[Polysplenia with partial abdominal visceral heterotaxia and small pancreas]

Several morphological congenital abnormalities are associated in the polysplenia syndrome: partial visceral abdominal heterotaxy, levoisomerism, abdominal vascular abnormalities, cardiac and extrahepatic biliary malformations and polysplenia. The syndrome is usually apparent during childhood because of its severe outcome; its detection during adult life is rare. We present a 42-years-old patient, asymptomatic, with right sided polysplenia and stomach, intestinal malrotation, centrally located liver with both lobes of similar size, preduodenal portal vein, interruption of the inferior caval vein with continuation via the azygos vein. There were no cardiac malformations nor lung levoisomerism. The pancreas was atrophic, an occasionally described finding. CT scan may be useful for the study of this patients in order to identify every anatomic abnormality, and plan for future surgical operations.