Abstract
BACKGROUND: Splenic torsion is a rare but potentially serious event. It can occur in association with various anatomical anomalies, such as polysplenia. The presence of multiple small spleens is often associated with other abdominal and chest abnormalities, defining a condition known as Polysplenia Syndrome (PSS). CASE PRESENTATION: We report on a 14-year-old girl who presented with a 3-day history of left lower abdominal pain and vomiting, with a known history of congenital heart disease associated with Situs Viscerum Inversus and polysplenia. She was found to have normal vital signs but elevated C-reactive protein, and leukocytosis with neutrophilia. In light of the clinical and laboratory data, in addition to the suggestive imaging findings revealed on US and contrast-enhanced abdominal CT, a splenic torsion was suspected. The patient underwent a laparotomy, which confirmed a pedunculated mass in the left flank and iliac fossa, consistent with torsion of one of the spleens, and allowing complete resection of the lesion. The postoperative course was uneventful, and she was discharged after 10 days. CONCLUSIONS: Splenic torsion in PSS is an extremely rare condition that may present in a time-critical manner, with nonspecific clinical manifestations and abnormal laboratory results, representing a real diagnostic challenge.