Megacystis-microcolon hyopoperistalsis syndrome is rare and lethal in most cases. In all cases, the enlarged bladder and absence of oligohydramnios typical of this syndrome were easy to define by ultrasound. The length of gestation and birth weight are within normal limits. There is no universally agreed upon explanation as yet for the etiology and pathogenesis. The diagnosis is simple, the treatment symptomatic, and the outcome fatal.
Most cases are sporadic.
The fibrosis would destroy the intestinal neural network, producing hypoperistalsis and causes neuromuscular incoordination of the bladder. The resulting bladder distension could interfere with the rotation of the intestine causing malrotation. Another theory suggests the urinary tract obstruction in this condition is secondary to the intestinal defect. It is not clear whether this is a primary neuropathy or myopathy 14 08D0C9EA79F9BACE118C8200AA004BA90B02000000080000000E0000005F005200650066003400350033003100340031003100340031000000 . At pathology, vacuolar degeneration of smooth muscle is observed.