Thoraco-omphalopagus twins with complex biliary tree, pancreatic and intestinal anatomy: Planning behind successful separation, “the Santorini difference”

Here we report the separation of thoraco-omphalopagus twins at week 17th after birth, that are already 11-year-old and performing satisfactorily at the family, school, and social level. At birth, hearts shared a common pericardium and livers were fused along the anterior edge. Small bowel was common from duodenum down to the terminal ileum and invested by a dual mesentery. From the bifurcation bowels were normal. Bile and pancreatic ducts discharged into the duodenum but whether they were fused, or not, and details of the terminal ducts did not show up in the study. Vasculatures were independent. Teams -anesthesia, reconstructive, cardio-surgery, digestive, management-were formed and given specific tasks. Separation, programed for week 24, was performed at week 17 due to an emergency. In the intervention, the central step was the exploration of bile and pancreatic discharges. The duct of Santorini was searched for and identified; it discharged at the minor papilla in the duodenum prior to fusion. Both Wirsungs and the already fused common bile duct discharged in a single major papilla in a fused duodenum. The distance between papillae allowed for a duodenal transection between them. This scenario, considered during planning, commanded the separation. T1 kept the major papilla, while T2, the minor papilla complemented with a cholecystojejunostomy. Careful planning and management were keys to overcome both emergency and incomplete preoperative information.