Infra-anastomotic Innervation of Residual Aganglionic Distal Rectum After Pull-through Surgery for Hirschsprung Disease

Pediatr Dev Pathol. 2019 Oct;22(5):420-430. doi: 10.1177/1093526619837788. Epub 2019 Mar 27.

Abstract

Background: Descending neurons are important for intestinal reflex activities, including the recto-anal inhibitory reflex involved in normal defecation. Pull-through surgery for Hirschsprung disease results in the anastomosis of ganglionic bowel to native aganglionic rectum just superior to the internal anal sphincter, which potentially could allow for physiologically significant infra-anastomotic innervation.

Methods: The density and distribution of intramuscular neuronal nitric oxide synthase (nNOS)- and mucosal calretinin-immunoreactive nerves were evaluated proximal and distal to the anastomosis in redo resection specimens after pull-through surgery for Hirschsprung disease. The findings were compared with data collected from cadaveric controls with no history of dysmotility and the distal aganglionic segments of primary rectal resections from patients with Hirschsprung disease.

Results: Native aganglionic rectum of Hirschsprung patients lacks the normal lush intramuscular nNOS- and mucosal calretinin-immunoreactive nerves present in normal bowel. In post-pull-through resection specimens obtained more than 7 months after pull-through surgery, nNOS- and calretinin-positive innervation is at least partially restored for variable distances up to 10 to 12 mm inferior to the anastomosis, respectively.

Conclusions: Innervation of infra-anastomotic muscularis propria and mucosa in the aganglionic distal rectum occurs to a variable degree after pull-through surgery for Hirschsprung disease and may contribute to individual differences in postoperative obstructive symptoms. Strategies to enhance infra-anastomotic innervation may improve clinical outcome.

Keywords: Hirschsprung disease; anal sphincter; calretinin; innervation; nitric oxide synthase; pull-through; rectum.

MeSH terms

  • Adolescent
  • Anal Canal / innervation*
  • Child
  • Child, Preschool
  • Female
  • Hirschsprung Disease* / pathology
  • Hirschsprung Disease* / surgery
  • Humans
  • Intestinal Mucosa / innervation
  • Male
  • Rectum / innervation*
  • Young Adult