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However, in a minority, other anomalies may be present, some as defined syndromes, others as a non-random association. The most fully characterized is that of the biliary atresia splenic malformation syndrome seen in about 10% of European and North American series with a typical array of unusual extrahepatic anomalies (<jats:italic>e.g.<\/jats:italic>, situs inversus, polysplenia, absence of the inferior vena cava, and a preduodenal portal vein). Its underlying genetic background is obscure in most cases. 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