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Review
. 2023 Aug 20;10(8):1418.
doi: 10.3390/children10081418.

State of the Art Bowel Management for Pediatric Colorectal Problems: Hirschsprung Disease

Elizaveta Bokova  1 , Ninad Prasade  1 , Sanjana Janumpally  1 , John M Rosen  2   3 , Irene Isabel P Lim  1   4 , Marc A Levitt  5 , Rebecca M Rentea  1   4
Affiliations
Review

State of the Art Bowel Management for Pediatric Colorectal Problems: Hirschsprung Disease

Elizaveta Bokova et al. Children (Basel). .

Abstract

After an initial pull-though, patients with Hirschsprung disease (HD) can present with obstructive symptoms, Hirschsprung-associated enterocolitis (HAEC), failure to thrive, or fecal soiling. This current review focuses on algorithms for evaluation and treatment in children with HD as a part of a manuscript series on updates in bowel management. In constipated patients, anatomic causes of obstruction should be excluded. Once anatomy is confirmed to be normal, laxatives, fiber, osmotic laxatives, or mechanical management can be utilized. Botulinum toxin injections are performed in all patients with HD before age five because of the nonrelaxing sphincters that they learn to overcome with increased age. Children with a patulous anus due to iatrogenic damage of the anal sphincters are offered sphincter reconstruction. Hypermotility is managed with antidiarrheals and small-volume enemas. Family education is crucial for the early detection of HAEC and for performing at-home rectal irrigations.

Keywords: Hirschsprung disease; botox; botulinum toxin; bowel management; constipation; enema; enterocolitis; fecal incontinence; irrigation; laxatives; obstruction; total colonic aganglionosis.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Management of obstructive symptoms following a pull-through. EUA—examination under anesthesia; H&P—history and physical examination.
Figure 2
Figure 2
Anatomic causes of obstruction revealed on a contrast enema or examination under anesthesia and requiring surgical correction.
Figure 3
Figure 3
Bowel management based on the patient’s ability to relax the anal sphincters and response to the bowel regimen. ACE—antegrade continence enema.
Figure 4
Figure 4
Management of postoperative soiling in patients with Hirschsprung disease. ACE—antegrade continence enema; EUA—examination under anesthesia; H&P—history and physical examination.
Figure 5
Figure 5
Contrast enema of a hypomotile (on the left) and hypermotile (on the right) colon. Reprinted from Ref. [55] with permission from Springer Nature.
Figure 6
Figure 6
Management of Hirschsprung-associated enterocolitis. BID—twice a day; ER—emergency room; HAEC—Hirschsprung-associated enterocolitis; PICU—pediatric intensive care unit; PO—per oral; WOCN—wound, ostomy, and continence nursing. Modified from Ref. [92] with permission from Springer Nature.
Figure 7
Figure 7
Technique of rectal (colonic) irrigations.
See this image and copyright information in PMC

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