Megacystis-microcolon-intestinal hypoperistalsis syndrome. Antenatal appearance in two cases.

[megacystis-microcolon-intestinal hypoperistalsis syndrome]

Two cases with severe congenital megacystis-microcolon -intestinal hypoperistalsis syndrome (MMIHS ) are presented . This is a rarely encountered syndrome in neonates and 45 cases have earlier been reported . The disease is usually lethal and it now seems clear that MMIHS is an autosomal recessive disorder . The enlarged bladder , typical of this syndrome , is however easy to define by ultrasound , sometimes even in early pregnancy . The concomitant finding of a dilatation of the urinary tract and the absence of oligohydramnios may lead the physician to suspect the diagnosis . Because of the information available from sonography , appropriate investigations can be undertaken immediately after delivery . Prenatal ultrasound examination in subsequent pregnancies is recommended .