The enteric nervous system (ENS) controls gastrointestinal (GI) motility, and defects in ENS development underlie pediatric GI motility disorders. In disorders such as Hirschsprung’s disease (HSCR), pediatric intestinal pseudo-obstruction (PIPO), and intestinal neuronal dysplasia type B (INDB), ENS structure is altered with noted decreased neuronal density in HSCR and reports of increased neuronal density in PIPO and INDB. The developmental origin of these structural deficits is not fully understood. Here, we review the current understanding of ENS development and pediatric GI motility disorders incorporating new data on ENS structure. In particular, emerging evidence demonstrates that enteric neurons are patterned into circumferential stripes along the longitudinal axis of the intestine during mouse and human development. This novel understanding of ENS structure proposes new questions about the pathophysiology of pediatric GI motility disorders. If the ENS is organized into stripes, could the observed changes in enteric neuron density in HSCR, PIPO, and INDB represent differences in the distribution of enteric neuronal stripes? Here, we review mechanisms of striped patterning from other biological systems and propose how defects in striped ENS patterning could explain structural deficits observed in pediatric GI motility disorders.

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肠神经系统条纹模式与疾病：未经探索的病理生理学

肠神经系统 (ENS) 控制胃肠道 (GI) 运动，ENS 发育缺陷是小儿胃肠道运动障碍的基础。在先天性巨结肠 (HSCR)、小儿假性肠梗阻 (PIPO) 和 B 型肠神经元发育不良 (INDB) 等疾病中，ENS 结构发生改变，HSCR 中神经元密度显着降低，并且有报道称 PIPO 和 INDB 中神经元密度增加。这些结构性缺陷的发展根源尚不完全清楚。在这里，我们结合有关 ENS 结构的新数据回顾了目前对 ENS 发展和儿童胃肠道运动障碍的理解。特别是，新出现的证据表明，在小鼠和人类发育过程中，肠神经元沿着肠道纵轴形成环形条纹。这种对 ENS 结构的新颖理解提出了关于小儿胃肠道运动障碍的病理生理学的新问题。如果 ENS 被组织成条纹，那么在 HSCR、PIPO 和 INDB 中观察到的肠神经元密度变化是否可以代表肠神经元条纹分布的差异？在这里，我们回顾了其他生物系统的条纹图案机制，并提出条纹 ENS 图案的缺陷如何解释在儿科胃肠道运动障碍中观察到的结构缺陷。