Anysmay, polysplenia syndrome is an unusual disposition of intra-abdominal organs and unlike situs inversus it’s a spectrum of abnormalities and not a single set.
An asymptomatic 62-year-old woman underwent abdominopelvic computed tomography (CT) for inaugural diabetes.
CT showed dorsal pancreatic (P) agenesis (A) (Figure 1a).
Incidental abnormalities were found, including:
The diagnosis of type II diabetes was retained and after four months of metformin and insulin therapy, the rate of HbA1c was almost normalized. The remaining of the follow-up was unremarkable.
Heterotaxy syndrome (HS) (or situs ambiguus) is the result of an early embryological developmental failure in which there is an abnormal arrangement of thoraco-abdominal organs. In contrast to situs inversus, HS is not characterized by a single set of abnormalities but rather a spectrum.
Polysplenia syndrome (PS) (or left isomerism) is the subtype of HS with features of bilateral left-sidedness. No single anomaly is pathognomonic but the association of a sufficient number allows the diagnosis. The commonest is the presence of multiple spleens, right- or left-sided, with a consistent relationship to the stomach.
As in the present case, the other intra-abdominal abnormalities include:
Compared to the other HS (i.e., right isomerism [or asplenia]) PS is often detected incidentally in adults. Indeed, it is associated with less severe or no congenital heart disease and no immune system deficiency .
The author has no competing interests to declare.
Fulcher, AS and Turner, MA. Abdominal manifestations of situs anomalies in adults. Radiographics. 2002; 22(6): 1439–56. DOI: https://doi.org/10.1148/rg.226025016