Pediatric Neurogastroenterology

Part of the series Clinical Gastroenterology pp 9-21


Development of the Enteric Neuromuscular System

  • Tiffany A. HeanueAffiliated withDivision of Molecular Neurobiology, National Institute for Medical Research
  • , Alan J. BurnsAffiliated withNeural Development Unit, UCL Institute of Child Health Email author 

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In order for the gut to perform essential functions, including moving contents along its length via the organized movement of peristalsis, as well as absorption of water and electrolytes, secretion from glands, and regulation of blood flow, the integrated function of multiple tissues and cell types must occur. The mature, functioning neuromuscular system of the gut is composed of smooth muscle cells, neurons and glial cells of the enteric nervous system (ENS), and interstitial cells of Cajal (ICC). These diverse components arise from distinct sources during development, and must, during the course of embryogenesis, acquire appropriate integration to enable a functioning neuromuscular system to commence coordinated activity around birth. Here, we utilize information gleaned from studies in animal models such as mouse, chick, guinea pig, and zebrafish, as well as human studies, to describe the development of each constituent part of the neuromuscular system as well as to outline how these component parts become integrated into a functioning whole. Moreover, our discussions touch on diseases affecting development of the enteric neuromuscular system, notably Hirschsprung’s disease (HSCR), one of the most common gut motility disorders.