Intestinal congenital malformations

22.1 General considerations Atresias can occur at any level of the gastrointestinal tract Atresias result most likely after mesenteric vascular accidents caused by intrauterine intussusception, volvulus or internal hernia No extraintestinal-associated anomalies are consistently present Jejunoileal atresia is the most common cause of congenital intestinal obstruction in newborns (incidence 1:1000 live births) About 35% of jejunoileal atresia patients are significantly premature Differential diagnosis of jejunoileal atresia includes: meconium disease, meconium plug syndrome, malrotation, Hirschsprung's disease, and intussusception Colonic atresia is a rare congenital disorder (comprises 5%-10% of all intestinal atresias) Differential diagnosis of colonic atresia includes: malrotation with volvulus, meconium disease, Hirschsprung's disease, and megacystis hypoperistalsis syndrome 22.2 General considerations Malrotation includes any aberration of the gut position The incidence of malrotation in the general population is 0.5% Malrotation can be present throughout life without clinical signs Approximately 60% of malrotation patients present with signs in the neonatal period (40% in the first week) Every second to third patient with malrotation has associated anomalies (duodenal atresia, prune-belly syndrome, Hirschsprung's disease) Malrotation is an integral component of omphalocele, gastroschisis, and diaphragmatic hernia 22.3 General considerations Intestinal volvulus is a condition in which the intestine twists around the mesentery Malrotation is a common cause During the process of embryonic gut rotation and fixation, three conditions develop where volvulus is possible: • Nonrotation (the midgut volvulus risk is high) • Abnormal rotation of the cecocolic limb (the volvulus risk is very high) • Abnormal rotation of the duodenojejunal limb (the volvulus risk is low) Volvulus during intrauterine life can lead to ischemic atresia Intestinal volvulus may occur later in life in situations involving postoperative or congenital adherences, and tumor masses 22.4 General considerations Duplications can occur at any location along the gastrointestinal tract Duplication cysts are the most common Gastrointestinal tract duplications are lined with intestinal epithelium and their walls have smooth muscles Communication with the intestinal tract is possible Antenatal diagnosis is possible for cystic structures Association with other malformations is common (50% of cases), e.g., vertebral abnormalities 22.5 Different types of stoma complications are presented and the best way of treatment. 22.6 In short bowel syndrome the prerequisite of survival is discussed. Conservative therapy, including initial total parenteral nutrition and its complications is mentioned. Important surgical procedures such as tapering, the Bianchi procedure and small bowel transplantation are presented.