Splenic torsion in the setting of polysplenia syndrome

Abstract

Polysplenia syndrome is a rare condition that occurs when polysplenia co-exists with various other congenital anomalies. Splenic torsion in the setting of polysplenia syndrome is extremely rare, having been documented only five times previously in the English literature. We present a case of a 9 year-old female who underwent laparoscopic splenectomy for ischemic polysplenia secondary to splenic torsion in the setting of polysplenia syndrome. This previously healthy patient presented to the emergency department with a 3-day history of abdominal pain and emesis. She was found to have leukocytosis and computed tomography (CT) revealed evidence of polysplenia with torsion of multiple splenules, as well as an annular pancreas, intestinal malrotation, and an interrupted inferior vena cava (IVC) with azygous continuation. To our knowledge, this was the first documented case of splenic torsion in the setting of polysplenia syndrome with concurrent anomalies of intestinal malrotation, interruption of the IVC with azygous continuation, and preduodenal portal vein (PDPV). The goal of our case report is to describe this rare phenomenon while detailing the diagnostic and management considerations. Splenic Torsion, Polysplenia Syndrome, Polysplenia, Malrotation.