Polysplenia syndrome

Case contributed by Dayana Emanuilova
Diagnosis certain


The patient was admitted to the hospital presenting symptoms of chest pain radiating to the back and tachycardia. CT scan made to rule out aortic dissection. Previous interventions - aortic valve replacement, resection of the ascending aorta and implantation of aortic prosthesis. Aortic coarctation surgery in childhood.

Patient Data

Age: 60 years
Gender: Male

The right lung is bilobed (absence of the horizontal fissure) with the presence of an inferior accessory fissure. Bilateral hyparterial main bronchi.

There is a narrowing of the proximal descending aorta with known aortic coarctation that underwent surgery in childhood and a residual stenosis remains.

There is SVC duplication.

Multiple spleens of different sizes (oval-shaped) are located in the left upper quadrant.

Right-sided duodenal-jejunal junction relative to the mid-line. Small intestine with a right-sided location primarily. On the other hand, the large colon is located on the left for the most part.

Dorsal pancreatic agenesis.

Additional findings:

  • aortic prosthesis of the ascending aorta with periprosthetic retrograde contrast filling (might be mistaken for aortic dissection - pitfall)

  • aortic valve replacement

  • dilated ascending aorta

  • enlargement of the caudate lobe of the liver

  • atypical position of the gallbladder (ventral and adjacent to the anterior abdominal wall)

  • mild prostatic hypertrophy

Case Discussion

Polysplenia syndrome is a rare congenital anomaly that is associated with multiple spleens and a variety of congenital anomalies of other organs such as intestinal malrotation, short pancreas, midline or left-sided liver, cardiovascular anomalies and many others.

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