Polysplenia syndrome

Case contributed by Mostafa Elfeky
Diagnosis certain

Presentation

Bilateral loin pain and suspected urinary tract infection.

Patient Data

Age: 40 years
Gender: Male

CT abdomen

ct

Multiple rounded soft tissue density structures at the anatomical site of the spleen, largest measured 4.2 x 4.5 cm (polysplenia).

The liver is average in size with diffuse homogeneous decreased density, likely diffuse fatty infiltration.

The pancreatic head is only seen with irregular shape, absent body, and tail (dorsal pancreatic agenesis).

Azygos continuation of the IVC.

Case Discussion

The combination of incidental findings of multiple splenules with absent spleen, dorsal agenesis of the pancreas and azygos continuation of the IVC are suggestive of polysplenia syndrome.

Dorsal pancreatic agenesis is one of the associations of polysplenia syndrome and appears as a short, rounded pancreatic head adjacent to the duodenum with the absence of the pancreatic neck, body, and tail as well as the duct of Santorini, and the minor duodenal papilla.  

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