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Intra-Abdominal Abnormalities Associated with Polysplenia Syndrome

Author:

Hadrien Renier

Université de Liège, BE
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Abstract

What to look for in case of polysplenia and/or unusual disposition of several intra-abdominal organs.

How to Cite: Renier H. Intra-Abdominal Abnormalities Associated with Polysplenia Syndrome. Journal of the Belgian Society of Radiology. 2019;103(1):54. DOI: http://doi.org/10.5334/jbsr.1903
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  Published on 02 Oct 2019
 Accepted on 11 Sep 2019            Submitted on 07 Aug 2019

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.

Report

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:

  • – Four spleens (S), one adjacent to the stomach (Figure 1b).
  • – Right renal hypotrophy (Figure 1c).
  • – Midline falciform ligament (Figure 1d).
  • – Duplicated inferior vena cava system (IVC) with dilated azygos (A) and hemiazygos (H) continuation and no communication with hepatic veins (N) (Figure 2).
  • – Intestinal nonrotation: the small bowel was right-sided (S), the colon was left-sided (C), the superior mesenteric artery (A) was to the right of the vena (V), and there was no midline crossing by the duodenum (D) under the aorto-mesenteric junction (P) (Figure 3).
Figure 1 
Figure 2 
Figure 3 

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.

Comment

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:

  • – midline liver with or without biliary abnormality,
  • – truncated pancreas with presence of the head and a variable portion of the body,
  • – azygos continuation of the IVC,
  • – midline or right-sided aorta,
  • – right-sided stomach and/or abnormalities of the mesentery rotation.

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 [1].

Competing Interests

The author has no competing interests to declare.

References

  1. 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 

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