Polysplenia syndrome in adulthood: A case report of incidental discovery.

The Polysplenia Syndrome (PSS) is a form of heterotaxy, a rare congenital anomaly with an estimated incidence of 1 in 250,000 live births, first described by Helwig in 1929. Most patients with polysplenia syndrome die during the neonatal period due to severe associated cardiac and biliary anomalies. Nevertheless, some individuals present with moderate cardiovascular malformations or abdominal anomalies, often discovered incidentally in adulthood.