Splenic Torsion in a Patient with Polysplenia Syndrome and Dorsal Pancreatic Agenesis: A Case Report

Partial splenic torsion in polysplenia syndrome is an extremely rare condition. We encountered and performed surgery for a case of partial splenic torsion in a patient with polysplenia syndrome coexisting with agenesis of the dorsal pancreas.

A 30-year-old woman with heterotaxy syndrome presented with intermittent epigastric and right lateral abdominal pain persisting for 2 weeks. She was referred to our hospital. Abdominal contrast-enhanced CT revealed multiple spleens on the right side, a right-sided stomach, and agenesis of the dorsal pancreas. One of the 5 spleens showed no contrast enhancement. Suspecting torsion or infarction, we opted for surgical intervention. Intraoperative findings revealed a small spleen with poor coloration and 180-degree torsion, which we removed. Additionally, we identified another poorly anchored spleen and performed splenopexy to secure this wandering spleen.

Polysplenia syndrome is a rare condition involving multiple spleens in the setting of heterotaxy syndrome-a defect in the left-right axis of the thoracic or abdominal organs without a complete mirror image. Several reports have described polysplenia syndrome coexisting with agenesis of the dorsal pancreas. Including our case, 11 instances of splenic torsion associated with polysplenia syndrome have been reported in English, with the current patient being the oldest. Furthermore, this is the 1st reported case of splenic torsion occurring in the context of polysplenia syndrome with agenesis of the dorsal pancreas. Our patient had 2 notable anatomical characteristics: first, a vascular variation in which the right celiac artery lacked a common hepatic artery and instead formed a common trunk with the superior mesenteric artery and common hepatic artery, resembling Adachi type VI (group 24); and second, a centrally located, unfixed dorsal spleen that underwent torsion. We hypothesize that splenic torsion occurred due to the central spleen's mobility and its slightly elongated splenic artery.