Polysplenia syndrome with semi-annular pancreas, midgut malrotation and interrupted inferior vena cava: A rare adult case report

Polysplenia syndrome is a rare congenital anomaly within the heterotaxy spectrum. It is typically diagnosed in childhood due to associated cardiac malformations, whereas detection in adults without cardiovascular involvement is uncommon. We report a 50-year-old woman presenting with recurrent abdominal pain and vomiting. Contrast-enhanced CT demonstrated multiple splenic nodules consistent with polysplenia, a semi-annular pancreas partially encircling the duodenum, midgut malrotation, and absence of the intrahepatic segment of the inferior vena cava (IVC) with azygos/hemiazygos continuation. This constellation of anomalies is unusual in adults and clinically significant. Pancreatic and intestinal malformations may predispose to abdominal symptoms, while vascular variants such as interrupted IVC have important implications for surgical and interventional procedures. CT plays a pivotal role in characterising these abnormalities and guiding safe management. Adult presentation of polysplenia syndrome without cardiac involvement is rare. Awareness of associated gastrointestinal and vascular anomalies is crucial to avoid misdiagnosis and intraoperative complications.