (Associate Editors) (Associate Editors)

  10. Division of Colon and Rectal Surgery, Department of Surgery, University of California, Irvine Medical Center, Orange, CA, USA

    Michael J. Stamos MD, FASCRS (Associate Editors) (Associate Editors)

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Peña, A., Sher, M. (2007). Pediatric: Hirschsprung's, Anorectal Malformations, and Other Conditions. In: Wolff, B.G., et al. The ASCRS Textbook of Colon and Rectal Surgery. Springer, New York, NY. https://doi.org/10.1007/978-0-387-36374-5_51

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  • DOI: https://doi.org/10.1007/978-0-387-36374-5_51

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-24846-2

  • Online ISBN: 978-0-387-36374-5

  • eBook Packages: MedicineReference Module Medicine

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