Computed tomography features of polysplenia syndrome in adulthood: a case series

Due to the rare condition of polysplenia syndrome (PS) in adulthood, this study aims to present the computed tomography (CT) features of PS in adulthood.

This retrospective study was conducted from January 2010 and September 2024 in cases with CT-proven PS. Presentations in CT images, laboratory tests, and concomitant diseases were recorded.

Twenty one cases (median age, 52 years; range, 23 to 79) were evaluated. According to the CT images, multiple spleens were observed in all cases with the median count of 4 and bizarre-shaped spleens were revealed in 7 (33.3%) cases. Nine (42.9%) cases presented with short pancreas and 2 of whom had the history of diabetes mellitus. Additionally, 3 (14.3%) cases had liver morphologic anomalies. There were 2 (9.5%) cases presented with left-sided inferior vena cava and 3 (14.3%) cases of dilation of azygos vein. None of these cases was found to have hypersplenism or cardiac anomaly.

In adult cases with PS, bizarre-shaped spleens and short pancreas were more frequently observed than other anomalies. Absence of severe cardiac anomalies and congenital heart defects might be the reason why these cases survived into adulthood.