The shared patient was a 7-year-old who underwent multiorgan (liver, small bowel, and pancreas) transplantation because of megacystis microcolon intestinal hypoperistalsis
syndrome, a rare congenital condition characterized by a largely dilated nonobstructed urinary bladder (megacystis), very small colon (microcolon), and decreased or absent intestinal movements (intestinal peristalsis).
Congenital mydriasis associated with megacystis microcolon intestinal hypoperistalsis
Hypoperistalsis or adynamic ileus is frequently seen in the postoperative period and may persist for several weeks.
Outcomes are affected cholestasis secondary to long-term TPN, malrotation, midgut volvulus, hypoperistalsis, Gastroesophageal Reflux (GER) and aspiration pneumonia.
Diagnosed with an incredibly rare digestive condition called megacystis microcolon intestinal hypoperistalsis
syndrome, little Delilah could not eat or drink normally and was unable to pass waste through her body.
Participants reported other symptom experiences such as visual and auditory disturbances, muscle spasms, headaches, infections, numbness, insomnia, and hypoperistalsis
, but there were no discernible patterns or correlations in the data.
Of the foetuses with Hydronephrosis, 3 had Posterior urethral valve, one as a part of Megacystis Microcolon Hypoperistalsis
INTRODUCTION-Megacystis-microcolon-intestinal hypoperistalsis syndrome consists of distended unobstructed bladder, dilated small bowel, micro colon, and decreased or absent peristalsis with presence ganglion cells.
DISCUSSION- Megacystis-microcolon hypoperistalsis syndrome is rare and lethal in most cases.
In mice a deficiency in alpha3 subunit of the neuronal nicotinic acetylcholine receptor (a mediator of normal function of the autonomic nervous system) results in similar symptoms to those of megacystismicrocolon-intestinal hypoperistalsis .
On USG megacystis-microcolon-intestinal hypoperistalsis syndrome should be considered in the presence of an abnormally distended bladder with a normal or increased amount of amniotic fluid.
Megacystis-microcolon-intestinal hypoperistalsis syndrome must be distinguished from isolated "prune belly" caused by urethral obstruction.