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Hirschsprung’s Disease

  • Alberto Peña
  • Andrea Bischoff

Abstract

This chapter is a review of the publications that illustrate the history of this condition, beginning with the presentation of Harald Hirschsprung and continuing with the different concepts related with the etiology of the disease until the classic description of the first rational surgical technique employed for the repair of this condition by Dr. Swenson. The chapter also includes an analysis of the different types of procedures proposed by different authors through time until the present.

A brief discussion on the abundant literature related with the pathogenesis of this condition. This enormous amount of literature reflects the interest awakened by this condition in different researchers. The retrospective analysis of the different theories and developments related with the pathogenesis of this disease illustrates the trajectory and future trends followed by research-oriented pediatric surgeons and scientists.

Since the diagnosis of Hirschsprung’s disease is based fundamentally on the histologic abnormalities of the colon, different types of biopsies and histopathological techniques represent a source of controversy and extensive discussions between different authors. In this chapter, the authors try to make simple a subject that is becoming more and more complex based on recent advances in histological methodology. Since the book is focused on pragmatic solutions for clinical problems, the authors present a simplified way to deal with this overwhelming, and at times rather confusing, literature on histologic findings in Hirschsprung’s disease.

The authors present important diagnostic tools and clinical manifestations to make an early diagnosis and adequate initial management. Special space is dedicated to the treatment of enterocolitis, which is the most dangerous manifestation of this disease. The indications for colostomy as well as contraindications are also discussed.

The treatment of Hirschsprung’s is well illustrated with photographic material and diagrams of the preferred surgical technique used by the authors, which is the transanal approach. Special emphasis is placed on how to prevent damage to the anal canal to avoid the most feared sequelae in Hirschsprung’s disease which is fecal incontinence.

The authors discuss the most common indications for a reoperation in Hirschsprung’s disease, including: resection of a remaining aganglionic segment and resection of a rectal pouch in cases operated with a Duhamel technique. It also deals with the surgical treatment of sequelae from catastrophic operative events, such as abscess, recto-urinary or vaginal fistula, retraction, and stenosis.

The authors discuss the so-called “ultrashort Hirschsprung’s,” which is a very controversial subject. The authors present a critical analysis of the publications related with conditions such as “achalasia of the internal sphincter” and “achalasia of the puborectalis.” An attempt to simplify the subject is offered, as well as a practical way to take care of these patients.

Total colonic aganglionosis is a very serious condition that we are not really curing, since most of those patients will have symptoms for life. This chapter shows a description of what the authors think is important in the treatment of this condition to give these patients the best possible quality of life. The importance of removing the aganglionic colon as early as possible is emphasized because leaving the colon in place may produce important symptoms to the patient. The pull-through can be done any time provided the surgeon has experience in this surgical technique, but special emphasis is placed on avoiding the closure of the protective ileostomy until the patient is toilet trained for urine and accepts rectal irrigations. Closing an ileostomy in an infant without colon provokes one of the most severe diaper rashes that interfere with the quality of life of the patient.

Keywords

Fecal Incontinence Anal Canal Anorectal Malformation Contrast Enema Transanal Approach 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

Animation 24.1

Animation showing a rectal irrigation (WMV 10784 kb)

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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Alberto Peña
    • 1
  • Andrea Bischoff
    • 1
  1. 1.Pediatric SurgeryColorectal Center for Children Cincinnati Children’s HospitalCincinnatiUSA

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