Abdominal heterotaxy syndrome with annular pancreas, adhesive bands, and internal herniation: A rare case managed at a tertiary hospital in Lilongwe, Malawi

Abstract

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Heterotaxy syndrome is a disorder of embryonic development resulting in anomalous thoracoabdominal organ determination of the left–right axis. It presents with cardiac and extracardiac abnormalities associated with significant morbidity and mortality. We managed a 3-year-and-5-month-old boy with heterotaxy, who presented with bilious emesis, abdominal pain, distension, and obstipation. After resuscitation and nasogastric tube placement, an upright abdominal x-ray revealed a distended, right-sided stomach with an air–fluid level. Exploratory laparotomy confirmed this finding and revealed a midline liver, left-sided gallbladder, shortened large bowel, and internal herniation. Initial surgery reduced the small bowel and pexyed the large bowel to the right. The patient presented with obstructive symptoms 14 days after discharge, requiring a duodenojejunostomy to bypass an annular pancreas. Heterotaxy is a rare condition with variable anatomical presentations that may cause obstructive symptoms, often requiring surgical intervention. These cases can be complex, especially due to the potential for multiple coexisting sources of obstruction.