Digestive tract disorders associated with asplenia/polysplenia syndrome

Polysplenia syndrome with preduodenal portal vein detected in adults

“…Since splenic anomalies (splenic agenesis, hypogenesis and polysplenia) are often accompanied by anomalies in the cardiovascular tract and other abdominal organs, it can be inferred that the spleen plays a significant role during the early embryonic stage [12] .…”

Anatomical Variation and Its Management in Transplantation

“…Recognition of this anomaly, and preservation of the vein during retrieval and implantation, is critical. It is important to remember that it may be one manifestation of portal vein duplication (vide infra), and that other associated anomalies such as polysplenia syndrome and absence of an infra-hepatic IVC may be present (47)(48)(49).…”

“…This anomaly may be associated with other vascular anomalies and it is also associated with the biliary atresia-polysplenia syndrome, which is itself associated with a preduodenal portal vein and abdominal situs inversus (48,49,51).…”

A Canine Case of Partial Heterotaxia Detected by Radiography and Ultrasound.

“…In a study of 78 asplenic humans, cardiac abnormalities and heterotaxia, cardiac abnormalities, and asplenia alone were detected in 37.2%, 25.6%, 7.7%, and 5.1%, respectively [8]. It has been reported that 40% of asplenia and polysplenic cases have digestiv tract disorders [6]. In another study of 85 humans with asplenia, 45 were determined to have malformations in the systemic venous system [4].…”

“…In humans, splenic malformations include asplenia, splenic hypogenesis, spleen accessories, and polysplenia. Asplenia, splenic hypogenesis, and polysplenia are frequently complicated by cardiac and vascular abnormalities and/or partial heterotaxia [1,2,6]. Splenic malformation complicated by cardiac/large vessel malformation and/or heterotaxia is generally called "asplenia syndrome" or "asplenia-polysplenia syndrome" [1,11].…”