Enteric nervous system cell replacement therapy for Hirschsprung disease: beyond tissue-engineered intestine

Eur J Pediatr Surg. 2014 Jun;24(3):214-8. doi: 10.1055/s-0034-1376311. Epub 2014 May 28.


Hirschsprung disease (HD), a neurocristopathy characterized by failed migration of neural crest cells to the distal colon, requires surgical resection of the aganglionic segment. Advances in stem cell and regenerative medicine research have opened the possibility to treat HD less invasively using enteric nervous system (ENS) cell replacement therapy. This article reviews the progress to date of culturing and delivering ENS stem cells in various in vitro and in vivo models, as well as review the available evidence of functionality of the transplant-derived cells. Potential areas of future study are identified, and application of conditions other than HD is briefly discussed.

Publication types

  • Review

MeSH terms

  • Animals
  • Cell Culture Techniques
  • Cell Engineering*
  • Enteric Nervous System / cytology*
  • Enteric Nervous System / physiology
  • Forecasting
  • Hirschsprung Disease / therapy*
  • Humans
  • Intestines / innervation*
  • Nerve Regeneration
  • Stem Cell Transplantation* / trends

Supplementary concepts

  • Hirschsprung disease 1