Megacystis microcolon intestinal hypoperistalsis syndrome: late sequelae and possible pathogenesis.

[megacystis-microcolon-intestinal hypoperistalsis syndrome]

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS ) is characterized by hypoperistalsis in the presence of ganglion cells , malrotation , microcolon , bladder distension , and female predilection . We draw attention to the long -term management of a patient with MMIHS and propose a pathogenetic mechanism to account for this syndrome . We propose that the initial event in the pathogenesis of MMIHS is an intramural inflammatory process that affects the gastrointestinal and urinary tracts . This leads to extensive fibrosis which destroys the intestinal neural network , producing hypoperistalsis . The same process causes neuromuscular incoordination in the bladder wall , resulting in irregular bladder contractions against a "closed sphincter " leading to bladder distension . The enlarged bladder then interferes with the rotation of the intestine causing malrotation .