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Key Points

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  1. Diagnoses are typically made with suction rectal biopsy, however, when a full-thickness biopsy is needed, it is important to carefully determine your distance from the dentate line. This insures an accurate ruling in or out of Hirschsprung disease.

  2. A colostomy may be needed in those with severe enterocolitis or those with a delayed diagnosis and very dilated colon. In those cases, a laparoscopic inspection of the abdomen can often facilitate the identification of the transition zone, and placement of the initial incision (see later in chapter for laparoscopic leveling biopsies).

  3. The decision to perform an open versus laparoscopic-assisted or transanal pull-through probably does not affect the long-term results of the infant. Thus, the approach that the surgeon is most familiar with should be selected.

  4. For total colonic Hirschsprung disease either a standard Duhamel or endorectal pull-through is acceptable, and associated with similar outcomes.

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Introduction

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The first therapeutic approach to Hirschsprung disease was described by Harold Hirschsprung, himself. He performed a diverting colostomy in one of the first children he recognized with the disease itself. Over the past 100 years, tremendous advancements in the approach to this disease process have occurred. Figure 44-1 summarizes in a diagrammatic fashion some of the more commonly performed procedures in the historical development of the surgical correction of Hirschsprung disease. While many of these techniques have been retained, the dominate approach in the last decade has been the endorectal pull-through and the Duhamel approach. The chapter will thus concentrate on these operations.

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Figure 44-1

Schematic illustrations of several operative pull-through techniques for the treatment of Hirschsprung disease.

Graphic Jump Location
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Diagnosis

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Rectal Biopsy

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Indications
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The suction rectal biopsy has become the gold standard for the diagnosis of Hirschsprung disease. However, in some patients, particularly those over 4 years of age, the thick mucosal layer prevents the biopsy of sufficient submucosal tissue to make a definitive diagnosis of aganglionosis. Although it is worth attempting a suction rectal biopsy first in such patients, the diagnostic yield decreases with increased age. In other cases, a full-thickness biopsy is indicated for the child in whom there has been more than 1 indeterminate suction rectal biopsy. Although one would think that performing a full-thickness biopsy may preclude or increase the difficulty of an endorectal pull-through, the author has not found this to be the case.

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Preoperative Preparation
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No formal bowel prep is required, as this often causes excess loose stool to run into the operative field. The child's rectum is irrigated with saline at the beginning of the procedure, followed by the placement of a sponge into the proximal rectal vault to prevent stool from entering the operative field. A single dose of preoperative broad-spectrum cephalosporin is given.

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