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Two newborn infants with megacystis-microcolon-intestinal hypoperistalsis syndrome
are described. Review of the literature revealed twenty previously reported cases
of this syndrome. Electron microscopic examination of the ileum and urinary bladder
showed vacuolar degenerative changes in the smooth muscle cells with abundant amount
of connective tissue between the muscle cells. These ultrastructural findings suggest
that a degenerative disease of smooth muscle may be the cause of megacystis microcolon
intestinal hypoperistalsis syndrome.