ABSTRACT
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital and generally fatal cause of functional intestinal obstruction in the newborn. The main characteristics for this syndrome are abdominal distension caused by a massive enlarged nonobstructed urinary bladder, microcolon, and decreased or absent intestinal peristalsis. In the majority of MMIHS patients, histologic studies of the myenteric and submucosal plexuses of the bowel revealed normal ganglion cells. However, in some patients, decreased amounts of ganglion cells or hyperganglionosis together with giant ganglia were found. Recent reports have described prenatal magnetic resonance imaging (MRI) in patients with suspected genitourinary and gastrointestinal tract abnormalities following ultrasound examination. Radiological evaluation usually suggests the diagnosis of MMIHS. Plain abdominal films showed either dilated small bowel loops or a gasless abdomen with evident gastric bubble in the vast majority of 182 reviewed cases. Surgical findings and management vary throughout the publications, and exact surgical procedures are reported inconsistently.
