Dyssynergic defecation may play an important role in postoperative Hirschsprung's disease patients with severe persistent constipation: analysis of a case series

J Pediatr Surg. 2014 Oct;49(10):1488-92. doi: 10.1016/j.jpedsurg.2014.05.001. Epub 2014 Jul 8.

Abstract

Background: After surgery for Hirschsprung's disease (HD) the majority of patients have satisfactory clinical outcomes. Nevertheless, a substantial number of patients remain who suffer from severe persistent constipation. Current consensus attributes these complaints to the hallmarks of HD. In non-HD patients a cause for severe constipation is dyssynergic defecation.

Methods: Retrospectively, we reviewed the medical records of ten postoperative HD patients with severe persistent constipation who had undergone extensive anorectal function tests to diagnose the reason for the constipation. We analyzed the results of these tests.

Results: During the last three years, ten postoperative HD patients with severe persistent constipation were given extensive anorectal function tests. All ten patients were diagnosed with dyssynergic defecation. The ages at the time of diagnosis ranged from 7 to 19years with a median age of 12years. Signs of an enlarged rectum were seen in all ten patients, with a maximum measured value of 845mL.

Conclusions: Patients with HD may also suffer from dyssynergic defecation. It is important to consider this possibility when dealing with severe persistent constipation in postoperative HD patients. Viable options for treating dyssynergic defecation are available that could prevent irreversible long-term complications.

Keywords: Anismus; Congenital megacolon; Dyssynergic defecation; Hirschsprung's disease; Pelvic floor dyssynergia.

MeSH terms

  • Adolescent
  • Anal Canal / physiopathology
  • Ataxia / etiology
  • Ataxia / physiopathology*
  • Child
  • Constipation / etiology
  • Constipation / physiopathology*
  • Defecation / physiology
  • Defecography
  • Female
  • Hirschsprung Disease / complications*
  • Hirschsprung Disease / surgery
  • Humans
  • Male
  • Manometry
  • Rectum / physiopathology
  • Retrospective Studies
  • Young Adult