Embryology and anatomy of Hirschsprung disease

摘要

Bowel has its own elegant nervous system - the enteric nervous system (ENS) which is a complex net-work of neurons and glial clones. Derived from neural crest cells (NCCs), this little brain controls muscle contraction, motility, and bowel activities in response to stimuli. Failure of developing enteric ganglia at the distal bowel results in intestinal obstruction and Hirschsprung disease (HSCR). This Review sum-marises the important embryological development of the ENS including proliferation, migration, and dif-ferentiation of NCCs. We address the signalling pathways which determine NCC cell fate and discuss how they are altered in the context of HSCR. Finally, we outline the anatomical defects and the mechanisms underlying gut motility in HSCR.(c) 2022 Elsevier Inc. All rights reserved.