Autonomic innervation of the intestine from a baby with megacystis microcolon intestinal hypoperistalsis syndrome: I. Immunohistochemical study

Sonographic findings in a fetus with megacystis-microcolon–intestinal hypoperistalsis syndrome

“…It is also not clear whether MMIHS is primarily a neuropathy or a myopathy. 17 The hypoperistalsis has been attributed to visceral myopathy, 6 dysganglionosis, 18,19 an imbalance of gut peptides, 20 functional disturbances in inhibitory autonomic innervation, 21 axonal dystrophy, 22 and an endorgan receptor defect confined to the smooth muscle. 13 In addition, Srikanth et al 10 proposed that an in utero intramural inflammatory process causes hollow viscus smooth muscle and the neural network to be destroyed, leading to fibrosis of the bowel and bladder wall.…”

Interstitial cells of Cajal in the normal gut and in intestinal motility disorders of childhood

“…This syndrome is characterised by abdominal distension caused by a distended non-obstructed urinary bladder, microcolon, and decreased or absent intestinal peristalsis. Usually incomplete intestinal rotation and shortened small bowel are associated [167][168][169][170][171][172][173][174][175][176][177][178].…”

“…Several hypotheses have been proposed to explain the pathogenesis of MMIHS: genetic [173], neurogenic [172,175], myogenic [167,169,174] and hormonal origin [175,176]. Histological studies of the myenteric and submucosal plexuses of the bowel of MMIHS patients have revealed normal ganglion cells in the majority of the patients, decreased in some, hyperganglionosis and giant ganglia in others [168][169][170][171][172].…”

Isolated congenital megacystis without intestinal obstruction: a mild variant of chronic intestinal pseudoobstruction syndrome?

“…The pathogenesis is varied with possible associations with an abnormal autonomic nervous system [10,11,12,13], degenerative muscle disease [14], imbalance of gut peptides [15], and dysganglinosis or inflammation [16]. Histological studies of the myenteric and submucosal plexuses of the bowel of patients with this syndrome have found normal ganglion cells in the majority of patients, decreased in some, hyperganglionosis and giant ganglia in others [17].…”