POLYSPLENIA WITH PARTIAL VISCERAL ABDOMIN AL HETEROTAXY AND ATROPHY OF THE PANCREAS

Several morphological congenital abnormalities are associated in the p olysplenia syndrome: partial visceral abdominal heterotaxy, levoisomer ism, abdominal vascular abnormalities, cardiac and extrahepatic biliar y malformations and polysplenia. The syndrome is usually apparent duri ng childhood because of its severe outcome; its detection during adult life is rare. We present a 42-years-old patient, asymptomatic, with r ight 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 az ygos 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 eve ry anatomic abnormality, and plan for future surgical operations.