Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS): report of a case with prolonged survival and literature review.

[megacystis-microcolon-intestinal hypoperistalsis syndrome]

Megacystis-microcolon -intestinal hypoperistalsis syndrome (MMIHS ) was first described in 1976 . A rare congenital autosomal recessive alteration that predominantly affects females (4 :1 ratio ), it is characterized by the presence of distended bladder (without distal urinary tract obstruction ), microcolon , and decreased or absent intestinal peristalsis . Inconsistent and non-specific histological changes affecting the bladder and intestinal smooth muscle , and intrinsic innervations , have been reported most frequently . MMIHS usually has a fatal prognosis in the first year of life ; nevertheless there are some case reports of longer survival . Here is presented the case report of a boy with a diagnosis of MMIHS who has achieved prolonged survival , followed by a review of the literature .