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.
From the case:
Polysplenia syndrome
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In summary:
- polysplenia
- dextrocardia
- azygous continuation of the IVC
- direct drainage of hepatic veins into the right atrium
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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