Locally invasive cholangiocarcinoma causing gastric outlet obstruction in heterotaxy syndrome: A case report and review of literature

Heterotaxy syndrome is a disease of embryo development resulting in abnormal distribution of thoracic and abdominal organs across the left-right axis. In this case, A 77-year-old gentleman was admitted with gastric outlet obstruction secondary to cholangiocarcinoma. This is on a background of heterotaxy syndrome, specifically situs ambiguus. The patient's anatomical variations included a right-sided stomach, midline liver, and asplenia. Due to variant anatomy and risk of aspiration; endoscopy was abandoned in favor of surgical bypass via a gastrojejunostomy. Although technically challenging, complex upper abdominal surgery in heterotaxy syndrome has been described in the literature. Variations in anatomy observed in heterotaxy syndrome may render patients ineligible for resection of cholangiocarcinoma and increase the risk of complications. Careful preoperative planning with imaging and meticulous intraoperative dissection is required.