Megacystis Microcolon Intestinal Hypoperistalsis Syndrome

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a congenital disease distinguished by a markedly dilated acontractile bladder and a microcolon with dysfunctional peristalsis in the absence of mechanical obstruction. While the exact pathophysiology of MMIHS is not yet fully understood, the literature suggests a combination of genetic, neurogenic, and myogenic causes contribute to the disease. Most patients are diagnosed in utero or as neonates. Fetal US often suggests MMIHS. However, the diagnosis is often confirmed on fetal MRI. Advances in total parenteral nutrition, intestinal rehabilitation, and intestinal transplant have helped improve patients’ survival.