Ileal atresia, malrotation and Hirschsprung's disease: A case report

Gordon G. Wisbach, W. David Vazquez
2013 Journal of Pediatric Surgery Case Reports  
Ileal atresia associated with malrotation is rare, but the additional diagnosis of Hirschsprung's disease (HD) in the same patient has not been described in the literature. This case report presents a newborn with a bowel obstruction requiring surgery for ileal atresia and malrotation. The post-operative course was complicated by a distal bowel obstruction. Evaluation of the resected bowel from the original surgery was consistent with total colonic aganglionosis, and a diverting ileostomy was
more » ... rformed. Delay in diagnosis of HD is not uncommon when an initial gastrointestinal anomaly is diagnosed and should be suspected when bowel function fails to return after corrective surgery. Published by Elsevier Inc. Atresia of the intestinal tract associated with malrotation is rare, but the additional diagnosis of Hirschsprung's disease (HD), or total colonic aganglionosis, in the same patient does not appear in the literature. Jejunoileal atresia (JIA) occurs in approximately 1 in 5000 live births and occurs equally in males and females [1e4]. In rare instances, JIA has been found to be associated with HD, malrotation and other disorders [1,5e7]. The coexistence of these conditions can complicate the evaluation of bowel obstruction, and the co-diagnosis of HD is often not confirmed appropriately at the time of laparotomy. This article presents an infant who had ileal atresia with malrotation further complicated by total colonic aganglionosis. We report a unique combination of gastrointestinal anomalies in the same patient that has not been previously reported. Case report A full-term male infant was born via an uncomplicated spontaneous vaginal delivery with a birth weight of 3.4 kg in a community hospital. He developed progressive abdominal distention during the first day of life and did not pass meconium. After 2 bouts of bilious emesis, transfer was arranged for further evaluation and management in a tertiary medical center. On admission, the patient was in mild distress. His abdomen was distended, and bowel sounds were present. The anus was patent with normal sphincter tone, and a small amount of meconium was present on rectal stimulation. No other malformations were apparent on physical exam. Abdominal roentgenograms (AXR) demonstrated numerous loops of bowel in a right-sided abdominal distribution and a separate enlarged loop in the left lower quadrant. No free air was appreciated. As well, on the plain film, hemivertebral bodies were seen at the distal thoracic spine with dysplasia of the left ribs. Concern for a distal bowel obstruction was evaluated by a contrast enema that showed a decreased caliber, but not micro colon, located in the left side of the abdomen. An upper gastrointestinal contrast study revealed passage of contrast in a corkscrew fashion in the right upper quadrant without crossing midline consistent with malrotation, (Fig. 1) . Exploratory celiotomy revealed a type IIIb ileal atresia as well as malrotation with a narrow mesenteric base and no volvulus. The cecum and 5 cm of atretic terminal ileum were in the left lower quadrant (Fig. 2) . A Ladd procedure and ileocecectomy followed by ileoascending colostomy were performed. Post-operatively, the patient recovered well in the neonatal intensive care unit and received total parental nutrition while awaiting return of bowel function. After 2 weeks, he tolerated complete nutritional support orally and passed smears of meconium, however only with rectal stimulation. In the third post-operative week he developed abdominal distension and obstipation. An AXR revealed dilated loops of bowel without signs of perforation or pneumatosis. A repeat contrast enema on post-operative day 24 revealed a patent anastomosis with no sign of distal obstruction. The next day, a repeat AXR showed persistent dilated bowel loops
doi:10.1016/j.epsc.2012.12.002 fatcat:7xvus4rzizeltmi62afw5n32ma