Unusual cause of neonatal intestinal obstruction

Abstract

There are many causes of intestinal obstruction in the neonatal age. The most common types are mechanical and result from congenital malformations of the gastrointestinal tract. However, functional disorders also occur. In some cases, diagnosis can be made prenatally but in others manifestation occurs after birth. The aim of this article is to present the case of a newborn with intestinal obstruction characterized by microcolon, dilated small bowel and megacystis known as Megacystis-Microcolon-Intestinal Hypoperistalsis syndrome, a very rare cause of intestinal obstruction. So far, less than three hundred cases have been reported in the literature. The prognosis of this syndrome is generally poor with most affected children dying in the neonatal period or infancy. Intestinal obstruction is a serious and life threatening condition. It may be explained by gross anatomical, histological or other abnormalities affecting the gut of the fetus.  Differential diagnosis may also take into account various anatomical, histological, or other abnormalities that may result in the obstruction of gut.  The incidence of intestinal obstruction is estimated at about one in 1500 live births.2 The cause of obstruction can be extrinsic or intrinsic. Atresia is quite common in the anorectal region, while atresia of the stomach occurs rarely. Colonic atresia is a rare condition of bowel obstruction in neonates. Membranous or complete obstruction may be present in the small intestine and may affect multiple sites. Stenosis may result from extrinsic or intrinsic factors. It arises from the failure of physiological rotation or fixation of the intestine in utero. Malrotation is one of the most serious cases, when there is incomplete rotation of the intestine into flexura duodenojejunalis or incomplete rotation of the caecum. Duodenal obstruction may also result when adhesion bands of Ladd straps down the second part of the duodenum. The second group of causes of bowel obstruction in the newborn consists of functional intestinal obstructions. It develops due to defects of bowel inervation and differentiation of ganglion cells. It includes small left colon syndrome or neuronal intestinal dysplasia type A and neuronal intestinal dysplasia type B.