The syndrome of polysplenia

Zach, M.; Beitzke, A.; Gypser, G.; Sager, W.

Rofo Fortschritte auf dem Gebiete der Rontgenstrahlen und der Nuklearmedizin 126(5): 454-460


ISSN/ISBN: 1438-9029
PMID: 142049
DOI: 10.1055/s-0029-1230615
Accession: 068521881

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A strong tendency to symmetrical development of the organ systems manifests itself in the syndrome of Polysplenia. These symmetrical organs show the anatomy of leftsided structures. For this reason the syndrome is also referred to as the "syndrome of bilateral leftsidedness". It encompasses complex abnormalities of situs, typical cardiac and vascular deformities as well as extracardiac malformations. Polysplenia is the leading symptom. Diagnosis is rarely made intra vitam. Certain typical deformities can give hints to this diagnosis, which is finally proved by szintigraphic and arteriographic visualization of multiple spleens. A typical case is reported and subsequently discussed according to the picture of this syndrome in the literature.