Imaging findings in megacystis-microcolon-intestinal hypoperistalsis syndrome.

[megacystis-microcolon-intestinal hypoperistalsis syndrome]

Megacystis-microcolon -intestinal hypoperistalsis syndrome (MMIHS ) is a rare and frequently lethal form of severe functional intestinal obstruction more commonly found in girls . Imaging features characteristic of this disease include a large dilated bladder , microcolon and intestinal dysmotility . Additional imaging findings may include intestinal malrotation , hydronephrosis and vesicoureteral reflux . It is usually fatal in the first year of life . Because presenting clinical and imaging features can mimic other causes of neonatal bowel obstruction , we compiled examples of this disorder to help the pediatric radiologist recognize imaging findings associated with MMIHS and aid in the development of a long -term management plan and in counseling the family regarding implications of this disorder . We reviewed recent and historical literature relevant to MMIHS and present the imaging and clinical features of four patients with MMIHS treated at our institution as examples of this uncommon disorder .