Polysplenia syndrome

Case contributed by Dr Ian Bickle

Presentation

Attended respiratory clinic with a persistent cough. The resulting chest radiograph prompted further investigation with CT.

Patient Data

Age: 55 years
Gender: Female

Note: This case has been tagged as "legacy" as it no longer meets image preparation and/or other case publication guidelines.

ct

In summary:

Case Discussion

Features of polysplenia syndrome (a.k.a. left sidedness or left isomerism):

  • interruption of the hepatic segment of the IVC with azygos continuation (present in 65% of cases); the azygos vein is typically also enlarged
  • multiple spleens - usually at least one almost normal sized spleen with numerous additional spleens of variable size
  • dextrocardia is present in 35-40% of cases with the prevalence of congenital heart disease even higher at nearly 1 in 2 patients
  • hepatic veins drain directly into the right atrium

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