Reconstructing the anal sphincters to reverse iatrogenic overstretching following a pull-through for Hirschsprung disease. One-year outcomes

Published:November 06, 2022DOI:


      • Soiling after Hirschsprung disease pull-through may be related to sphincter damage which currently has no optimal treatment.
      • A new technique of anal sphincter reconstruction showed improvement of bowel control in these patients at one year.
      • Non-sedate 3D-anorectal manometry showed improvement of squeeze function and ability to complete close the anal sphincters.
      • In patients without Down syndrome, improvement in Baylor Continence Scale was demonstrated (4.5 vs. 0.75).



      In patients with Hirschsprung disease (HSCR), soiling may be related to anal sphincter damage following the initial pull-through. No optimal treatment has been developed for such patients, although enemas (rectal or antegrade) have been applied with some success. We present the one-year outcomes of a new technique for anal sphincter reconstruction.


      All patients with HSCR referred from other institutions for post pull-through soiling were studied. Seven patients with patulous sphincters underwent sphincter reconstruction. Six had a full preoperative evaluation and were included in the study. Their 12-month outcomes were assessed.


      All six patients had soiling without voluntary bowel movements (VBMs). One patient was clean on Malone flushes when referred. Three underwent pre- and post-reconstruction non-sedated three-dimensional anorectal manometry, and objectively were able to close their sphincters following the reconstruction. All patients without Down syndrome (4 of 6) showed improvement in the abbreviated Baylor Continence Scale (4.5 vs. 0.75). One patient has achieved total bowel control without antegrade flushes, three now have VBMs which they did not have before but have occasional accidents and use antegrade flushes intermittently. They reported higher productivity, the ability to participate in sports and be away from home with confidence in their regimen. Two of 6 patients have Down syndrome and required a redo pull-through for other indications and underwent empiric sphincter reconstruction. For these two patients we do not have an outcomes assessment.


      A new technique for sphincter reconstruction shows promising results in improvement of bowel control at one year.

      Level of Evidence




      3D HR-ARM (three-dimensional high resolution anorectal manometry), ACE (antegrade continence enema), BCS (Baylor Continence Scale), BM (bowel movement), EUA (examination under anesthesia), HSCR (Hirschsprung disease), RAIR (rectoanal inhibitory reflex), SR (sphincter reconstruction), VBM (voluntary bowel movement)
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