Key result
Asplenia syndrome was more commonly associated with conotruncal anomalies, whereas polysplenia syndrome was specifically associated with the absence of the inferior vena cava with azygos continuation.
Case Report (n=10)
The study highlights distinct morphological and cardiovascular anomalies between asplenia and polysplenia syndromes, suggesting different pathogenetic mechanisms.
May inform recognition of heterotaxy variants; hypothesis-generating and requires validation in larger cohorts.
This report described the morphological characteristics of seven cases of asplenia syndrome and three of polysplenia syndrome. Each syndrome has been characterized by a tendency for symmetric development of normally asymmetric organs, with varying degrees of cardiovascular anomalies. These latter anomalies are usually present in asplenia syndrome to a greater extent than in polysplenia syndrome. While, as observed in our material, the conotruncal anomalies were present more commonly in cases with asplenia, and absence of inferior vena cava with azygos continuation was seen specifically in all the cases with polysplenia. This evidence implied the presence of some pathogenetic distinction between the two syndromes.
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Shinohara et al. (1982) conducted a case report in Asplenia and polysplenia syndrome (n=10). Asplenia syndrome vs. Polysplenia syndrome was evaluated on Morphological characteristics and cardiovascular anomalies. Asplenia syndrome was more commonly associated with conotruncal anomalies, whereas polysplenia syndrome was specifically associated with the absence of the inferior vena cava with azygos continuation.
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